benign mediastinal psammomatous neurilemmoma

A schwannoma that arises from the posterior mediastinum. It is characterized by the presence of psammoma bodies.


ependymal tumor of spinal cord

biolink:Disease biolink:DiseaseOrPhenotypicFeature biolink:BiologicalEntity biolink:NamedThing biolink:Entity biolink:ThingWithTaxon

An ependymal tumor that arises from the spinal cord.


brain stem ependymoma

An ependymoma that arises from the brain stem.


classical glioblastoma

A molecular subtype of glioblastoma characterized by lack of p53 mutations, chromosome 7 amplifications or deletions, and high levels of EGFR amplification.


grade III glioma

A group of malignant gliomas that includes anaplastic astrocytoma, anaplastic oligodendroglioma, anaplastic oligoastrocytoma, and anaplastic ependymoma.


childhood low-grade glioma

A low-grade glioma that occurs in children and encompasses tumors of astrocytic, oligodendroglial, and mixed glial-neuronal histology.


adult embryonal tumor with multilayered rosettes, c19mc-altered

An embryonal tumor with multilayered rosettes, C19MC-altered, occurring in adults.


spinal cord glioma

A neoplasm that arises from glial cells in the spinal cord. Representative examples include astrocytoma, oligodendroglioma, and ependymoma.


nodular medulloblastoma

A medulloblastoma characterized by nodularity and neuronal differentiation.


adult brainstem glioma

A brain stem glioma that occurs in an adult.


myxopapillary ependymoma

Myxopapillary ependymoma (MEPN) describes a slow growing ependymoma located almost exclusively in the conus medullaris-cauda equina-filum terminale region of the spinal cord, presenting in all age groups, and manifesting with variable symptoms such as neck pain, vomiting and unsteady gait and metastasis. It has a more aggressive disease course and is seen in the pediatric population.


large cell medulloblastoma

A medulloblastoma composed of large cells with prominent nucleoli and a larger amount of cytoplasm in contrast with the cells of the classic medulloblastoma.


astroblastoma, MN1-altered

An astroblastoma that is characterized by astroblastoma-like morphology with MN1 rearrangements involving the meningioma 1 (MN1) gene on chromosome 22q.


mediastinal granular cell myoblastoma

An exceptionally rare, generally benign, granular cell tumor that arises from the mediastinum. All the reported cases were located in the posterior mediastinum.


sympathetic neurilemmoma

A benign tumor derived from schwann cells of the peripheral sympathetic nervous system, including the sympathetic plexus.


cellular ependymoma

An ependymoma which shows conspicuous cellularity without a significant increase in mitotic rate. (Adapted from WHO)


adult oligodendroglioma

An oligodendroglioma occurring during adulthood.


IDH-mutant and 1p/19q-codeleted oligodendroglioma

An anaplastic oligodendroglioma that has material basis in IDH gene family mutation and combined whole-arm losses of 1p and 19q (1p/19q codeletion).


An embryonal tumor with multilayered rosettes, C19MC-altered that arises from the infratentorial region and occurs in children.


papillary tumor of the pineal region

Papillary tumor of the pineal region (PTPR) is a very rare neoplasm of the pineal region that is thought to arise from the specialized ependymocytes of the subcommissural organ and that manifests with visual disturbances, headaches, loss of coordination and balance, nausea and vomiting due to obstructive hydrocephalus.


high grade astrocytic tumor

An anaplastic astrocytoma (grade III astrocytic tumor) or glioblastoma (grade IV astrocytic tumor).


medulloblastoma non-WNT/non-SHH

A medulloblastoma that is characterized as a molecular subtype that is not associated with activation of the WNT pathway or sonic hedgehog (SHH) pathway and TP53 mutations are absent.


central nervous system Ewing sarcoma/peripheral primitive neuroectodermal tumor

A rare Ewing sarcoma/peripheral primitive neuroectodermal tumor that affects the central nervous system either as a primary dural neoplasm or by direct extension from adjacent soft tissues or bone.


malignant perineurioma

A very rare malignant tumor with morphologic features similar to those of benign perineurioma of soft tissue along with hypercellularity, nuclear atypia, hyperchromasia, and a high mitotic rate.


cerebellar pilocytic astrocytoma

A WHO Grade 1 astrocytoma which arises in the cerebellum. The tumor is composed of spindle shaped cells with numerous collections of reddish astrocytic fibers called Rosenthal fibers. Over 80% or the cerebellar astrocytomas of childhood are pilocytic. Pilocytic astrocytomas may rarely occur in adults. They are usually treated by surgical resection and in most cases have a favorable prognosis.


retroperitoneal neuroblastoma

A neuroblastoma that involves the retroperitoneal space.


intracranial primitive neuroectodermal tumor

A primitive neuroectodermal tumor that involves the brain.


adult pineoblastoma

A pineoblastoma occurring in adults.


low grade astrocytic tumor

A grade I or grade II astrocytic tumor. This category includes pilocytic astrocytoma (grade I), subependymal giant cell astrocytoma (grade I), and diffuse astrocytoma (grade II).


childhood pilocytic astrocytoma

A pilocytic astrocytoma that occurs during childhood.


infant-type hemispheric glioma

A malignant astrocytoma that is characterized by receptor tyrosine kinase fusions in the NTRK family, ROS1, ALK, or MET genes, that arises in the cerebral hemisphere and occurs in early childhood.


diffuse astrocytoma

A low-grade (WHO grade II) astrocytic neoplasm. It is characterized by diffuse infiltration of neighboring central nervous system structures. These lesions typically affect young adults and have a tendency for progression to anaplastic astrocytoma and glioblastoma. Based on the IDH genes mutation status, diffuse astrocytomas are classified as IDH-mutant, IDH-wildtype, and not otherwise specified.


gemistocytic astrocytoma

A rare variant of diffuse astrocytoma. It is characterized by the presence of a conspicuous, though variable, fraction of gemistocytic neoplastic astrocytes. Gemistocytes are round to oval astrocytes with abundant, glassy, non-fibrillary cytoplasm which appears to displace the dark, angulated nucleus to the periphery of the cell. To make the diagnosis of gemistocytic astrocytoma, gemistocytes should amount to more than approximately 20% of all tumor cells. (Adapted from WHO)


brain oligodendroglioma

A oligodendroglioma that involves the brain.


adult brainstem mixed glioma

A mixed glioma of the brain stem that occurs during adulthood.


A mixed glioma characterized by the presence of astrocytic, ependymal, and oligodendroglial neoplastic components.


intermixed schwannian stroma-rich ganglioneuroblastoma

A ganglioneuroblastoma characterized by the presence of neuroblastic cells in a Schwannian stroma, without the presence of hemorrhagic neuroblastic nodules.


atypical teratoid rhabdoid tumor

Atypical teratoid rhabdoid tumor (ATRT) is a highly malignant central nervous system (CNS) rhabdoid tumor (RT) found almost exclusively in children.


adult infiltrating astrocytic neoplasm

An infiltrating astrocytic tumor of the brain occurring in adults.


plexiform schwannoma

A schwannoma characterized by a plexiform or multinodular growth pattern. It usually arises from the skin or subcutaneous tissues in the extremities, trunk, and head and neck.


posterior fossa ependymoma

A high grade ependymoma that is located within the posterior fossa.


cerebral astrocytoma

An astrocytoma that arises from the cerebral hemispheres.


oligodendroglial tumor

Oligodendrogliomas are cerebral tumors that are differentiated from other gliomas on the basis of their unique genetic characteristics and better response to chemotherapy. These tumors are classified according to their grade (low grade oligodendrogliomas: grade II of the WHO classification and anaplastic oligodendrogliomas: grade III of the WHO classification) and according to their pure or mixed histology (oligoastrocytomas).


medulloblastoma SHH activated

A medulloblastoma that is characterized as a molecular subtype by activation of the sonic hedgehog (SHH) pathway and TP53 mutations that may be present or absent.


astrocytic tumor

A glial tumor of the brain or spinal cord showing astrocytic differentiation. It includes the following clinicopathological entities: pilocytic astrocytoma, diffuse astrocytoma, anaplastic astrocytoma, pleomorphic xanthoastrocytoma, subependymal giant cell astrocytoma, and glioblastoma.


epithelioid neurofibroma

A rare neurofibroma with epithelioid morphology.


granular cell cancer

An uncommon granular cell tumor which may metastasize to other anatomic sites. Morphologic characteristics include the presence of spindling neoplastic cells, necrosis, extensive pleomorphism, prominent nucleoli, and increased mitiotic activity.


childhood optic tract astrocytoma

An astrocytoma that arises from the visual pathway and occurs during childhood.


diffuse low-grade glioma, MAPK pathway–altered

A low grade glioma that is characterized by a gene alteration that results in a MAPK pathway abnormality, with morphological features of astrocytoma or oligodendroglioma.


nasal ganglioglioma

Nasal ganglioglioma is a rare tumor, presenting in newborns, containing both neuronal and astrocytic components and that can be endonasal, extranasal or both. It is usually identified as a nasal mass that may cause feeding difficulties and nasal obstruction.


olfactory neuroblastoma

An olfactory neuroblastoma arising in the paranasal sinus.


melanotic neurilemmoma

A rare circumscribed, non-encapsulated and grossly pigmented nerve sheath tumor. It is composed of cells with the immunophenotypic and electron microscopic features of Schwann cells which contain melanosomes and are positive for melanoma markers. It usually involves spinal nerve roots but may occur in other locations. It may be associated with PRKAR1A gene mutation and Carney complex. Malignant behavior has been reported in a significant number of patients.


RELA fusion-positive ependymoma

A supratentorial ependymoma characterized by a gene fusion involving ZFTA and RELA genes. It accounts for the majority of supratentorial ependymomas in children. It has an unfavorable outcome when compared to other ependymoma subtypes.


cellular schwannoma

A morphologic variant of schwannoma characterized by hypercellularity, Antoni A pattern, and the absence of well-formed Verocay bodies.


extraneural perineurioma

Extraneural perineurioma is a rare tumor of cranial and spinal nerves arising from peripheral nerve sheet and composed exclusively or predominantly of cells showing perineurial differentiation. It presents as a well-circumscribed, rarely encapsulated mass, not associated with a recognizable nerve, most commonly arising in the dermis and subcutis of the extremities or trunk, or, rarely, in deep soft tissue or skin (e.g., in the stomach, kidney, pancreas, maxillary sinus, mandible, bronchial tree and the face). The clinical presentation depends on the localization.


cerebellar astrocytoma

Benign and malignant neoplasms of the cerebellum that arise from astrocytes. During childhood the majority are benign pilocytic astrocytomas. In adults both benign and relatively higher grade forms may occur. The most common presenting symptoms are headache, nausea, vomiting, ataxia of gait or limb, paresis, diplopia, and dizziness. Objective signs include weakness, long tract signs, dysmetria, gait ataxia, papilledema, and nystagmus. Surgical resection is often curative.


adult malignant schwannoma

A malignant peripheral nerve sheath tumor occurring during adulthood.


adult brain ependymoma

An ependymoma of the brain occurring in adults.


intraneural perineurioma

A WHO grade I perineurioma that arises within the endoneurium. It is characterized by the formation of pseudo-onion bulbs by the proliferating perineural cells.


retinal neuroblastoma

A neuroblastoma arising from the retina.


ependymal tumor of brain

A tumor arising from the ependymal lining of the ventricles.


vulvar granular cell tumor

A usually benign granular cell tumor that arises from the vulva.


A malignant astrocytoma that is characterized by the presence of five or more mitoses per 10 high-power fields.


spinal cord astrocytoma

A low or high grade astrocytoma that arises in the spinal cord.


mediastinum ganglioneuroblastoma

A ganglioneuroblastoma arising from the mediastinum.


medullomyoblastoma with myogenic differentiation

A rare malignant embryonal neoplasm arising from the cerebellum. It is characterized by the morphologic features of a medulloblastoma and the presence of a striated muscle component. Its clinical behavior is similar to medulloblastoma.


mixed astrocytoma-ependymoma

A tumor of mixed cell type with astrocytic components as well as ependymoma components.


diffuse intrinsic pontine glioma

A neuroglial tumor that arises from the middle portion of the brain stem. It usually affects children and has a poor prognosis.


epithelioid malignant peripheral nerve sheath tumor

A rare variant of malignant peripheral nerve sheath tumor composed predominantly or exclusively of epithelioid cells.


subependymoma

Subependymoma is a rare and slow growing type of ependymoma, often presenting in middle-aged adults, found more commonly in men than in women, usually located in the fourth and lateral ventricles and manifesting with variable symptoms including headache, nausea, and loss of balance. In some cases it can be asymptomatic. It is usually associated with a better prognosis than other forms of ependymoma.


Askin tumor

A primitive neuroectodermal tumor (small round blue cell tumor) of the thorax which can involve the periosteum, thoracic wall and/or pleura though it spares the lung parenchyma.


protoplasmic astrocytoma

A rare variant of diffuse astrocytoma. It is predominantly composed of neoplastic astrocytes showing a small cell body with few, flaccid processes with a low content of glial filaments and scant GFAP expression. This lesion is not well defined and is considered by some authors as an occasional histopathological feature rather than a reproducibly identifiable variant. When occurring in children, this neoplasm may be difficult to separate from pilocytic juvenile astrocytoma. (Adapted from WHO)


mediastinal melanocytic neurilemmoma

A melanotic schwannoma that affects the mediastinum.


neurohypophysis granular cell tumor

A generally benign intrasellar and/or suprasellar mass arising from the neurohypophysis or infundibulum. It is composed of nests of large cells with granular, eosinophilic cytoplasm due to abundant intracytoplasmic lysosomes. It generally has a slow progression and lacks invasive growth. (Adapted from WHO)


pilomyxoid astrocytoma

An astrocytic tumor of uncertain relation to pilocytic astrocytoma. It occurs predominantly in infants and young children. It is characterized by a monomorphic architectural pattern, usually associated with the absence of Rosenthal fibers and eosinophilic granular bodies. The clinical course is usually aggressive.


nodular ganglioneuroblastoma

A ganglioneuroblastoma characterized by the presence of neuroblastic cells in a Schwannian stroma, and the formation of hemorrhagic neuroblastic nodules.


embryonal tumor of neuroepithelial tissue


schwannomatosis 1

Schwannomatosis caused by germline mutations in the SMARCB1 gene.


neuroepithelioma

Peripheral neuroepithelioma is a rare noncentral nervous system tumor with evidence of primitive neuroectodermal differentiation.


childhood malignant schwannoma

A malignant peripheral nerve sheath tumor occurring in children.


IDH-wildtype glioblastoma

A glioblastoma that is characterized by high cellularity, high mitotic activity, necrosis or microvascular proliferation and that lacks mutations in IDH genes.


nerve sheath neoplasm

A benign or malignant neoplasm arising from the perineural cells in the sheaths surrounding the nerves. Representative examples include neurofibroma, schwannoma, and malignant peripheral nerve sheath tumor.


supratentorial ependymal tumor

An ependymal tumor arising from the supratentorial region of the brain.


adult spinal cord ependymoma

An ependymoma of the spinal cord occurring in adults.


medulloblastoma WNT activated

A medulloblastoma that is characterized as a molecular subtype by activation of the WNT pathway and TP53 mutations may be present or absent.


frontal lobe ependymal tumor

An ependymal tumor affecting the frontal lobe of the brain.


anaplastic ependymoma

Anaplastic ependymoma is a rare, malignant type of ependymoma that most often arises in the supratentorial region of the brain of children and young adults and that manifests with variable symptoms including headaches, nausea, vision impairment, memory loss and difficulty walking.


angiocentric glioma

Angiocentric glioma (AG) is an extremely rare slow-growing glial neoplasm of the central nervous system, usually arising in a superficial location in the cerebrum, affecting all ages and both sexes, and characterized by intractable seizures and headaches, with most cases being cured by surgical incision alone and therefore having a good prognosis.


cervical neuroblastoma

A relatively uncommon neuroblastoma that is found in the neck.


adrenal gland neuroblastoma

A neuroblastoma arising from the adrenal gland.


mediastinum neurofibroma

A neurofibroma that arises from the posterior mediastinum. Excision is usually curative.


cauda equina intradural extramedullary astrocytoma


nasal cavity olfactory neuroblastoma

An olfactory neuroblastoma arising in the nasal cavity.


corpus callosum oligodendroglioma

A oligodendroglioma that involves the corpus callosum.


infratentorial ependymal tumor

An ependymal tumor arising from the infratentorial region of the brain.


adult medulloblastoma

A medulloblastoma arising from the brain, occurring in adults.


malignant melanocytic neoplasm of the peripheral nerve sheath

A rare variant of malignant peripheral nerve sheath tumor. It is characterized by the presence of malignant cells that contain melanin.


anaplastic ganglioglioma

A WHO grade III neuroepithelial neoplasm composed of neoplastic, mature ganglion cells and anaplastic glial cells. The anaplastic changes in the glial component and high MIB-1 and TP53 labeling indices may indicate aggressive behavior. However, the correlation of histological anaplasia with clinical outcome is inconsistent. (Adapted from WHO)


mixed glioma

A tumor composed of two or more glial cell types (astrocytes, ependymal cells, and oligodendrocytes).


cerebellar liponeurocytoma

A rare, WHO grade II cerebellar neoplasm which shows consistent neuronal, variable astrocytic and focal lipomatous differentiation. It occurs in adults, has a low proliferative potential and usually has a favorable prognosis. (Adapted from WHO)


cutaneous ganglioneuroma

A ganglioneuroma arising from the skin.


adult spinal cord glioblastoma

A rare spinal tumor which is highly malignant and tends to be locally invasive of surrounding neural tissue. The tumor also tends to spread throughout the neuroaxis and is often rapidly progressive. Histologically the tumors are highly cellular with nuclear and cellular pleomorphism, endothelial proliferation, mitotic figures, and, often, necrosis. This tumor has a relatively poor prognosis. Clinical features may include pain followed by rapidly progressive neurologic deficits such as extremity weakness, sensory changes, spasticity, and incontinence. (From Innocenzi et al., Clin Neurol Neurosurg 1997 Feb;99(1):1-5)


central nervous system tumor with bcor internal tandem duplication

A central nervous system embryonal tumor that is characterized by the presence of uniform oval or spindle-shaped cells with round or oval nuclei, pseudorosette formation, and heterozygous internal tandem duplication in exon 15 of the BCOR gene.


parietal lobe ependymal tumor

An ependymal tumor affecting the parietal lobe of the brain.


schwannoma of ureter

A benign nerve sheath tumor composed of Schwann cells, occurring in the ureter.


mediastinum neuroblastoma

A neuroblastoma arising from the mediastinum.


childhood brain stem glioma

An abnormal growth of the cells that comprise the tissues of the brainstem. While the tumor may be histologically benign, it can produce great morbidity due to its location. It presents most commonly in the first two decades of life.


diencephalic astrocytomas

A astrocytoma that involves the diencephalon.


microcystic/reticular schwannoma

The rarest histopathologic subtype of Schwannoma. The reported cases have been located in the gastrointestinal submucosa or subcutaneous tissue. Morphologically it is characterized by the presence of a microcyst-rich network of spindle cells with minimal amount of cytoplasm and Antoni A tissue.


dopaminergic neuroblastoma

A neuroblastoma associated with increased dopamine excretion.


cellular neurofibroma

A neurofibroma characterized by the presence of areas with increased cellularity.


Desmoplastic infantile astrocytoma/ganglioglioma are mixed neuronal-glial tumors representing a histological spectrum of the same tumor. They are usually supratentorially located, large, cystic masses with a peripheral solid component, characterized by prominent desmoplastic stroma and pleomorphic populations of neoplastic cells with either astrocytic or ganglionic differentiation and poorly differentiated cells in variable proportions. They usually present in the first 18 months of age with rapid head growth, bulging anterior fontanel and bone structures over the tumor, signs of raised intracranial pressure (headache, vomiting, papilledema), focal neurological signs and sometimes seizures.


spinal cord ependymoma

An ependymoma that arises from the spinal cord.


pediatric infratentorial ependymoma

An ependymoma that arises from the infratentorial region of the brain and occurs during childhood.


cns neuroblastoma with FOXR2 activation

A central nervous system neuroblastoma that is characterized by FOXR2 activation and that is composed of small, round cells with hyperchromatic nuclei surrounded by a clear halo.


cutaneous granular cell tumor

A granular cell tumor that involves the zone of skin.


adult glioblastoma

A glioblastoma occurring in adults.


breast granular cell tumor

A usually benign neoplasm that arises from the breast. It presents as a single, firm, and painless mass. It is characterized by the presence of neoplastic cells with eosinophilic granular cytoplasm.


schwannomatosis

The least frequent form of the rare genetic disorder neurofibromatosis. It is clinically and genetically distinct from NF1 and NF2 and is characterized by the development of multiple schwannomas (nerve sheath tumors), without involvement of the vestibular nerves. NF3 develops in adulthood and is often associated with chronic pain. Dysesthesia and paresthesia may also be present. Common localizations include the spine, peripheral nerves, and the cranium.


supratentorial ependymoma

A high grade ependymoma that is located within the supratentorial brain.


adult central nervous system primitive neuroectodermal neoplasm

A central nervous system embryonal tumor, not otherwise specified that occurs in adults.


giant cell glioblastoma

A rare histological variant of glioblastoma (WHO grade IV) with a predominance of bizarre, multinucleated giant cells, an occasionally abundant stromal reticulin network, and a high frequency of TP53 mutations. (WHO)


pediatric supratentorial ependymoma

An ependymoma that arises from the supratentorial region of the brain and occurs during childhood.


childhood cerebral astrocytoma

An astrocytoma, without designation of benign or malignant, that is found in the supratentorial region. The infratentorial location is more common in children.


fibrillary astrocytoma

The most frequent histological variant of diffuse astrocytoma. It is predominantly composed of fibrillary neoplastic astrocytes. Nuclear atypia is a diagnostic criterion but mitotic activity, necrosis and microvascular proliferation are absent. The occasional or regional occurrence of gemistocytic neoplastic cells is compatible with the diagnosis of fibrillary astrocytoma. (WHO)


neurilemmoma of the pleura

A schwannoma that involves the pleura.


malignant glandular tumor of peripheral nerve sheath

A variant of malignant peripheral nerve sheath tumor characterized by the presence of glandular epithelium.


adult astrocytic tumor

An astrocytic tumor occurring during adulthood. Representative examples include diffuse astrocytoma, anaplastic astrocytoma, and glioblastoma.


childhood oligodendroglioma

An oligodendroglioma that arises from the central nervous system and occurs during childhood.


granular cell tumor

An unusual benign or malignant neoplasm characterized by the presence of neoplastic large polygonal cells with granular, eosinophilic cytoplasm which contains abundant lysosomes. It was originally thought to be a tumor originating from muscle cells and was named granular cell myoblastoma. Subsequent studies have suggested a derivation from Schwann cells. It affects females more often than males and it usually presents as a solitary mass. A minority of patients have multiple tumors. It can arise from many anatomic sites including the posterior pituitary gland, skin, oral cavity, esophagus, stomach, heart, mediastinum, and breast.


gliosarcoma

A rare histological variant of glioblastoma (WHO grade IV) characterized by a biphasic tissue pattern with alternating areas displaying glial and mesenchymal differentiation (WHO).


schwannoma of jugular foramen

A rare intracranial schwannoma that affects the jugular foramen.


brain astrocytoma

A astrocytoma (excluding glioblastoma) that involves the brain.


Grade 4 Retinoic Acid Syndrome, CTCAE

biolink:PhenotypicFeature biolink:DiseaseOrPhenotypicFeature biolink:BiologicalEntity biolink:NamedThing biolink:Entity biolink:ThingWithTaxon

Life-threatening consequences; ventilatory support indicated


Grade 3 Tumor Lysis Syndrome, CTCAE

Present


Grade 5 Tumor Lysis Syndrome, CTCAE

Death


Cytokine Release Syndrome or Acute Infusion Reaction, CTCAE


Grade 1 Retinoic Acid Syndrome, CTCAE

Fluid retention; <3 kg of weight gain; intervention with fluid restriction and/or diuretics indicated


Grade 3 Retinoic Acid Syndrome, CTCAE

Severe symptoms; hospitalization indicated


Grade 1 Cytokine Release Syndrome or Acute Infusion Reaction, CTCAE


Grade 2 Cytokine Release Syndrome or Acute Infusion Reaction, CTCAE


Grade 5 Cytokine Release Syndrome or Acute Infusion Reaction, CTCAE


Grade 4 Cytokine Release Syndrome or Acute Infusion Reaction, CTCAE


Grade 3 Alcohol Intolerance Antabuse-Like Syndrome, CTCAE


Alcohol Intolerance Antabuse-Like Syndrome, CTCAE


Grade 5 Alcohol Intolerance Antabuse-Like Syndrome, CTCAE


Grade 3 Cytokine Release Syndrome or Acute Infusion Reaction, CTCAE


Grade 5 Retinoic Acid Syndrome, CTCAE

Death


Grade 2 Retinoic Acid Syndrome, CTCAE

Moderate signs or symptoms; steroids indicated


Retinoic Acid Syndrome, CTCAE

A disorder characterized by weight gain, dyspnea, pleural and pericardial effusions, leukocytosis and/or renal failure originally described in patients treated with all-trans retinoic acid.


disulfide

Compounds of structure RSSR'.


organic disulfide

Compounds of structure RSSR in which R and R' are organic groups.


Recurrent Lung Neuroendocrine Neoplasm

The reemergence of a lung neuroendocrine neoplasm after a period of remission.


benign testicular sertoli cell tumor

A non-metastasizing sex cord-stromal tumor that arises from the testis. Morphologically, it is characterized by the presence of Sertoli cells forming tubules. Leydig cells are rare or absent.


testicular sertoli cell tumor

A sex cord-stromal tumor that arises from the testis and is characterized by the presence of neoplastic cells with features of Sertoli cells. It usually presents as a slow growing testicular mass. The vast majority of cases follow a benign clinical course.


Grade 4 Esophageal Varices Hemorrhage, CTCAE

Life-threatening consequences; urgent intervention indicated


Refractory Childhood Lymphoma

Childhood Hodgkin or non-Hodgkin lymphoma that is resistant to treatment.


Refractory Childhood Hodgkin Lymphoma

Hodgkin lymphoma that occurs during childhood and is resistant to treatment.


serine/threonine-protein kinase receptor R3 sequence variant L285F (human)

A serine/threonine-protein kinase receptor R3 (human) that has a Phe residue at the position equivalent to Leu-285 of the amino acid sequence represented by UniProtKB:P37023. UniProtKB:P37023, Leu-285, CHEBI:29997.


developmental and epileptic encephalopathy, 86


Tuberculous bronchopneumonia

Acute and progressive bronchopneumonia caused by Mycobacterium tuberculosis bacteria.  It is characterized by widespread pulmonary patchy consolidations.


Advanced HER2-Low Breast Carcinoma

HER2-low breast adenocarcinoma that has spread extensively to other anatomic sites or is no longer responding to treatment.


combined oxidative phosphorylation defect type 30

Any combined oxidative phosphorylation deficiency in which the cause of the disease is a mutation in the TRMT10C gene.


15-hydroxy steroid

A hydroxy steroid carrying a hydroxy group at position 15.


15alpha-hydroxy steroid

A 15-hydroxy steroid in which the hydroxy group at position 15 has an alpha-configuration.


dual specificity tyrosine-phosphorylation-regulated kinase 1A isoform 1 (human)

A dual specificity tyrosine-phosphorylation-regulated kinase 1A isoform 2 that is encoded in the genome of human.


dual specificity tyrosine-phosphorylation-regulated kinase 1A isoform Short (rat)

A dual specificity tyrosine-phosphorylation-regulated kinase 1A isoform 2 that is encoded in the genome of rat.


serine/threonine-protein kinase Nek10 isoform h1 (human)

A serine/threonine-protein kinase Nek10 (human) that is a translation product of some mRNA whose exon structure and start site selection renders it capable of giving rise to a protein with the amino acid sequence represented by UniProtKB:Q6ZWH5-1.


Ovarian Mucinous Cystic Tumor with Mural Nodules

A mucinous cystic tumor of the ovary characterized by the presence of one or more well circumscribed solid nodules in the wall of the cysts (mural nodules) that protrude into the lumen.  The mural nodules may be benign (sarcoma-like) or malignant.  The malignant mural nodules histologically are anaplastic carcinomas, carcinosarcomas, or sarcomas.  The clinical course depends on the histology of the mural nodules.  The prognosis of mucinous cystic tumors with benign mural nodules is the same as the corresponding types of mucinous cystic tumors without the mural nodules.  Mucinous cystic tumors with malignant mural nodules may follow a malignant clinical course.


Epithelioid sarcoma recurrent

The reemergence of epithelioid sarcoma after a period of remission


invasive hydatidiform mole

A complete hydatidiform mole or very rarely a partial mole that invades the myometrium.


placenta neoplasm

A neoplasm (disease) that involves the placenta.


Metastatic Neoplasm to the Placenta

A malignant neoplasm that has spread to the placenta from another anatomic site.


Placental polyp

A polyp in the uterine cavity that is formed by placenta remnants.


placental choriocarcinoma

Choriocarcinoma that develops in the placenta. It is the rarest form of gestational choriocarcinoma. Metastases to the mother and infant may occur.


benign neoplasm of placenta

A benign neoplasm that involves the placenta.


placenta cancer

A malignant neoplasm involving the placenta.


Metastatic Hydatidiform Mole

The spread of abnormal molar chorionic villi from an invasive hydatidiform mole in the uterine cavity to other anatomic sites, usually the vaginal wall and pelvis.


autosomal recessive limb-girdle muscular dystrophy type 2B

Autosomal recessive limb-girdle muscular dystrophy type 2B (LGMD2B) is a subtype of autosomal recessive limb-girdle muscular dystrophy characterized by an onset in late adolescence or early adulthood of slowly progressive, proximal weakness and atrophy of shoulder and pelvic girdle muscles. Cardiac and respiratory muscles are not involved. Hypertrophy of the calf muscles and highly elevated serum creatine kinase levels are frequently observed.


Blasts Under 10,000 of Peripheral Blood White Cells

A quantitative microscopic finding indicating that less than 10,000 of the total number of nucleated cells in a peripheral leukocyte sample are immature mononuclear cells.


suppurative lymphadenitis

A form of lymphadenitis that is characterized by formation of pus; it is most often caused by staphylococcal or streptococcal bacteria.


pericardium cancer

A malignant neoplasm involving the pericardium.


Pericardial Sarcoma

A rare sarcoma that arises from the pericardium. The two most common types are angiosarcoma and synovial sarcoma. Patients present with symptoms related to pericardial effusion.


Pericarditis malignant

Pericarditis caused by the infiltration of the pericardium by a malignant neoplasm.  The diagnosis is based on the cytological examination of pericardial fluid or the histologic examination of pericardial tissue.


Metastatic malignant neoplasm to pericardium

A malignant neoplasm that has spread to the pericardium from another primary anatomic site.


Pericardial mesothelioma malignant recurrent

The reemergence of malignant mesothelioma in the pericardium after a period of remission.


Pericardial Primary Effusion Lymphoma

Primary effusion lymphoma that affects the pericardial cavity. The majority of patients are HIV-positive men.


Pericardial Yolk Sac Tumor

A yolk sac tumor that arises within the pericardium.


Pericardial Carcinomatosis

Carcinoma that has spread diffusely to the pericardium.


Pericardial Synovial Sarcoma

A rare synovial sarcoma that arises from the pericardium. Patients present with symptoms related to pericardial effusion.


Malignant Pericardial Germ Cell Tumor

A malignant germ cell tumor that arises within the pericardium.


Pericardial Angiosarcoma

A rare angiosarcoma that arises from the pericardium. Patients present with symptoms related to pericardial effusion.


intellectual developmental disorder, X-linked, syndromic, Hackmann-Di Donato type


endogenous depression

Depression which is considered strictly biological.


A synthetic phenylalanine derivative consisting of a 1:1 diatereoisomeric mixture of ochratoxin A and its isochromenyl C-3 epimer.


Finding During Childbirth Labor

Symptom, signs, test results in a pregnant mother childbirth.


glycine receptor subunit alpha-3 isoform Alpha-3K (human)

A glycine receptor subunit alpha-3 isoform 2 that is encoded in the genome of human.


gamma-aminobutyric acid receptor subunit alpha-2 N-glycosylated 10 (rat)

A gamma-aminobutyric acid receptor subunit alpha-2 (rat) that has been N-glycosylated at the residue equivalent to Asn-138 of the amino acid sequence represented by UniProtKB:P23576-1, in which the glycan composition is represented by GlyTouCan:G62765YT. Example: UniProtKB:P23576-1, Asn-138, MOD:00160~GNO:G62765YT.


Bladder carcinoma stage I, without cancer in situ

Stage I includes: T1, N0, M0.  T1: Tumor invades subepithelial connective tissue. N0: No regional lymph node metastasis.  M0: No distant metastasis.  (AJCC 6th and 7th eds.)


Bladder Cancer by AJCC v7 Stage

A term that refers to the staging of bladder cancer according to the American Joint Committee on Cancer, 7th edition.


Bladder squamous cell carcinoma stage 0

Stage 0 includes: Tis, N0, M0.  Tis: Carcinoma in situ.  N0: No regional lymph node metastasis.  M0: No distant metastasis.  (AJCC 6th and 7th Eds.)


Bladder transitional cell carcinoma stage II

Stage II includes: (T2a, N0, M0); (T2b, N0, M0).  T2a: Tumor invades superficial muscle (inner half).  T2b: Tumor invades deep muscle (outer half).  N0: No regional lymph node metastasis.  M0: No distant metastasis.  (AJCC 6th and 7th Eds.)


Stage 0a Bladder Urothelial Carcinoma AJCC v6 and v7

Stage 0a includes: Ta, N0, M0. Ta: Papillary noninvasive carcinoma.  N0: No regional lymph node metastasis.  M0: No distant metastasis. (AJCC 6th and 7th eds.)


Bladder carcinoma stage I, with cancer in situ

Stage I bladder carcinoma associated with the presence of bladder carcinoma in situ.


Stage IV Bladder Cancer AJCC v7

Stage IV includes: (T4b, N0, M0); (Any T, N1-3, M0); (Any T, Any N, M1).  T4b: Tumor invades pelvic wall, abdominal wall.  N0: No regional lymph node metastasis.  N1: Single regional lymph node metastasis in the true pelvis (hypogastric, obturator, external iliac, or presacral lymph node).  N2: Multiple regional lymph node metastases in the true pelvis (hypogastric, obturator, external iliac, or presacral lymph node metastasis).  N3: Metastasis to the common iliac lymph nodes.  M0: No distant metastasis.  M1: Distant metastasis.  (AJCC 7th ed.)


Bladder transitional cell carcinoma stage III

Stage III includes: (T3a, N0, M0); (T3b, N0, M0); (T4a, N0, M0).  T3a: Tumor microscopically invades perivesical tissue.  T3b: Tumor macroscopically invades perivesical tissue (extravesical mass).  T4a: Tumor invades prostatic stroma, uterus, vagina.  N0: No regional lymph node metastasis.  M0: No distant metastasis.  (AJCC 6th and 7th eds.)


Bladder squamous cell carcinoma stage III


Bladder adenocarcinoma stage II

Stage II includes: (T2a, N0, M0); (T2b, N0, M0).  T2a: Tumor invades superficial muscle (inner half).  T2b: Tumor invades deep muscle (outer half).  N0: No regional lymph node metastasis.  M0: No distant metastasis. (AJCC 6th and 7th eds.)


Bladder transitional cell carcinoma stage IV


Bladder squamous cell carcinoma stage IV


Bladder transitional cell carcinoma stage I


Stage 0 Bladder Carcinoma

Stage 0 includes: 0a (Ta, N0, M0); 0is (Tis, N0, M0).  Ta: Noninvasive papillary carcinoma. Tis: Carcinoma in situ: "flat tumor".  N0: No regional lymph node metastasis.  M0: No distant metastasis.  (AJCC 6th and 7th Eds.)


Refractory Stage 0 Bladder Cancer AJCC v6 and v7

Stage 0 bladder cancer defined according to the AJCC v6 and v7 criteria that is resistant to treatment.


Bladder squamous cell carcinoma stage II


neuropeptide Y receptor

A rhodopsin-like G-protein coupled receptor that is a translation product of the human NPY1R, NPY2R, or NPY4R to NPY6R genes, 1:1 orthologs thereof, or a semi-ortholog thereof.


neuropeptide Y receptor type 2

A neuropeptide Y receptor that is a translation product of the human NPY2R gene or a 1:1 ortholog thereof.


Advanced Undifferentiated Pleomorphic Sarcoma

An undifferentiated pleomorphic sarcoma that has spread extensively to other anatomic sites or is no longer responding to treatment.


solute carrier family 22 member 1 isoform h2 (human)

A solute carrier family 22 member 1 (human) that is a translation product of some mRNA whose exon structure and start site selection renders it capable of giving rise to a protein with the amino acid sequence represented by UniProtKB:O15245-2.


interleukin-1 receptor accessory protein

A protein that is a translation product of the human IL1RAP gene or a 1:1 ortholog thereof.


interleukin-1 receptor accessory protein isoform h3 (human)

An interleukin-1 receptor accessory protein (human) that is a translation product of some mRNA whose exon structure and start site selection renders it capable of giving rise to a protein with the amino acid sequence represented by UniProtKB:Q9NPH3-3.


interleukin-1 receptor accessory protein isoform h4 (human)

An interleukin-1 receptor accessory protein (human) that is a translation product of some mRNA whose exon structure and start site selection renders it capable of giving rise to a protein with the amino acid sequence represented by UniProtKB:Q9NPH3-5.


interleukin-1 receptor accessory protein isoform 1 (human)

An interleukin-1 receptor accessory protein isoform 1 that is encoded in the genome of human.


interleukin-1 receptor accessory protein isoform h2 (human)

An interleukin-1 receptor accessory protein (human) that is a translation product of some mRNA whose exon structure and start site selection renders it capable of giving rise to a protein with the amino acid sequence represented by UniProtKB:Q9NPH3-2.


Grade 2 Spermatic Cord Perforation, CTCAE


Superficial injury of abdomen, lower back and pelvis, part unspecified

An injury to the soft tissues overlying the abdomen, lower back or pelvis.


CAAX prenyl protease 2

A protein that is a translation product of the human RCE1 gene or a 1:1 ortholog thereof.


CAAX prenyl protease 2, initiator methionine removed form (human)

A CAAX prenyl protease 2 (human) that has had the initiator methionine removed. UniProtKB:Q9Y256, 2-329.


CAAX prenyl protease 2, initiator methionine removed ubiquitinated form (human)

A CAAX prenyl protease 2 (human) that has had the initiator methionine removed and that includes at least one ubiquitinated lysine. UniProtKB:Q9Y256, 2-329, MOD:01148.


CAAX prenyl protease 2 isoform 1 (human)

A CAAX prenyl protease 2 isoform 1 that is encoded in the genome of human.


funisitis

An acute inflammation of the umbilical cord. It is characterized by the presence of polymorphonuclear cells migrating from the fetal umbilical cord vessels through the umbilical cord towards the bacteria containing amniotic fluid.


Grade 2 Renal Colic, CTCAE

Moderate pain; limiting instrumental ADL; prescription medication indicated


Grade 3 Eczema, CTCAE

Severe or medically significant but not immediately life-threatening; IV intervention indicated


Non-Functioning Pancreatic Neuroendocrine Tumor G2

An intermediate grade well differentiated neoplasm with neuroendocrine differentiation that arises from the pancreas. It is characterized by the absence of a hormone-related clinical syndrome.


Pancreatic Neuroendocrine Tumor G2

An intermediate grade well differentiated neoplasm with neuroendocrine differentiation that arises from the pancreas. The mitotic count is 2-20 per 10 HPF and/or the Ki67 index is 3-20%.


Functioning Pancreatic Neuroendocrine Tumor G2

An intermediate grade well differentiated neoplasm with neuroendocrine differentiation that arises from the pancreas. It is characterized by the presence of a clinical syndrome that results from hormone hypersecretion.


Grade 2 Nose Infection Documented Clinically or Microbiologically with Grade 3 or 4 Neutrophils, CTCAE


autoimmune disorder of endocrine system

A hypersensitivity reaction type II disease that involves the endocrine system.


adenohypophysitis

An autoimmune disease of the pituitary gland which can present with varying degrees of pituitary hormonal impairment and/or with symptoms related to pituitary enlargement. It predominantly affects young women in pregnancy or the peripartum period.


autoimmune oophoritis

Autoimmune oophoritis is a rare cause of primary ovarian insufficiency (POI). It happens when the body's immune system mistakenly attacks the ovaries causing inflammation, atrophy and fibrosis. These changes stop the ovaries from working normally. The main symptoms of autoimmune oophorotis are irregular or absent menstrual period (amenorrhea) and symptoms related to ovarian cysts such as abdominal cramping, bloating, nausea and vomiting. Autoimmune oophoritis may occur as part of autoimmune polyglandular syndrome type I and type II but has also been associated with lupus, pernicious anemia, myasthenia gravis and other autoimmune conditions. The underlying cause of autoimmune oophoritis is unknown. Diagnosis involves a special blood test which looks for anti-steroid or anti-ovarian antibodies, a pelvic ultrasound to look for enlarged cystic ovaries and tests to rule out other possible causes of POI. Management of autoimmune oophoritis involves emotional support, possible estrogen replacement therapy and management of other autoimmune conditions.


atrophic thyroiditis

Atrophic thyroiditis is an organ-specific autoimmune disease characterized by thyroid autoantibodies, functional hypothyroidism, and absence of goiter.


primary hypophysitis

Immune-mediated inflammation of the pituitary gland often associated with other autoimmune diseases (e.g., hashimoto disease; graves disease; and addison disease).


autoimmune primary ovarian failure

An autoimmune form of primary ovarian failure.


postpartum thyroiditis

A transient autoimmune Inflammatory disorder of thyroid gland that occurs postpartum due to any partum problem. It is characterized by the presence of high titers of autoantibodies against thyroid peroxidase and thyroglobulin. Clinical signs include the triphasic thyroid hormone pattern: beginning with thyrotoxicosis, followed with hypothyroidism, then return to euthyroid state by 1 year postpartum


IgG4-related thyroid disease

Riedel thyroiditis is a fibroinflammatory disorder of the thyroid gland, occuring more frequently in females, characterized a large, hard thyroid mass, and presenting with pressure symptoms (breathing difficulB,ties and dysphagia) or voice hoarseness and aphonia (impingement of recurrent laryngeal nerve). It can often be associated with extracervical fibroinflammatory disorders such as retroperitoneal fibrosis, primary scleroisng cholangitis and autoimmune diseases such as Hashimoto struma, Addison disease, and Biermer disease.


autoimmune polyendocrinopathy

A group of diverse conditions that are characterized by spontaneous, multi-organ autoimmunity, which target both endocrine (adrenal, gonad, pancreatic islet cells, parathyroid, pituitary, thyroid) and non-endocrine (gastrointestinal, integumentary, lymphatic) tissues.


autoimmune polyendocrinopathy type 2

Autoimmune polyglandular syndrome of likely polygenic etiology characterized by the presence of primary adrenal insufficiency in association with autoimmune thyroiditis and/or type 1 diabetes mellitus; this condition is not associated with mucocutaneous candidiasis.


autoimmune polyendocrine syndrome type 1

Autoimmune polyendocrinopathy type 1, or APECED syndrome, is a genetic disease that manifests in childhood or early adolescence with a combination of chronic mucocutaneous candidiasis, hypoparathyroidism and autoimmune adrenal failure.


Abediterol

A quinolone that is 8-hydroxyquinolin-2(1H)-one which carries a 2-{[6-(2,2-difluoro-2-phenylethoxy)hexyl]amino}-1-hydroxyethyl group at position 5. It is a long acting beta2-adrenoceptor agonist currently in development for the treatment of chronic obstructive pulmonary disease (COPD) and asthma.


AV Block First Degree by ECG Finding

An electrocardiographic finding of prolonged PR interval for a specific population. For adults one common threshold is a PR interval greater than 0.20 seconds. Note that other thresholds may be applicable. (CDISC)


parathyroid hormone/parathyroid hormone-related peptide receptor

A parathyroid hormone receptor that is a translation product of the human PTH1R gene or a 1:1 ortholog thereof.


parathyroid hormone receptor

A secretin-like G-protein coupled receptor that is a translation product of the human PTH1R or PTH2R genes, 1:1 orthologs thereof, or a semi-ortholog thereof.


parathyroid hormone 2 receptor

A parathyroid hormone receptor that is a translation product of the human PTH2R gene or a 1:1 ortholog thereof.


parathyroid hormone 2 receptor, signal peptide removed form (human)

A parathyroid hormone 2 receptor (human) that has had the signal peptide removed. UniProtKB:P49190, 25-550.


parathyroid hormone/parathyroid hormone-related peptide receptor isoform 1 (rat)

A parathyroid hormone/parathyroid hormone-related peptide receptor isoform 1 that is encoded in the genome of rat.


parathyroid hormone/parathyroid hormone-related peptide receptor, signal peptide removed form (human)

A parathyroid hormone/parathyroid hormone-related peptide receptor (human) that has had the signal peptide removed. UniProtKB:Q03431, 27-593.


Unresectable NUT Carcinoma

NUT carcinoma that is not amenable to surgical resection.


A53 Diffuse Large B-Cell Lymphoma

A genetic subtype of diffuse large B-cell lymphoma characterized by TP53 gene mutations and deletions that is often accompanied by aneuploidy.


spindle cell nevus

A nevus characterized by the presence of spindle-shaped melanocytes.


Childhood Recurrent Malignant Solid Neoplasm

The reemergence of a childhood malignant solid neoplasm after a period of remission.


amelogenesis imperfecta type 2A1

Any amelogenesis imperfecta in which the cause of the disease is a mutation in the KLK4 gene.


Pelvic Floor Muscle Weakness

A reduction in the strength of the muscles of the pelvic floor.


Grade 1 Skin Papilloma, CTCAE

Asymptomatic; intervention not indicated


specific developmental disorder

A developmental disorder of mental health that categorizes specific learning disabilities and developmental disorders affecting coordination.


conduct disorder

A disorder diagnosed in childhood or adolescence age group characterized by aggressive behavior, deceitfulness, destruction of property or violation of rules that is persistent and repetitive, and within a one year period.


attention deficit-hyperactivity disorder 8


specific language impairment

A language disorder characterized by difficulty in language acquisition despite otherwise normal development and in the absence of any obvious explanatory factors.


fetal alcohol syndrome

Fetal alcohol syndrome (FAS) is a rare malformation syndrome caused by excessive maternal consumption of alcohol during pregnancy. It is characterized by prenatal and/or postnatal growth deficiency (weight and/or height <10th percentile), a unique cluster of minor facial anomalies (short palpebral fissures, flat and smooth philtrum, and thin upper lip) and severe central nervous system (CNS) abnormalities including microcephaly, and cognitive and behavioral impairment (intellectual disability, deficit in general cognition, learning and language, executive function, visual-spatial processing, memory, and attention).


mixed receptive-expressive language disorder

A disorder characterized by an impairment in the development of an individual's expressive and receptive language capabilities which is in contrast to his/her nonverbal intellect. The impairment may be acquired (i.e., due to a brain lesion or head trauma) or developmental (i.e., no known neurological insult).


transient tic disorder

A neurological disorder presenting in childhood that is characterized by motor and/or phonic tics that occur daily or nearly daily for one to twelve months and are not attributed to an identifiable cause.


articulation disorder

A disorder characterized by the failure to use developmentally expected speech sounds that are appropriate for the individual's age (i.e., the individual makes errors in sound production or use or omits sounds such as final consonants).


partial fetal alcohol syndrome

A fetal alcohol spectrum disorder that results in most, but not all, of the growth deficiency and/or craniofacial features of fetal alcohol syndrome including central nervous system dysfunction due to prenatal alcohol exposure.


dysgraphia

Loss or impairment of the ability to write (letters, syllables, words, or phrases) due to an injury to a specific cerebral area or occasionally due to emotional factors. This condition rarely occurs in isolation, and often accompanies aphasia. (From Adams et al., Principles of Neurology, 6th ed, p485; apa, Thesaurus of Psychological Index Terms, 1994)


specific language impairment 5

A communication disorder that involves the processing of linguistic information.


dyslexia

A learning disorder characterized by an impairment in processing written words. Reading difficulties can include distortions, omissions or substitutions of characters. Oral and silent reading difficulties can include faulty and slow comprehension.


A disorder characterized by a marked pattern of inattention and/or hyperactivity-impulsivity that is inconsistent with developmental level and clearly interferes with functioning in at least two settings (e.g. at home and at school). When present, the symptoms of hyperactivity are most often present before the age of 7 years. There are three recognized presentations or subtypes from most to least common: combined type, inattentive/distractible type, hyperactive/impulsive type.


fetal nicotine spectrum disorder

A specific developmental disorder that is characterized by physical, behavioral and learning birth defects resulting from maternal ingestion of nicotine during pregnancy.


writing disorder

A learning disability that involves impaired written language ability such as impairments in handwriting, spelling, organization of ideas, and composition.


expressive language disorder

A disorder characterized by an impairment in the development of an individual's expressive language which is in contrast to his/her nonverbal intellect and receptive language development. The impairment may be acquired (i.e., due to a brain lesion or head trauma) or developmental (i.e., no known neurological insult).


chronic tic disorder

A neurological disorder presenting in childhood that is characterized by either motor or phonic tics, but not both, that occur daily or nearly daily for at least a year and are not attributed to an identifiable cause.


alcohol-related birth defect

A physical or cognitive mental abnormality caused by maternal alcohol consumption and its toxic effect on the developing embryo during pregnancy.


fetal alcohol spectrum disorder

A group of disorders caused by a prenatal exposure to maternal consumption of alcohol leading to a range of behavioral, cognitive and neurological deficits in the offspring. It is characterized by physical growth problems, distinct facies, and varying psycho-neurological issues.


hereditary attention deficit-hyperactivity disorder

An instance of attention deficit-hyperactivity disorder that is caused by an inherited genomic modification in an individual.


neurobehavioral disorder with prenatal alcohol exposure

A fetal alcohol spectrum disorder that is characterized by one or more deficits in neurocognition and in self-regulation plus two or more deficits in adaptive functioning, with at least 1 in communication or social communication and interaction.


high affinity immunoglobulin gamma Fc receptor I isoform h2 (human)

A high affinity immunoglobulin gamma Fc receptor I (human) that is a translation product of some mRNA whose exon structure and start site selection renders it capable of giving rise to a protein with the amino acid sequence represented by UniProtKB:P12314-2.


Testicular Mixed Embryonal Carcinoma and Yolk Sac Tumor with Seminoma

A malignant germ cell tumor arising from the testis.  It is characterized by a mixture of embryonal carcinoma, yolk sac, and seminomatous morphologic elements.  Patients may present with painless or painful testicular swelling.


Lugano Classification Advanced Stage Adult Non-Hodgkin Lymphoma AJCC v8

Advanced stage adult non-Hodgkin lymphoma based on the Lugano classification criteria. It includes stages III and IV. (from AJCC 8th Ed.)


Advanced Non-Hodgkin Lymphoma

Non-Hodgkin lymphoma that has spread extensively to other anatomic sites or is no longer responding to treatment.


Stage I Diffuse Large B-Cell Lymphoma

Ann Arbor Classification: Stage I: Involvement of a single lymph node region (I); or localized involvement of a single extralymphatic organ or site in the absence of any lymph node involvement (IE).


Helicobacter Pylori-Associated Gastric Mucosa-Associated Lymphoid Tissue Lymphoma

A low grade, indolent B-cell lymphoma that is associated with Helicobacter pylori infection.


Intestinal T-cell lymphoma recurrent

The reemergence of enteropathy-associated T-cell lymphoma after a period of remission.


Adult T-Cell Leukemia/Lymphoma by Ann Arbor Stage

An anatomic stage for adult T-cell leukemia/lymphoma based on the Ann Arbor classification criteria.


Mycosis Fungoides and Sezary Syndrome by AJCC v7 Stage

A term that refers to the staging of mycosis fungoides and Sezary syndrome, following the rules of the TNM AJCC v7 classification system.


Primary Cutaneous B-Cell Non-Hodgkin Lymphoma

A B-cell non-Hodgkin lymphoma arising from the skin.  Representative examples include cutaneous marginal zone B-cell lymphoma and cutaneous follicle center lymphoma.


Refractory EBV-Positive Diffuse Large B-Cell Lymphoma, Not Otherwise Specified

EBV-positive diffuse large B-cell lymphoma, not otherwise specified that is resistant to treatment.


Central Nervous System Anaplastic Large Cell Lymphoma, ALK-Negative

An anaplastic large cell lymphoma, ALK-negative, arising from the central nervous system.


Stage IV Childhood Lymphoblastic Lymphoma

Stage IV: Tumors involve bone marrow and/or central nervous system (CNS) regardless of other sites of involvement (from PDQ).


Stage IVA1 Mycosis Fungoides and Sezary Syndrome AJCC v8

Stage IVA1 includes: T1-4, N0-2, M0, B2. T1: Limited patches, papules, and/or plaques covering less than 10% of the skin surface. T2: Patches, papules, or plaques covering 10% or more of the skin surface. T3: One or more tumors (equal or greater than 1 cm in diameter). T4: Confluence of erythema covering 80% or more of body surface area. N0: No clinically abnormal peripheral lymph nodes; biopsy not required. N1: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 1 or National Cancer Institute (NCI) LN0-2. N2: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3. M0: No visceral organ involvement. B2: High blood tumor burden: 1,000 per microliter or more Sezary cells with positive clone. (AJCC 8th ed.)


Adult Burkitt Lymphoma

A Burkitt lymphoma occurring in adults.


Advanced Follicular Lymphoma

Follicular lymphoma that has spread extensively to other anatomic sites or is no longer responding to treatment.


Recurrent Transformed Mycosis Fungoides

The reemergence of transformed mycosis fungoides after a period of remission.


Grade 1 Cutaneous Follicular Lymphoma

A cutaneous lymphoma which contains up to 5 centroblasts per 40X high-power microscopic field.


Recurrent Diffuse Large B-Cell Lymphoma Associated with Chronic Inflammation

The reemergence of diffuse large B-cell lymphoma associated with chronic inflammation after a period of remission.


Primary mediastinal large B-cell lymphoma stage IV

Ann Arbor Classification: Stage IV: Diffuse or disseminated involvement of one or more extralymphatic organs, with or without associated lymph node involvement; or isolated extralymphatic organ involvement in the absence of adjacent regional lymph node involvement, but in conjunction with disease in distant site(s); or any involvement of the liver or bone marrow, lungs (other than by direct extension from another site), or cerebrospinal fluid.


Centroblastic lymphoma stage II

Ann Arbor Classification: Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II); or localized involvement of a single extralymphatic organ or site in association with regional lymph node involvement with or without involvement of other lymph node regions on the same side of the diaphragm (IIE). The number of regions involved may be indicated by a subscript (e.g., II3).


Recurrent Breast Implant-Associated Anaplastic Large Cell Lymphoma

The reemergence of breast implant-associated anaplastic large cell lymphoma after a period of remission.


Primary Cutaneous Non-Hodgkin Lymphoma

A B-or T-cell non-Hodgkin lymphoma arising in the skin.


Lugano Classification Stage II Adult Non-Hodgkin Lymphoma AJCC v8

Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm. (from AJCC 8th Ed.)


Lung Intravascular Large B-Cell Lymphoma

An aggressive extranodal B-cell non-Hodgkin lymphoma that affects the lung.  It is characterized by the presence of large neoplastic lymphocytes exclusively in the lumina of small vessels, particularly capillaries.


Transformed Mycosis Fungoides

Histologic transformation of mycosis fungoides to an aggressive non-Hodgkin lymphoma.


Adult Lymphoblastic Lymphoma

A lymphoblastic lymphoma occurring in adults.


Mycosis fungoides/Sezary syndrome stage I

Stage I includes: IA (T1, N0, M0, B0-1); IB (T2, N0, M0, B0-1). T1: Limited patches, papules, and/or plaques covering less than 10% of the skin surface.  May further stratify into T1a (patch only) vs. T1b (plaque +/- patch).  T2: Patches, papules, or plaques covering 10% or more of the skin surface.  May further stratify into T2a (patch only) vs. T2b (plaque +/- patch).  N0: No clinically abnormal peripheral lymph nodes; biopsy not required.  M0: No visceral organ involvement.  B0: Absence of significant blood involvement: 5% or less of peripheral blood lymphocytes are atypical (Sezary cells).  B1: Low blood tumor burden: more than 5% of peripheral blood lymphocytes are atypical (Sezary cells) but does not meet the criteria of B2.  (AJCC 7th ed.)


Ann Arbor Stage II Subdiaphragmatic Nodular Lymphocyte Predominant Hodgkin Lymphoma


Refractory High Grade B-Cell Lymphoma with MYC, BCL2, and BCL6 Rearrangements

High grade B-cell lymphoma with MYC, BCL2, and BCL6 rearrangements that is resistant to treatment.


Ann Arbor Stage IV Grade 2 Follicular Lymphoma


grade 3A follicular lymphoma

A grade 3 follicular lymphoma in which centrocytes are present.


Refractory Grade 1 Follicular Lymphoma

Grade 1 follicular lymphoma that is resistant to treatment.


Refractory Primary Vitreoretinal Non-Hodgkin Lymphoma

Primary vitreoretinal non-Hodgkin lymphoma that does not respond to treatment.


Recurrent Adult Immunoblastic Lymphoma

The reemergence of adult immunoblastic lymphoma after a period of remission.


Follicle center lymphoma, follicular grade I, II, III stage IV


Childhood Primary Cutaneous Anaplastic Large Cell Lymphoma

An anaplastic large cell lymphoma that occurs during childhood and is limited to the skin at the time of diagnosis.


Follicular lymphoma stage I

Ann Arbor Classification: Stage I: Involvement of a single lymph node region (I); or localized involvement of a single extralymphatic organ or site in the absence of any lymph node involvement (IE).


Colorectal High Grade B-Cell Lymphoma, Not Otherwise Specified

A colorectal high grade B-cell lymphoma with blastoid features or features between diffuse large B-cell lymphoma and Burkitt lymphoma which lacks MYC, BCL2, and BCL6 rearrangements.


Lymphoplasmacytic lymphoma resistant to treatment.


Indolent T-Cell Non-Hodgkin Lymphoma

A T-cell non-Hodgkin lymphoma with an indolent clinical course.


Precursor B-lymphoblastic lymphoma stage II

Ann Arbor Classification: Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II); or localized involvement of a single extralymphatic organ or site in association with regional lymph node involvement with or without involvement of other lymph node regions on the same side of the diaphragm (IIE).


non-Hodgkin's lymphoma of spleen

Non-Hodgkin lymphoma involving the spleen.


Primary Ciliary Body Non-Hodgkin Lymphoma

An extremely rare low-grade B-cell non-Hodgkin lymphoma that arises from the ciliary body. It is characterized by the presence of a diffuse infiltrate of small, round lymphocytes. Most cases represent extranodal marginal zone lymphomas of mucosa-associated lymphoid tissue.


Ann Arbor Stage II Grade 3 Follicular Lymphoma


Cutaneous Nasal Type Extranodal NK/T-Cell Lymphoma

Nasal type extranodal NK/T-cell lymphoma affecting the skin.


Methotrexate-Associated Diffuse Large B-Cell Lymphoma

A diffuse large B-cell lymphoma that develops in patients who are immunosuppressed with methotrexate.


Stage IIIA Mycosis Fungoides and Sezary Syndrome AJCC v7

Stage IIIA includes: T4, N0-2, M0, B0.  T4: Confluence of erythema covering 80% or more of body surface area.  N0: No clinically abnormal peripheral lymph nodes; biopsy not required.  N1: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 1 or NCI LN0-2.  N2: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3.  M0: No visceral organ involvement.  B0: Absence of significant blood involvement: 5% or less of peripheral blood lymphocytes are atypical (Sezary cells).  (AJCC 7th ed.)


Recurrent Primary Cutaneous Anaplastic Large Cell Lymphoma

The reemergence of primary cutaneous anaplastic large cell lymphoma after a period of remission.


Tonsillar Diffuse Large B-Cell Lymphoma

Diffuse large B-cell lymphoma that affects the tonsil and the bulk of the tumor is in this anatomic area.


Stage IVA Mycosis Fungoides and Sezary Syndrome AJCC v7

Stage IVA includes: IVA1 (T1-4, N0-2, M0, B2); IVA2 (T1-4, N3, M0, B0-2). T1: Limited patches, papules, and/or plaques covering less than 10% of the skin surface.  May further stratify into T1a (patch only) vs. T1b (plaque +/- patch).  T2: Patches, papules, or plaques covering 10% or more of the skin surface.  May further stratify into T2a (patch only) vs. T2b (plaque +/- patch).  T3: One or more tumors (equal or greater than 1 cm diameter).  T4: Confluence of erythema covering 80% or more of body surface area.  N0: No clinically abnormal peripheral lymph nodes; biopsy not required.  N1: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 1 or NCI LN0-2.  N2: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3.  N3: Clinically abnormal peripheral lymph nodes; histopathology Dutch grades 3-4 or NCI LN4; clone positive or negative.  M0: No visceral organ involvement.  B0: Absence of significant blood involvement: 5% or less of peripheral blood lymphocytes are atypical (Sezary cells).  B1: Low blood tumor burden: more than 5% of peripheral blood lymphocytes are atypical (Sezary cells) but does not meet the criteria of B2.  B2: High blood tumor burden: 1000/microL Sezary cells or more with positive clone.  (AJCC 7th ed.)


Burkitt's lymphoma refractory

Burkitt lymphoma that is resistant to treatment.


Extranodal Non-Hodgkin Lymphoma

A non-Hodgkin lymphoma that arises from an anatomic site other than a lymph node.


T-cell childhood lymphoblastic lymphoma

A T lymphoblastic lymphoma that occurs during childhood.


Mycosis fungoides/Sezary syndrome

Mature T and NK neoplasms predominantly affecting the skin and the peripheral blood.  Peripheral blood involvement by abnormal T-cells (cerebriform cells) is a late manifestation of mycosis fungoides, whereas it is the presenting finding in Sezary syndrome.


Localized Anaplastic Large Cell Lymphoma

Anaplastic large cell lymphoma that is confined to a specific site without evidence of spread to other anatomic sites.


Conjunctival Diffuse Large B-Cell Lymphoma

A diffuse large B-cell lymphoma that arises from the conjunctiva.


B-Cell Non-Hodgkin Lymphoma by Ann Arbor Stage

An anatomic stage for B-cell non-Hodgkin lymphoma based on the Ann Arbor classification criteria.


non-Hodgkin's lymphoma of accessory sinus

A non-Hodgkin lymphoma that arises from the paranasal sinus.  Representative examples include diffuse large B-cell lymphoma and extranodal NK/T-cell lymphoma, nasal type.


Cecum Non-Hodgkin Lymphoma

An extranodal non-Hodgkin lymphoma that arises from the cecum.  The majority are B-cell non-Hodgkin lymphomas.


Cutaneous Follicular Lymphoma

A follicular lymphoma involving the skin.  A cutaneous follicular lymphoma may be metastatic to the skin from the lymph nodes or other anatomic sites or primary (cutaneous follicle center lymphoma).


Stage III Childhood Non-Hodgkin Lymphoma

Ann Arbor Classification: Stage III: Involvement of lymph node regions on both sides of the diaphragm (III), which also may be accompanied by extralymphatic extension in association with adjacent lymph node involvement (IIIE) or by involvement of the spleen (IIIS) or both (IIIE,S).


systemic Epstein-Barr virus-positive T-cell lymphoproliferative disease of childhood

Systemic Epstein-Barr virus (EBV)-positive T-cell lymphoproliferative disease of childhood is a rare and very aggressive neoplastic disease emerging after a primary acute or chronic active EBV infection. It presents with persisting fever and malaise, hepatosplenomegaly with or without lymphadenopathy, liver failure, severe pancytopenia and a rapid progression towards multi-organ failure and hemophagocytic syndrome with a fatal issue. It is characterized by clonal proliferation of EBV-infected T cells with an activated cytotoxic phenotype.


Systemic Anaplastic Large Cell Lymphoma

An anaplastic large cell lymphoma that is not confined to a single anatomic site and involves multiple nodal and/or extranodal sites.


Lacrimal System Diffuse Large B-Cell Lymphoma

A diffuse large B-cell lymphoma that arises from the lacrimal gland or the lacrimal drainage system.


Primary mediastinal large B-cell lymphoma recurrent

The reemergence of primary mediastinal (thymic) large B-cell lymphoma after a period of remission.


Stage II Adult Burkitt Lymphoma

Ann Arbor Classification: Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II); or localized involvement of a single extralymphatic organ or site in association with regional lymph node involvement with or without involvement of other lymph node regions on the same side of the diaphragm (IIE).


Marginal zone lymphoma recurrent

The reemergence of marginal zone lymphoma after a period of remission.


Ann Arbor Stage IIX (Bulky) Diffuse Large B-Cell Lymphoma

Ann Arbor Stage II diffuse large B-cell lymphoma with a mass of nodes with one diameter more than 10cm or a mediastinal mass larger than one third of the transthoracic width. (Cotswolds modification)


Transformed Marginal Zone Lymphoma to Diffuse Large B-Cell Lymphoma

Histologic transformation of a marginal zone lymphoma to an aggressive diffuse large B-cell lymphoma.


Cutaneous Angioimmunoblastic T-Cell Lymphoma

An aggressive mature T-cell non Hodgkin lymphoma involving the skin. It is characterized by the presence of a polymorphic cellular infiltrate which contains neoplastic small to medium sized T-lymphocytes.


Diffuse follicle center lymphoma

A lymphoma with the morphologic and immunophenotypic features of a follicular lymphoma that shows entirely a diffuse pattern.  It is usually seen in small biopsy specimens and most probably represents a diffuse area of a follicular lymphoma.


Stage I Adult Immunoblastic Lymphoma

Ann Arbor Classification: Stage I: Involvement of a single lymph node region (I); or localized involvement of a single extralymphatic organ or site in the absence of any lymph node involvement (IE).


Intestinal T-cell lymphoma refractory

Enteropathy-associated T-cell lymphoma that is resistant to treatment.


Colorectal Mucosa-Associated Lymphoid Tissue Lymphoma

An extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue that arises from the colon or rectum.


Ann Arbor Stage II Non-Contiguous Adult Non-Hodgkin Lymphoma

Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II) or localized involvement of a single associated extralymphatic organ or site and its regional lymph nodes with or without other lymph node regions on the same side of the diaphragm (IIE). Note: The number of lymph node regions involved may be indicated by a subscript (e.g., II3). (from PDQ)


Refractory Aggressive B-Cell Non-Hodgkin Lymphoma

An aggressive B-cell non-Hodgkin lymphoma that is resistant to treatment.


Recurrent Aggressive T-Cell Non-Hodgkin Lymphoma

The reemergence of an aggressive T-cell non-Hodgkin lymphoma after a period of remission.


Primary Vitreoretinal Diffuse Large B-Cell Lymphoma

An uncommon and potentially fatal diffuse large B-cell lymphoma that involves the uvea, retina, vitreous body, and optic nerve. It is a subset of primary central nervous system diffuse large B-cell lymphoma.


Stage II Non-Hodgkin Lymphoma

Ann Arbor Classification: Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II); or localized involvement of a single extralymphatic organ or site in association with regional lymph node involvement with or without involvement of other lymph node regions on the same side of the diaphragm (IIE). The number of regions involved may be indicated by a subscript (e.g., II3).


Primary cutaneous diffuse large cell B-cell lymphoma

A diffuse large B-cell lymphoma that arises from the skin. This category includes primary cutaneous diffuse large B-cell lymphoma, leg type, and primary cutaneous diffuse large B-cell lymphoma, other, which includes primary cutaneous intravascular large B-cell lymphoma and primary cutaneous T-cell/histiocyte-rich large B-cell lymphoma.


B-cell small lymphocytic lymphoma stage I


mantle cell lymphoma of small intestine

A mantle cell lymphoma that arises from the small intestine.  It may present as an isolated mass in the small intestine or as a part of the multiple lymphomatous polyposis.


Refractory T Lymphoblastic Lymphoma

T-lymphoblastic lymphoma that does not respond to treatment.


Recurrent Grade III Lymphomatoid Granulomatosis


Childhood Systemic Anaplastic Large Cell Lymphoma

A systemic anaplastic large cell lymphoma that occurs during childhood.


Pituitary Gland Non-Hodgkin Lymphoma

A rare non-Hodgkin lymphoma that arises from the pituitary gland. The majority are diffuse large B-cell lymphomas.


Parotid Gland Follicular Lymphoma

A follicular lymphoma that manifests initially in the parotid gland.


Refractory High Grade B-Cell Lymphoma

High grade B-cell lymphoma that is resistant to treatment.


Primary Systemic Anaplastic Large Cell Lymphoma, ALK-Negative

A CD30 (Ki-1)-positive anaplastic large cell lymphoma with cytoplasmic and/or nuclear expression of the ALK (anaplastic lymphoma kinase) protein.  It usually affects both the lymph nodes and extranodal sites (primarily skin, bones, lungs, and soft tissues), and has been associated with a favorable clinical outcome.


Burkitt lymphoma associated with AIDS

A Burkitt lymphoma occurring in HIV-positive patients.


MCD diffuse large B-cell lymphoma

A diffuse large B-cell lymphoma that is categorized as MCD with high probability by the LymphGen algorithm. This is based on a combination of genetic features and MCD DLBCLs often, but do not always, have the most common hot spot mutation in MYD88 (L265P) and/or activating mutations in CD79B. This subgroup also commonly has mutations due to aberrant somatic hypermutation affecting PIM1 and/or ETV6, which can be coding or non-coding.


Childhood Mature T-Cell and NK-Cell Non-Hodgkin Lymphoma

A mature T- and NK-cell non-Hodgkin lymphoma that occurs during childhood.


primary cutaneous diffuse large B-cell lymphoma, Leg type

An aggressive primary cutaneous B-cell lymphoma, usually involving the lower leg. It is composed of a generally monotonous proliferation of immunoblasts, or less frequently centroblasts, with few admixed reactive cells. This type of lymphoma occurs most often in elderly women who present with rapidly growing tumors, usually on one or both legs. Dissemination to extracutaneous sites is frequent. Treatment with combination chemotherapy is usually required.


Stage IV Childhood Anaplastic Large Cell Lymphoma

Ann Arbor Classification: Stage IV: Diffuse or disseminated involvement of one or more extralymphatic organs, with or without associated lymph node involvement; or isolated extralymphatic organ involvement in the absence of adjacent regional lymph node involvement, but in conjunction with disease in distant site(s); or any involvement of the liver or bone marrow, lungs (other than by direct extension from another site), or cerebrospinal fluid.


Chronic Adult T-Cell Leukemia/Lymphoma

An adult T-cell leukemia/ lymphoma associated with skin lesions and mild lymphadenopathy.  Neoplastic lymphocytes are not numerous in the peripheral blood.  Hypercalcemia is absent.


Splenic marginal zone lymphoma recurrent

The reemergence of splenic marginal zone lymphoma after a period of remission.


Mycosis fungoides stage III

Stage III includes: (T4, N0-2, M0, B0-1); IIIA (T4, N0-2, M0, B0); IIIB (T4, N0-2, M0, B1). T4: Confluence of erythema covering 80% or more of body surface area.  N0: No clinically abnormal peripheral lymph nodes; biopsy not required.  N1: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 1 or NCI LN0-2.  N2: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3.  M0: No visceral organ involvement.  B0: Absence of significant blood involvement: 5% or less of peripheral blood lymphocytes are atypical (Sezary cells).  B1: Low blood tumor burden: more than 5% of peripheral blood lymphocytes are atypical (Sezary cells) but does not meet the criteria of B2.  (AJCC 7th ed.)


Central Nervous System Intravascular Large B-Cell Lymphoma

A rare extranodal B-cell non-Hodgkin lymphoma that affects the central nervous system.  It is characterized by the presence of lymphoma cells exclusively in the lumina of small vessels, particularly capillaries.


Ann Arbor Stage II Non-Contiguous Adult Lymphoblastic Lymphoma

Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II) or localized involvement of a single associated extralymphatic organ or site and its regional lymph nodes with or without other lymph node regions on the same side of the diaphragm (IIE). Note: The number of lymph node regions involved may be indicated by a subscript (e.g., II3). (from PDQ)


Early Primary Cutaneous T-Cell Non-Hodgkin Lymphoma

Primary cutaneous T-cell non-Hodgkin lymphoma that has spread from its original site of growth to nearby tissues.


Lymphoplasmacytoid lymphoma/immunocytoma stage I

Ann Arbor Classification: Stage I: Involvement of a single lymph node region (I); or localized involvement of a single extralymphatic organ or site in the absence of any lymph node involvement (IE).


Stage II Childhood Lymphoblastic Lymphoma

Stage II: Disease extent is limited to a single tumor with regional node involvement, 2 or more tumors or nodal areas involved on one side of the diaphragm, or a primary gastrointestinal tract tumor (completely resected) with or without regional node involvement (from PDQ).


Stage III Adult Immunoblastic Lymphoma

Ann Arbor Classification: Stage III: Involvement of lymph node regions on both sides of the diaphragm (III), which also may be accompanied by extralymphatic extension in association with adjacent lymph node involvement (IIIE) or by involvement of the spleen (IIIS) or both (IIIE,S).


central nervous system non-hodgkin lymphoma

A non-Hodgkin lymphoma that arises from the central nervous system.


Recurrent Indolent Adult Non-Hodgkin Lymphoma

The reemergence of indolent non-Hodgkin lymphoma in adults after a period of remission.


adult nasal type extranodal NK/T-cell lymphoma

Nasal type extranodal NK/T-cell lymphoma that occurs in adulthood.


Splenic B Lymphoblastic Lymphoma

B-lymphoblastic lymphoma that involves the spleen.


Nodal peripheral T-cell lymphoma with T follicular helper phenotype

A nodal peripheral T-cell non-Hodgkin lymphoma in which the neoplastic lymphocytes manifest a T follicular helper (TFH) cell phenotype. These lymphomas frequently show a diffuse infiltration pattern without a prominent polymorphic inflammatory background, vascular proliferation, or expansion of follicular dendritic cell meshworks. In some cases, a so-called T-zone pattern may be evident. (WHO 2017)


T-cell lymphoma refractory

T-cell non-Hodgkin lymphoma resistant to treatment.


Advanced Primary Cutaneous T-Cell Non-Hodgkin Lymphoma

Primary cutaneous T-cell non-Hodgkin lymphoma that has spread extensively to other anatomic sites or is no longer responding to treatment.


Transformed Lymphoplasmacytic Lymphoma to Diffuse Large B-Cell Lymphoma

Histologic transformation of a lymphoplasmacytic lymphoma to an aggressive diffuse large B-cell lymphoma.


Splenic Lymphoblastic Lymphoma

Lymphoblastic lymphoma that affects the spleen.


Ann Arbor Stage I Childhood Non-Hodgkin Lymphoma


Ann Arbor Stage II Contiguous Mantle Cell Lymphoma

Ann Arbor Classification: Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II); or localized involvement of a single extralymphatic organ or site in association with regional lymph node involvement with or without involvement of other lymph node regions on the same side of the diaphragm (IIE).


Recurrent Grade 3b Follicular Lymphoma

The reemergence of grade 3b follicular lymphoma after a period of remission.


Stage II Non-Contiguous Adult Indolent Non-Hodgkin Lymphoma


Extranodal marginal zone B-cell lymphoma (MALT type) stage IV

Extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue presenting as stage IV disease.


AIDS-Related Lymphoblastic Lymphoma

A lymphoblastic lymphoma occurring in HIV-positive patients.


granulomatous slack skin disease

Granulomatous slack skin (GSS) is a variant of mycosis fungoides (MF), a form of cutaneous T-cell lymphoma, and is characterized by the presence of circumscribed areas of pendulous lax skin.


EBV-Related Non-Hodgkin Lymphoma

Non-Hodgkin lymphoma that is caused by Epstein-Barr virus infection.


Stage I Anaplastic Large Cell Lymphoma

Ann Arbor Classification: Stage I: Involvement of a single lymph node region (I); or localized involvement of a single extralymphatic organ or site in the absence of any lymph node involvement (IE).


Centroblastic lymphoma refractory

Centroblastic lymphoma, resistant to treatment.


Stage III Mycosis Fungoides and Sezary Syndrome AJCC v7

Stage III includes: (T4, N0-2, M0, B0-1); IIIA (T4, N0-2, M0, B0); IIIB (T4, N0-2, M0, B1). T4: Confluence of erythema covering 80% or more of body surface area.  N0: No clinically abnormal peripheral lymph nodes; biopsy not required.  N1: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 1 or NCI LN0-2.  N2: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3.  M0: No visceral organ involvement.  B0: Absence of significant blood involvement: 5% or less of peripheral blood lymphocytes are atypical (Sezary cells).  B1: Low blood tumor burden: more than 5% of peripheral blood lymphocytes are atypical (Sezary cells) but does not meet the criteria of B2.  (AJCC 7th ed.)


Recurrent Immunoblastic Lymphoma

The reemergence of immunoblastic lymphoma after a period of remission.


Stage IIIB Mycosis Fungoides and Sezary Syndrome AJCC v8

Stage IIIB includes: T4, N0-2, M0, B1. T4: Confluence of erythema covering 80% or more of body surface area. N0: No clinically abnormal peripheral lymph nodes; biopsy not required. N1: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 1 or National Cancer Institute (NCI) LN0-2. N2: Clinically abnormal peripheral lymph nodes; histopathology Dutch grade 2 or NCI LN3. M0: No visceral organ involvement. B1: Low blood tumor burden: more than 5% of peripheral blood lymphocytes are atypical (Sezary cells) but does not meet the criteria of B2. (AJCC 8th ed.)


Leukemic Nonnodal Mantle Cell Lymphoma

Mantle cell lymphoma involving the peripheral blood, bone marrow, and often spleen. It usually has an indolent clinical course.


Aggressive Adult Non-Hodgkin Lymphoma

An aggressive non-Hodgkin lymphoma occurring in adults.  Representative examples include Burkitt lymphoma, precursor B-lymphoblastic lymphoma, precursor T-lymphoblastic lymphoma, and adult T-cell lymphoma/leukemia.


Recurrent Adult Burkitt Lymphoma

The reemergence of adult Burkitt lymphoma after a period of remission.


Advanced Diffuse Large B-Cell Lymphoma

Diffuse large B-cell lymphoma that has spread extensively to distant anatomic sites or is no longer responding to treatment.


Refractory Indolent T-Cell Non-Hodgkin Lymphoma

Indolent T-cell non-Hodgkin lymphoma that is resistant to treatment.


Refractory Indolent Non-Hodgkin Lymphoma

Indolent non-Hodgkin lymphoma that is resistant to treatment.


Mycosis Fungoides and Sezary Syndrome by AJCC v8 Stage

A term that refers to the staging of mycosis fungoides and Sezary syndrome, following the rules of the TNM AJCC v8 classification system. This prognostic stage groups system is based on the ISCL/EORTC revision to the staging of mycosis fungoides and Sezary syndrome. There is no prognostic stage groups system for primary cutaneous B-cell/T-cell (non-mycosis fungoides/Sezary syndrome) lymphomas at this time. This staging system does not apply to eyelid skin lymphomas (are staged according to the classification for ocular adnexal lymphoma).  (from AJCC 8th Ed.)


Refractory Transformed B-Cell Non-Hodgkin Lymphoma

An indolent B-cell non-Hodgkin lymphoma which has undergone histologic transformation to an aggressive B-cell non-Hodgkin lymphoma and has become resistant to treatment.


Bladder Mucosa-Associated Lymphoid Tissue Lymphoma

A rare mucosa-associated lymphoid tissue lymphoma affecting the bladder.


Primary mediastinal large B-cell lymphoma refractory

Primary mediastinal (thymic) large B-cell lymphoma that is resistant to treatment.


Burkitt lymphoma in a patient with immunodeficiency.


Recurrent Grade 2 Follicular Lymphoma

The reemergence of grade 2 follicular lymphoma after a period of remission.


B-cell lymphoma stage IV

Ann Arbor Classification: Stage IV: Diffuse or disseminated involvement of one or more extralymphatic organs, with or without associated lymph node involvement; or isolated extralymphatic organ involvement in the absence of adjacent regional lymph node involvement, but in conjunction with disease in distant site(s); or any involvement of the liver or bone marrow, lungs (other than by direct extension from another site), or cerebrospinal fluid.


Stage IA Mycosis Fungoides and Sezary Syndrome AJCC v8

Stage IA includes: T1, N0, M0, B0,1. T1: Limited patches, papules, and/or plaques covering less than 10% of the skin surface. N0: No clinically abnormal peripheral lymph nodes; biopsy not required. M0: No visceral organ involvement. B0: Absence of significant blood involvement: 5% or less of peripheral blood lymphocytes are atypical (Sezary cells). B1: Low blood tumor burden: more than 5% of peripheral blood lymphocytes are atypical (Sezary cells) but does not meet the criteria of B2. (AJCC 8th ed.)


Diffuse Large B-Cell Lymphoma, Not Otherwise Specified by Gene Expression Profile

A header term that includes the following diffuse large B-cell lymphoma subtypes by gene expression profiling: activated B-cell-like diffuse large B-cell lymphoma, germinal center B-cell-like diffuse large B-cell lymphoma, and type 3 diffuse large B-cell lymphoma.


Recurrent Childhood Grade III Lymphomatoid Granulomatosis

The reemergence of grade III lymphomatoid granulomatosis in childhood after a period of remission.


primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma

Primary cutaneous aggressive epidermotropic CD8+ T-cell lymphoma is a rare form of primary cutaneous T-cell lymphoma characterized by rapidly progressing, localized or disseminated nodules, tumors or eczematous skin lesions. It has a particularly aggressive clinical course with a high tendency to spread, in advanced stages, to extracutaneous locations (the central nervous system, lung, testes). Lymph nodes are often spared.


Refractory Intravascular Large B-Cell Lymphoma

Intravascular large B-cell lymphoma that is resistant to treatment.


Ann Arbor Stage IV Grade 1 Follicular Lymphoma


Pleural Primary Effusion Lymphoma

Primary effusion lymphoma that affects the pleural cavity. The majority of patients are HIV-positive men.


Favorable Non-Hodgkin Lymphoma

A non-Hodgkin lymphoma with favorable prognosis.


ocular adnexal lymphoma

A non-Hodgkin lymphoma arising from the conjunctiva, lacrimal gland, lacrimal drainage apparatus, eyelids, or other orbital tissues around the eye. The vast majority of cases are extranodal marginal zone lymphomas of mucosa-associated lymphoid tissue, however, other histologic types of lymphomas can originate from ocular adnexal tissues, including rare cases of NK/T-cell lymphomas of nasal type.


Ann Arbor Stage II Non-Contiguous Adult Burkitt Lymphoma

Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II) or localized involvement of a single associated extralymphatic organ or site and its regional lymph nodes with or without other lymph node regions on the same side of the diaphragm (IIE). Note: The number of lymph node regions involved may be indicated by a subscript (e.g., II3). (from PDQ)


Salivary Gland Mucosa-Associated Lymphoid Tissue Lymphoma

A low-grade extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue arising from a salivary gland. It usually occurs in the parotid gland and is often associated with Sjogren syndrome. The prognosis is favorable.


high grade B-cell lymphoma

A term that refers to high grade B-cell lymphoma, not otherwise specified or high grade B-cell lymphoma with MYC and BCL2 and/or BCL6 rearrangements.


Ann Arbor Stage I Grade 3 Follicular Lymphoma

Ann Arbor Classification: Stage I: Involvement of a single lymph node region (I); or localized involvement of a single extralymphatic organ or site in the absence of any lymph node involvement (IE).


Recurrent Subcutaneous Panniculitis-Like T-Cell Lymphoma

The reemergence of subcutaneous panniculitis-like T-cell lymphoma after a period of remission.


Ann Arbor Stage II Nodular Lymphocyte Predominant Hodgkin Lymphoma

Ann Arbor Classification: Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II); or localized involvement of a single extralymphatic organ or site in association with regional lymph node involvement with or without involvement of other lymph node regions on the same side of the diaphragm (IIE).


Pediatric nodal marginal zone lymphoma

A nodal marginal zone lymphoma affecting the pediatric population, predominantly males.  It presents with asymptomatic and localized (mainly head and neck) disease in the vast majority of cases.  Morphologically there is often progressive transformation of the germinal centers.  The prognosis is excellent.


retina lymphoma

A lymphoma that involves the retina.


Refractory NK-Cell Lymphoma, Unclassifiable

NK-cell lymphoma, unclassifiable that is resistant to treatment.


Refractory Follicular T-Cell Lymphoma

Follicular T-cell lymphoma that is resistant to treatment.


Pancreatic Non-Hodgkin Lymphoma

A non-Hodgkin lymphoma that arises from the pancreas with the bulk of the tumor localized to this organ.  The vast majority of cases are of B-cell phenotype and include mucosa-associated lymphoid tissue lymphomas, follicular lymphomas, and diffuse large B-cell lymphomas.


Precursor B-lymphoblastic lymphoma stage IV

Ann Arbor Classification: Stage IV: Diffuse or disseminated involvement of one or more extralymphatic organs, with or without associated lymph node involvement; or isolated extralymphatic organ involvement in the absence of adjacent regional lymph node involvement, but in conjunction with disease in distant site(s); or any involvement of the liver or bone marrow, lungs (other than by direct extension from another site), or cerebrospinal fluid.


Body Cavity Primary Effusion Lymphoma

A large B-cell lymphoma presenting as a serous effusion without detectable tumor masses. It is universally associated with human herpes virus 8 (HHV8), also called Kaposi sarcoma-associated herpesvirus.  It mostly occurs in the setting of immunodeficiency. The most common sites of involvement are the pleural, pericardial, and peritoneal cavities. (WHO 2017)


B-cell lymphoma, unclassifiable, with features intermediate between diffuse large b-cell lymphoma and classical Hodgkin lymphoma

A group of lymphomas displaying molecular, morphologic, immunophenotypic, and clinical overlap between classical Hodgkin lymphoma and diffuse large B-cell lymphoma. This term particularly applies to mediastinal lymphomas with overlapping features of mediastinal (thymic) large B-cell lymphoma and classical Hodgkin lymphoma.


Stage II Adult Immunoblastic Lymphoma

Ann Arbor Classification: Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II); or localized involvement of a single extralymphatic organ or site in association with regional lymph node involvement with or without involvement of other lymph node regions on the same side of the diaphragm (IIE). The number of regions involved may be indicated by a subscript (e.g., II3).


Stage IA Mycosis Fungoides AJCC v7

Stage IA includes: T1, N0, M0, B0-1.  T1: Limited patches, papules, and/or plaques covering less than 10% of the skin surface.  May further stratify into T1a (patch only) vs. T1b (plaque +/- patch).  N0: No clinically abnormal peripheral lymph nodes; biopsy not required.  M0: No visceral organ involvement.  B0: Absence of significant blood involvement: 5% or less of peripheral blood lymphocytes are atypical (Sezary cells).  B1: Low blood tumor burden: more than 5% of peripheral blood lymphocytes are atypical (Sezary cells) but does not meet the criteria of B2.  (AJCC 7th ed.)


Small Intestinal T-Cell Non-Hodgkin Lymphoma

A T-cell non-Hodgkin lymphoma that arises from the small intestine.  It includes the enteropathy-associated T-cell lymphoma and the monomorphic CD56 positive intestinal T-cell lymphoma.


Stage II Contiguous Adult Indolent Non-Hodgkin Lymphoma


Angiocentric lymphoma refractory

Nasal type extranodal NK/T-cell lymphoma that does not respond to treatment.


AIDS-Related Plasmablastic Lymphoma

A plasmablastic lymphoma in a patient with acquired immunodeficiency syndrome.


High Grade B-Cell Lymphoma, Not Otherwise Specified

A high grade B-cell lymphoma with blastoid features or features between diffuse large B-cell lymphoma and Burkitt lymphoma which lacks MYC, BCL2, and BCL6 rearrangements.


Pediatric follicular lymphoma

A nodal follicular lymphoma with favorable prognosis. It lacks BCL-2 rearrangement and 14;18 translocation. Nearly all cases are localized. It is usually seen in the pediatric population but similar lymphomas may occur in adults.


Sinonasal Extranodal NK/T-Cell Lymphoma

An extranodal NK/T-cell lymphoma that arises from the sinonasal tract.


large B-cell diffuse lymphoma of kidney

Diffuse large B-cell lymphoma that affects the kidney.


Diffuse Large B-Cell Lymphoma Post-Transplant Lymphoproliferative Disorder

A diffuse large B-cell lymphoma that arises in a patient with a history of organ transplantation.


Lymphoma, Mixed-Cell, Follicular

A follicular lymphoma which contains 6-15 centroblasts per 40X high-power microscopic field.


EZB diffuse large B-cell lymphoma

A diffuse large B-cell lymphoma that is categorized as EZB with high probability by the LymphGen algorithm. This is based on a combination of genetic features and EZB DLBCLs often, but do not always, have hot spot mutations in EZH2 and/or a BCL2 translocation. This class can be further subdivided into two sub-classes EZB-MYC+ and EZB-MYC- using the double hit gene expression signature (DHITsig). This subgroup also commonly has mutations due to aberrant somatic hypermutation affecting IRF8, which can be coding or non-coding.


B-cell lymphoma stage III

Ann Arbor Classification: Stage III: Involvement of lymph node regions on both sides of the diaphragm (III), which also may be accompanied by extralymphatic extension in association with adjacent lymph node involvement (IIIE) or by involvement of the spleen (IIIS) or both (IIIE,S).


Anaplastic Large Cell Lymphoma, Giant Cell Rich Subtype

An anaplastic large cell lymphoma characterized by the presence of numerous giant cells.


Cutaneous Mantle Cell Lymphoma

Mantle cell lymphoma that affects the skin.


Ann Arbor Stage II Grade 2 Follicular Lymphoma

Ann Arbor Classification: Stage II: Involvement of two or more lymph node regions on the same side of the diaphragm (II); or localized involvement of a single extralymphatic organ or site in association with regional lymph node involvement with or without involvement of other lymph node regions on the same side of the diaphragm (IIE).


Recurrent Diffuse Large B-Cell Lymphoma, Not Otherwise Specified

The reemergence of diffuse large B-cell lymphoma, not otherwise specified after a period of remission.


Recurrent T Lymphoblastic Lymphoma

The reemergence of T-lymphoblastic lymphoma after a period of remission.


Centroblastic Lymphoma Post-Transplant Lymphoproliferative Disorder

A centroblastic lymphoma that arises in a patient with a history of organ transplantation.


Refractory Transformed Mycosis Fungoides

Transformed mycosis fungoides that is resistant to treatment.


B-cell small lymphocytic lymphoma stage IV

Ann Arbor Classification: Stage IV: Diffuse or disseminated involvement of one or more extralymphatic organs, with or without associated lymph node involvement; or isolated extralymphatic organ involvement in the absence of adjacent regional lymph node involvement, but in conjunction with disease in distant site(s); or any involvement of the liver or bone marrow, lungs (other than by direct extension from another site), or cerebrospinal fluid.


Lacrimal System Non-Hodgkin Lymphoma

A non-Hodgkin lymphoma that arises from the lacrimal gland or the lacrimal drainage system. The majority of cases are of B-cell type. The most common subtypes are mucosa-associated lymphoid tissue lymphoma, follicular lymphoma, and diffuse large B-cell lymphoma.


Childhood Non-Hodgkin Lymphoma

Non-Hodgkin lymphoma occurring in childhood.


Stage I Non-Hodgkin Lymphoma

Ann Arbor Classification: Stage I: Involvement of a single lymph node region (I); or localized involvement of a single extralymphatic organ or site in the absence of any lymph node involvement (IE).


Small Lymphocytic Lymphoma with Plasmacytoid Differentiation

A small lymphocytic lymphoma characterized by the presence of neoplastic lymphocytes with plasmacytoid morphology.


Intestinal T-Cell Lymphoma

A group of mature T-cell and NK-cell non-Hodgkin lymphomas that includes enteropathy-associated T-cell lymphoma, monomorphic epitheliotropic intestinal T-cell lymphoma, and intestinal T-cell lymphoma, not otherwise specified. Most of these lymphomas arise from the small intestine and a minority from the large intestine or stomach.


Duodenal-type follicular lymphoma

A localized low-grade follicular lymphoma within the gastrointestinal tract, which is distinct from other gastrointestinal tract follicular lymphomas.  It has features that overlap with in situ follicular neoplasia as well as some features resembling an extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue. The prognosis is excellent.


Lymphoplasmacytoid lymphoma/immunocytoma stage IV


Oropharyngeal Non-Hodgkin Lymphoma

A non-Hodgkin lymphoma that arises from the oropharynx.


Stage I Indolent Adult Non-Hodgkin Lymphoma


Lugano Classification Stage II Bulky Adult Non-Hodgkin Lymphoma AJCC v8

Stage II Bulky: Stage II with disease bulk. (from AJCC 8th Ed.)