pulmonary atresia-intact ventricular septum syndrome

Pulmonary atresia with intact ventricular septum (PA-IVS) is a rare form of cyanotic congenital heart malformation characterized by severe cyanosis and tachypnea. PA-IVS presents significant morphologic diversity: at the end of the spectrum are patients with a mildly hypoplastic and tripartite right ventricle (RV) and mild tricuspid valve (TV) hypoplasia, and at the other end are patients with severe RV and TV hypoplasia, often with RV-dependent coronary circulation.


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Hypotrichosis - lymphedema - telangiectasia is an extremely rare syndromic lymphedema disorder characterized by early-onset hypotrichosis, childhood-onset lymphedema, and variable telangiectasia, particularly of the palms.


Hennekam syndrome

Hennekam syndrome is characterized by the association of lymphoedema, intestinal lymphangiectasia, intellectual deficit and facial dysmorphism.


atypical coarctation of aorta

Middle aortic coarctation is a rare vascular anomaly characterized by the segmental narrowing of the abdominal and/or distal descending thoracic aorta with varying involvement of the visceral and renal arteries that commonly presents in children and young adults with early onset and refractory hypertension, abdominal angina, lower-limb claudication and that can lead to life-threatening complications associated with severe hypertension (i.e. myocardial infarction, heart failure, aortic rupture, renal insufficiency and intracranial hemorrhage). It may be due to various congenital or acquired causes, but it is most often secondary to an acquired inflammatory disease (i.e. Takayasu arteritis or giant cell arteritis).


macrothrombocytopenia-lymphedema-developmental delay-facial dysmorphism-camptodactyly syndrome


congenital alveolar dysplasia

A congenital alveolar dysplasia characterized anatomically by a defective and hypoplastic development of pulmonary alveoli that is commonly associated with atelectasis and can be responsible for atelectasis.


lymphatic malformation

Primary lymphedema is caused by anatomic or functional defects in the lymphatic system, resulting in chronic swelling of body parts and lymphatic-system malformation.


right superior vena cava connecting to left-sided atrium

Right superior vena cava connecting to the left-sided atrium is a rare, congenital vascular malformation of the major vessels characterized by the right SVC passing medially and dorsally to the aortic root and draining into the left atrium. Patients usually present a right-to-left systemic venous blood shunt which may manifest with arterial hypoxemia, cyanosis, exercise dyspnea, clubbing of the fingers, palpitations, murmurs and/or potentially fatal brain abscess. Association with other cardiac anomalies has been reported.


pulmonary branch stenosis

Narrowing of the lumen of the right or left pulmonary artery branch.


segmental outgrowth-lipomatosis-arteriovenous malformation-epidermal nevus syndrome

Segmental outgrowth-lipomatosis-arteriovenous malformation-epidermal nevus syndrome is a rare, genetic, polymalformative syndrome characterized by progressive, proportionate, asymmetric segmental overgrowth (with soft tissue hypertrophy and ballooning effect) that develops and progresses rapidly in early childhood, arteriovenous and lymphatic vascular malformations, lipomatosis and linear epidermal nevus (arranged in whorls along the lines of Blaschko). Clinical symptoms of Cowden syndrome, such as macrocephaly and progressive development of numerous hypertrophic hamartomatous and neoplastic lesions involving multiple organs and systems, are also associated. Patients present an increased risk of developing cancer.


microcephaly with or without chorioretinopathy, lymphedema, or intellectual disability

Microcephaly with or without chorioretinopathy, lymphedema or intellectual disability (MCLID) is a rare autosomal dominant condition characterized by variable expression of microcephaly, ocular disorders including chorioretinopathy, congenital lymphedema of the lower limbs, and mild to moderate intellectual disability.


hereditary hemorrhagic telangiectasia

A disorder of angiogenesis leading to arteriovenous dilatations: cutaneo-mucosal hemorrhagic telangiectasias and visceral shunting.


campomelia, Cumming type

Campomelia, Cumming type, is characterized by the association of limb defects and multivisceral anomalies.


Hennekam lymphangiectasia-lymphedema syndrome 1

Any Hennekam syndrome in which the cause of the disease is a mutation in the CCBE1 gene.


premature closure of the arterial duct

Premature closure of the arterial duct is a rare arterial duct anomaly, defined as a significant constriction or closure of the fetal arterial duct in the absence of structural heart defects with pathognomonic features of increased right ventricular afterload, tricuspid regurgitation and, consequently, right atrial dilation and right ventricular hypertrophy. The severity of symptoms is related to the degree and rate of ductal constriction and ranges from mild postnatal respiratory distress to development of ventricular failure with fetal hydrops and intrauterine death or severe cardiopulmonary compromise in the postnatal period. It may be associated with a prenatal exposure to cyclooxygenase inhibitors or corticosteroids.


fixed subaortic stenosis

Fixed subaortic stenosis (FSS) is a rare heart malformation characterized by the obstruction by membranous or fibromuscular tissue of the left ventricular outflow tract (LVOT) below the aortic valve, that occurs as an isolated lesion or in association with additional cardiac malformations (e.g. ventricular septal defect, patent ductus arteriosus, coarctation of the aorta), that presents in childhood with signs of LVOT obstruction (e.g. dyspnea, chest pain, syncope, palpitations) and that can potentially lead to life-threatening complications (e.g. aortic regurgitation, infective endocarditis). It comprises three anatomical subforms: discrete fixed membranous subaortic stenosis (membranous tissue encircling the LVOT), discrete fibromuscular subaortic stenosis (fibromuscular tissue encircling the LVOT) and tunnel subaortic stenosis (fibromuscular diffuse tunnel-like narrowing of the LVOT), the two latter forms being generally more severe than the membranous form.


hereditary vascular anomaly

An instance of vascular anomaly that is caused by a modification of the individual's genome.


absence of the pulmonary artery

Unilateral absence of the pulmonary artery (UAPA) is a rare congenital great vessels anomaly that commonly presents by dyspnea, frequent respiratory infections, hemoptysis and high-altitude pulmonary edema. UAPA is often associated with congenital heart malformation (CHM). In the absence of associated cardiac malformation (isolated UAPA; IUAPA), the condition may be asymptomatic until adult age.


aortic valve disease 2

Any aortic valve disease in which the cause of the disease is a mutation in the SMAD6 gene.


persistent left superior vena cava connecting to the left-sided atrium

Persistent left superior vena cava connecting to the left-sided atrium is a rare, congenital vascular malformation of the major vessels characterized by a persitent left superior vena cava which drains directly to the left atrium, without passing through the coronary sinus (that may be absent in some cases). Patients are usually asymptomatic and discovered incidentally, however hypoxia, cyanosis, murmurs, palpitations, cardiac structural anomalies (e.g. atrial septal defect, bicuspid aortic valve, cor triatrium) and risk of paradoxical embolization may be associated.


vascular malformation

A non-neoplastic disorder that is the result of defects of vascular morphogenesis.


congenital pulmonary veins atresia or stenosis

Congenital pulmonary vein (PV) stenosis or atresia is a rare progressive life-threatening great vessels anomaly characterized by narrowing and obstruction of one or more normally positioned PV at their junction with the left atrium, that usually presents during early infancy with dyspnea, tachypnea, and repeated pulmonary infections, and eventually, when all PV of one lung are affected, results in pulmonary hypertension (PH) and consecutive pulmonary arterial hypertension (PAH). It may manifest as an isolated lesion or associated with other cardiac defects such as congenital pulmonary venous return anomaly and septal defects.


congenital alveolar dysplasia due to TBX4

Any congenital alveolar dysplasia in which the cause of the disease is a mutation in the TBX4 gene.


aortic arch interruption

Aortic arch interruption is a rare heart defect characterized by complete lack of anatomical continuity between the transverse aortic arch and the descending thoracic aorta. AAI should be distinguished anatomically from atresia of the aortic arch where continuity between these segments is achieved by an imperforate fibrous strand of various lengths.


alveolar capillary dysplasia without misalignment of pulmonary veins

A rare form of interstitial and vascular lung disease that presents as severe pulmonary hypertension and refractory hypoxemia early in life that is characterized by a lack of misalignment of the pulmonary veins.


congenital pulmonary venous return anomaly

Congenital pulmonary venous return anomaly is a cardiac malformation where some or all of the pulmonary veins drain into the right atrium or the systemic veins, with or without the presence of pulmonary venous obstruction, leading to various manifestations such as fatigue, exertional dyspnea, pulmonary arterial hypertension, cyanosis and progressive congestive heart failure. The two main subtypes are congenital partial pulmonary venous return anomaly (PAPVC), where one or a few of the pulmonary veins are anomalous, and congenital total pulmonary venous return anomaly (TAPVC), where all of the pulmonary veins are anomalous.


cerebral cavernous malformation 3

Any familial cerebral cavernous malformation in which the cause of the disease is a mutation in the PDCD10 gene.


left superior vena cava persisting to left-sided atrium


Hennekam lymphangiectasia-lymphedema syndrome 2

Any Hennekam syndrome in which the cause of the disease is a mutation in the FAT4 gene.


pulmonary valve agenesis-tetralogy of fallot-absence of ductus arteriosus syndrome

Pulmonary valve agenesis-tetralogy of Fallot-absence of ductus arteriosus syndrome is a rare congenital heart malformation characterized by a tetralogy of Fallot (pulmonary stenosis, overriding aorta, ventricular septal defect and right ventricular hypertrophy), complete absence or rudimentary pulmonary valve that is both stenotic and regurgitant and an absence of the ductus arteriosus. It presents prenatally with cardiomegaly, polyhydramnios, fetal heart failure, hydrops fetalis and fetal demise or postnatally with cyanosis and respiratory failure due to bronchomalacia secondary to bronchial compression from dilated pulmonary arteries. It is frequently associated with 22q11 deletion.


agenesis of the superior vena cava

Agenesis of the superior vena cava (SVC) is a rare congenital anomaly of the great veins characterized by unilateral or bilateral complete absence of the SVC. Unilateral agenesis is mainly asymptomatic (most of the time diagnosed incidentally) and patients usually have otherwise normal heart structure. Bilateral agenesis, however, is frequently associated with other congenital cardiac anomalies and/or conduction abnormalities (such as tetralogy of Fallot, atrial septal defect) and typically present symptoms of SVC syndrome.


famililal cerebral cavernous malformations

A rare evolutive vascular malformation disorder characterized by closely clustered irregular dilated capillaries that can be asymptomatic or that can cause variable neurological manifestations such as seizures, non-specific headaches, progressive or transient focal neurologic deficits, and/or cerebral hemorrhages.


congenital anomaly of the inferior vena cava


lymphatic malformation 4

Any hereditary lymphedema in which the cause of the disease is a mutation in the VEGFC gene.


azygos continuation of the inferior vena cava


congenital vascular malformation

A congenital abnormality of the arteries and veins, lymph vessels or veins and lymph vessels.


absence of innominate vein

Absence of innominate vein is a rare congenital anomaly of the great veins characterized by absence of the left brachiocephalic vein (or innominate vein), resulting in an anomalous venous vasculature. Patients are usually asymptomatic and the anomaly is typically discovered intraoperatively. An association with persistence of left superior vena cava, permanent levoatrial cardinal vein or anomaly of the inferior vena cava has been reported in some cases.


hereditary hemorrhagic telangiectasia type 4


telangiectasia, hereditary hemorrhagic, type 2

Any hereditary hemorrhagic telangiectasia in which the cause of the disease is a mutation in the ACVRL1 gene.


internal carotid agenesis

Internal carotid artery (ICA) agenesis (uni or bilateral) is a developmental defect that may be asymptomatic or lead to cerebrovascular lesions. It is a rare malformation, with only around hundred cases reported in the literature. When symptoms are present, they are caused by cerebrovascular insufficiency, compression of the brain by vessels that dilate to compensate for the absence of the ICA, or the presence of an aneurysm. Associated intracranial aneurysms occur in 25 to 35% of patients and are often responsible for intracranial hemorrhage, which may present as the initial symptom. The absence of the ICA is the result of either agenesis or aplasia. The term agenesis is used when both the ICA and its bony canal are absent, whereas there is some evidence of carotid canals in cases of aplasia. The absence of the ICA can be detected by angiography or by computerized tomography.


Norman-Roberts syndrome

Lissencephaly syndrome, Norman-Roberts type is characterized by the association of lissencephaly type I with craniofacial anomalies (severe microcephaly, a low sloping forehead, a broad and prominent nasal bridge and widely set eyes) and postnatal growth retardation.


right inferior vena cava connecting to left-sided atrium


congenital patent ductus arteriosus aneurysm

Congenital patent ductus arteriosus aneurysm is a rare, congenital, arterial duct anomaly characterized by a saccular dilatation of the ductus arteriosus. It is often asymptomatic or presents shortly after birth with respiratory distress, stridor, cyanosis and/or weak cry. Complications, such as rupture, thromboembolism, infection, airway erosion and/or compression of the adjacent thoracic structures, can develop. Spontaneous resolution has been reported.


capillary malformation-arteriovenous malformation 1


Grade 1 Dysmenorrhea, CTCAE

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Mild symptoms; intervention not indicated


Stage IVA Ovarian Cancer AJCC v8

Stage IVA includes: Any T, Any N, M1a. M1a: Ovarian cancer with pleural effusion with positive cytology. (from AJCC 8th Ed.)


Stage IV Ovarian Cancer AJCC v8

Stage IV includes: Any T, Any N, M1: M1: Distant metastasis, including pleural effusion with positive cytology; liver or splenic parenchymal metastasis; metastasis to extra-abdominal organs (including inguinal lymph nodes and lymph nodes outside the abdominal cavity); and transmural involvement of intestine. (from AJCC 8th Ed.)


Stage IVB Ovarian Cancer AJCC v8

Stage IVB includes: Any T, Any N, M1b. M1b: Ovarian cancer with liver or splenic parenchymal metastases; metastases to extra-abdominal organs (including inguinal lymph nodes and lymph nodes outside the abdominal cavity); transmural involvement of intestine. (from AJCC 8th Ed.)


Modified smallpox

Smallpox that occurs in an individual who has been vaccinated.


Testicular Germ Cell Neoplasia In Situ with Extratubular Extension

A malignant neoplasm that arises from the testis and is characterized by the presence of large germ cells with abundant cytoplasm in the seminiferous tubules, associated with an invasive germ cell tumor in the adjacent tissues.


Grade 1 Stoma Site Infection, CTCAE

Localized, local intervention indicated


trisomy 9p

Trisomy 9p is a rare chromosomal anomaly syndrome, resulting from a partial or complete trisomy of the short arm of chromosome 9, with a wide phenotypic variablility, typically characterized by intellectual disability, craniofacial dysmorphism (e.g. microcephaly, large anterior fontanel, hypertelorism, strabismus, downslanting palpebral fissures, malformed, low-set, protruding ears, bulbous nose, macrostomia, down-turned corners of mouth, micrognathia), digital anomalies (brachydactyly and clinodactyly), and short stature. Less frequently patients present with cardiopathy and renal, skeletal, and central nervous system malformations.


N-Acetyl-D-hexosamine

Any N-acetylhexosamine in which the hexosamine has D-configuration. The structure provided is an illustrative example of the pyranose form of an N-acetyl-D-hexosamine.


N-Acetyl-D-Glucosamine

The D isomer of N-acetylglucosamine.


Advanced Rectal Squamous Cell Carcinoma

Rectal squamous cell carcinoma that has spread extensively to other anatomic sites or is no longer responding to treatment.


Metastatic Rectal Squamous Cell Carcinoma

Rectal squamous cell carcinoma that has spread from the original site of growth to other anatomic sites.


Locally Advanced Rectal Squamous Cell Carcinoma

Rectal squamous cell carcinoma that has spread to nearby tissues or lymph nodes.


Hormonal Change

A finding indicating a deficiency or excess of one or more hormones.


Growth Hormone Secretion Abnormality

An abnormal production of growth hormone.


Grade 3 Sinusoidal Obstruction Syndrome, CTCAE

Blood bilirubin >5 mg/dL; coagulation modifier indicated (e.g., defibrotide); reversal of flow on ultrasound


Grade 1 Neonatal Pulmonary Hemorrhage, AE

An adverse event in a newborn characterized by limited hemorrhagic secretion in an endotracheal (ET) tube.


Grade 3 Erythema Multiforme, CTCAE

Target lesions covering >30% BSA and associated with oral or genital erosions


Grade 4 Hyperkalemia, CTCAE

>7.0 mmol/L; life-threatening consequences


Early menarche

Occurrence of the first menstrual period in a female before the usual or expected age.


Isolated Premature Menarche

Occurrence of the first menstrual period in a girl before the lower limit of the normal age range for the reference population, without other signs of puberty.


campomelic dysplasia

Campomelic dysplasia is a very rare disorder characterized by a variable association of skeletal abnormalities (bowed and fragile long bones, pelvis and chest abnormalities, eleven rib pairs instead of the usual twelve), and extraskeletal abnormalities (facial dysmorphology, cleft palate, sexual ambiguity or sex reversal in two thirds of the affected boys, and brain, heart and kidney malformations).


Grade 1 Lung Thoracic Nerve Intra-Operative Injury, CTCAE


Grade 2 Jejunum Stricture or Stenosis, CTCAE


Grade 5 Jejunum Stricture or Stenosis, CTCAE


Grade 3 Jejunum Stricture or Stenosis, CTCAE


Grade 1 Jejunum Stricture or Stenosis, CTCAE


Grade 4 Jejunum Stricture or Stenosis, CTCAE


myopathy caused by variation in FKRP

Any myopathy in which the cause of the disease is a variation in the FKRP gene.


qualitative or quantitative defects of alpha-dystroglycan


autosomal recessive limb-girdle muscular dystrophy type 2U

Any autosomal recessive limb-girdle muscular dystrophy in which the cause of the disease is a mutation in the ISPD gene.


qualitative or quantitative defects of protein glycosyltransferase-like


muscular dystrophy-dystroglycanopathy type B5

A congenital muscular dystrophy characterized by autosomal recessive inheritance of muscular dystrophy with variable penetrance of intellectual disability and structural brain abnormalities that has material basis in homozygous or compound heterozygous mutation in the FKRP gene on chromosome 19q13.3.


autosomal recessive limb-girdle muscular dystrophy type 2O

Autosomal recessive limb-girdle muscular dystrophy type 2O (LGMD2O) is a form of limb-girdle muscular dystrophy characterized by an onset in childhood or adolescence of rapidly progressive proximal limb muscle weakness (particularly affecting the neck, hip girdle, and shoulder abductors), hypertrophy in the calves and quadriceps, ankle contractures, and myopia.


muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type a, 8

Any muscular dystrophy-dystroglycanopathy, type A in which the cause of the disease is a mutation in the POMGNT2 gene.


qualitative or quantitative defects of protein involved in O-glycosylation of alpha-dystroglycan


primary qualitative or quantitative defects of alpha-dystroglycan


muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type a, 7

Any muscular dystrophy-dystroglycanopathy, type A in which the cause of the disease is a mutation in the ISPD gene.


myopathy caused by variation in POMGNT1

Any myopathy in which the cause of the disease is a variation in the POMGNT1 gene.


muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type A13


myopathy caused by variation in POMT2

Any myopathy in which the cause of the disease is a variation in the POMT2 gene.


limb-girdle muscular dystrophy due to POMK deficiency

Limb-girdle muscular dystrophy due to POMK deficiency is a form of limb-girdle muscular dystrophy presenting in infancy with muscle weakness and delayed motor development (eventually learning to walk at 18 months of age) followed by progressive proximal weakness, pseudohypertrophy of calf muscles, mild facial weakness, and borderline intelligence.


myopathy caused by variation in POMT1

Any myopathy in which the cause of the disease is a variation in the POMT1 gene.


muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type A5


qualitative or quantitative defects of protein O-mannosyltransferase 1


qualitative or quantitative defects of fukutin


muscular dystrophy-dystroglycanopathy (congenital with intellectual disability), type B3


qualitative or quantitative defects of FKRP


muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type a, 10

Any muscular dystrophy-dystroglycanopathy, type A in which the cause of the disease is a mutation in the RXYLT1 gene.


autosomal recessive limb-girdle muscular dystrophy type 2K

Autosomal recessive limb-girdle muscular dystrophy type 2K (LGMD2K) is a form of limb-girdle muscular dystrophy characterized by the onset of slowly progressive proximal muscle weakness during childhood (with fatigue and difficulty running and climbing stairs) and developmental delay. Mild intellectual deficit and microcephaly, without any obvious structural brain abnormality, are found in all patients. Mild pseudohypertrophy and joint contractures of the ankles have also been reported.


muscle-eye-brain disease

A rare, congenital muscular dystrophy due to dystroglycanopathy characterized by early onset muscular dystrophy, severe muscular hypotonia, severe mental retardation and typical brain and eye malformations, including pachygyria, polymicrogyria, agyria, brainstem and cerebellar structural anomalies, severe myopia, glaucoma, optic nerve and retinal hypoplasia. Patients may present with seizures, macrocephaly or microcephaly, microphthalmia, and congenital contractures. Depending on the severity, limited motor function is acquired. Less severe cases have been reported.


muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type a, 12

Any muscular dystrophy-dystroglycanopathy, type A in which the cause of the disease is a mutation in the POMK gene.


muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type A9


autosomal recessive limb-girdle muscular dystrophy type 2I

Autosomal recessive limb-girdle muscular dystrophy type 2I (LGMD2I) is a subtype of autosomal recessive limb-girdle muscular dystrophy that presents a highly variable age of onset and phenotypic spectrum typically characterized by slowly progressive proximal weakness of the pelvic and shoulder girdle musculature (predominantly affecting the lower limbs), frequently associated with waddling gait, scapular winging, calf and tongue hypertrophy, exercise-induced myalgia, and myoglobinuria and/or elevated creatine kinase serum levels. Abdominal muscle weakness, cardiomyopathy, respiratory muscle involvement and various brain abnormalities have also been reported.


autosomal recessive limb-girdle muscular dystrophy type 2N

Autosomal recessive limb-girdle muscular dystrophy type 2N (LGMD2N) is a form of limb-girdle muscular dystrophy characterized by proximal weakness (manifesting as slowness in running) presenting in infancy, along with calf hypertrophy, mild lordosis, scapular winging and normal intelligence or mild intellectual disability.


muscular dystrophy-dystroglycanopathy (congenital with intellectual disability), type B2

An autosomal recessive inherited congenital muscular dystrophy caused by mutations in the POMT2 gene. It is characterized by mental retardation and mild structural brain abnormalities resulting from defective glycosylation of alpha-dystroglycan.


qualitative or quantitative defects of protein O-mannose beta1, 2N-acetylglucosaminyltransferase


qualitative or quantitative defects of protein O-mannosyltransferase 2


muscular dystrophy-dystroglycanopathy (congenital with brain and eye anomalies), type a, 11

Any muscular dystrophy-dystroglycanopathy, type A in which the cause of the disease is a mutation in the B3GALNT2 gene.


muscular dystrophy-dystroglycanopathy (congenital with intellectual disability), type B1


Broad Ligament Papillary Cystadenoma

A cystadenoma that arises from the broad ligament. It is characterized by the presence of small papillary projections in the inner surface of the cysts. It may be sporadic or associated with von Hippel-Lindau disease.


5beta-cholanic acids

Members of the class of cholanic acids based on a 5beta-cholane skeleton.


hydroxy-5beta-cholanic acid

Any member of the class of 5beta-cholanic acids carrying at least one hydroxy group at unspecified position.


dihydroxy-5beta-cholanic acid

A hydroxy-5beta-cholanic acid carrying two hydroxy groups at unspecified positions.


bile acid

Any member of a group of hydroxy-5beta-cholanic acids occuring in bile, where they are present as the sodium salts of their amides with glycine or taurine. In mammals bile acids almost invariably have 5beta-configuration.


bile acids

Any member of a group of hydroxy steroids occuring in bile, where they are present as the sodium salts of their amides with glycine or taurine. In mammals bile acids almost invariably have 5beta-configuration, while in lower vertebrates, some bile acids, known as allo-bile acids, have 5alpha-configuration.


dihydroxycholanic acid

Any member of the class of cholanic acids carrying two hydroxy substituents at unspecified positions.


Grade 5 Fibrosis-Deep Connective Tissue, CTCAE


Cervical Warty Carcinoma

A human papillomavirus-related cervical squamous cell carcinoma characterized by the presence of a warty surface and koilocytotic atypia.


congenital vascular bone syndrome

An alteration in limb growth caused by congenital vascular malformations in childhood.


angioosteohypotrophic syndrome

Angioosteohypotrophic syndrome is a rare, congenital, vascular anomaly syndrome characterized by venous or, on occasion, arterial malformations which lead to soft tissue hypertrophy and bone hypoplasia. Affected limb is generally shortened, highly deformed, painful and edematous and associates bone and muscle hypotrophy. Single parts, or multiple small parts, of limbs are typically affected but more extensive involvement, including complete extremity, shoulder girdle and axilla, has been reported.


Grade 1 Rectal Mucositis, CTCAE

Asymptomatic or mild symptoms; intervention not indicated


pseudohypoaldosteronism type 2D

Any pseudohypoaldosteronism type 2 in which the cause of the disease is a mutation in the KLHL3 gene.


Craniomandibular Disorders

A disorder that affects the muscles of head and neck and temporomandibular joint.


osteofibrous dysplasia

A benign, usually self-limited fibro-osseous lesion of the bone that affects infants and children. It usually arises from the cortical bone of the anterior mid-shaft of the tibia. Patients usually present with swelling or painless bowing of the tibia. Progression to adamantinoma has been reported in some cases.


Grade 3 Anus Stricture or Stenosis, CTCAE


Stage IIIC Ovarian Cancer

Stage IIIC includes: (T3c, N0, M0); (Any T, N1, M0). T3c: Peritoneal metastasis beyond pelvis more than 2cm in greatest dimension and/or regional lymph node metastasis. N0: No regional lymph node metastasis.  N1: Regional lymph node metastasis.  M0: No distant metastasis. (AJCC 6th and 7th eds.)


Stage IIIB Ovarian Germ Cell Tumor AJCC v6 and v7

Stage IIIB includes: T3b, N0, M0. T3b: Macroscopic peritoneal metastasis beyond pelvis 2 cm or less in greatest dimension. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 6th and 7th eds.)


Stage IIB Ovarian Cancer AJCC v8

Stage IIB includes: T2b, N0, M0. T2b: Ovarian cancer with tumor extension to and/or implants on other pelvic tissues. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 8th Ed.)


Stage IIIA Ovarian Cancer AJCC v6 and v7

Stage IIIA includes: T3a, N0, M0.  T3a: Microscopic peritoneal metastasis beyond pelvis (no macroscopic tumor). N0: No regional lymph node metastasis.  M0: No distant metastasis. (AJCC 6th and 7th eds.)


Ovarian Sex Cord-Stromal Tumor, Not Otherwise Specified

A sex cord-stromal tumor that arises from the ovary and lacks the morphologic features that belong to the categories of granulosa-stromal tumors, Sertoli-stromal tumors, or steroid cell tumors.


Recurrent Malignant Ovarian Brenner Tumor

The reemergence of malignant ovarian Brenner tumor after a period of remission.


Stage IC Ovarian Germ Cell Tumor AJCC v6 and v7

Stage IC includes: (T1c, N0, M0).  T1c: Tumor limited to one or both ovaries with any of the following: capsule ruptured, tumor on ovarian surface, malignant cells in ascites or peritoneal washings.  N0: No regional lymph node metastasis.  M0: No distant metastasis. (AJCC 6th and 7th Eds.)


FIGO Stage IIIA2 Ovarian Cancer

Ovarian cancer with microscopic, extrapelvic (above the brim) peritoneal involvement +/- positive retroperitoneal lymph nodes. (FIGO, 2014)


ovarian endometrioid stromal and related neoplasms

A category of rare neoplasms that arise from the ovary. It includes low grade endometrioid stromal sarcoma and undifferentiated sarcoma.


Ovarian Neuroendocrine Carcinoma

A small cell carcinoma, pulmonary-type or large cell neuroendocrine carcinoma arising from the ovary.


Borderline Ovarian Mucinous Tumor/Atypical Proliferative Ovarian Mucinous Tumor with Microinvasion

A low grade ovarian epithelial neoplasm characterized by the presence of neoplastic mucinous epithelial cells, atypia, and microinvasion of the ovarian stroma.


Stage IV Ovarian Embryonal Carcinoma AJCC v6 and v7

Stage IV includes: Any T, Any N, M1. M1: Distant metastasis (excludes peritoneal metastasis). (AJCC 6th and 7th eds.)


Borderline Ovarian Mixed Epithelial Tumor/Atypical Proliferative Ovarian Mixed Epithelial Tumor

A low grade, non-invasive mixed epithelial proliferative neoplasm that arises from the ovary. In most cases is composed of serous and endocervical-type mucinous cells.


Stage IV Ovarian Cancer AJCC v6 and v7

Stage IV includes: Any T, Any N, M1.  M1: Distant metastasis (excludes peritoneal metastasis). (AJCC 6th and 7th eds.)


Metastatic Ovarian Clear Cell Adenocarcinoma

Ovarian clear cell adenocarcinoma that has spread from its original site of growth to another anatomic site.


Stage IIB Ovarian Germ Cell Tumor AJCC v6 and v7

Stage IIB includes: T2b N0, M0. T2b: Extension and/or implants on other pelvic tissues. No malignant cells in ascites or peritoneal washings. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 6th and 7th eds.)


FIGO Stage IVA Ovarian Cancer

Ovarian cancer with pleural effusion with positive cytology. (FIGO, 2014)


Borderline Ovarian Mucinous Tumor

A low grade mucinous epithelial neoplasm arising from the ovary.  It is characterized by an atypical proliferation of mucinous-type epithelial cells without evidence of stromal invasion.  The mucinous epithelial cells may be of intestinal or endocervical type.


Recurrent Ovarian Transitional Cell Carcinoma

The reemergence of ovarian transitional cell carcinoma after a period of remission.


Stage IIIC Ovarian Cancer AJCC v8

Stage IIIC includes: T3c, N0/N1, M0. T3c: Ovarian cancer with macroscopic peritoneal metastasis beyond the pelvis more than 2 cm in greatest dimension with or without metastasis to the retroperitoneal lymph nodes (includes extension of tumor to capsule of liver and spleen without parenchymal involvement of either organ). N0: No regional lymph node metastasis. N1: Positive retroperitoneal lymph nodes only (histologically confirmed). M0: No distant metastasis. (AJCC 8th Ed.)


Ovarian Mixed Cell Adenocarcinoma

An adenocarcinoma that arises from the ovary and is characterized by the presence of two or more adenocarcinoma components, most often endometrioid and clear cell.


Locally Advanced Ovarian Carcinoma

Ovarian carcinoma that has spread from its original site of growth to nearby tissues or lymph nodes.


Recurrent Ovarian Undifferentiated Carcinoma

The reemergence of ovarian undifferentiated carcinoma after a period of remission.


Ovarian Soft Tissue Neoplasm

A benign, intermediate, or malignant mesenchymal neoplasm of the ovary. Representative examples include leiomyoma, myxoma, and sarcoma.


ovarian stromal hyperthecosis

A non-neoplastic disorder that usually affects postmenopausal women. It is characterized by the leuteinization of ovarian stromal cells. The ovaries are bilaterally involved and enlarged. When it affects women in reproductive age, it causes virilization, high blood pressure, and increased insulin levels.


Metastatic Ovarian Carcinosarcoma

Ovarian carcinosarcoma that has spread from its original site of growth to another anatomic site.


Stage I Ovarian Germ Cell Tumor AJCC v6 and v7

Stage I includes: (T1, N0, M0). T1: Tumor limited to ovaries (one or both).  N0: No regional lymph node metastasis.  M0: No distant metastasis. (AJCC 6th and 7th Eds.)


malignant sex cord stromal tumor of ovary

Malignant sex cord stromal tumor (SCST) of ovary is a rare ovarian cancer arising from granulosa, theca, sertoli and leydig cells or stromal fibroblasts, occurring at any age and presenting with abdominal or pelvic mass, and characterized (with the exception of fibroma) by the production of sex steroids resulting in manifestations of hormone excess, with a relatively favorable prognosis.


Stage IIIB Ovarian Cancer AJCC v8

Stage IIIB includes: T3b, N0/N1, M0. T3b: Ovarian cancer with macroscopic peritoneal metastasis beyond pelvis 2 cm or less in greatest dimension with or without metastasis to the retroperitoneal lymph nodes. N0: No regional lymph node metastasis. N1: Positive retroperitoneal lymph nodes only (histologically confirmed). M0: No distant metastasis. (AJCC 8th Ed.)


Stage II Borderline Ovarian Surface Epithelial-Stromal Tumor

Stage II includes: T2, N0, M0.  T2: Tumor involves one or both ovaries with pelvic extension and/or implants.  N0: No regional lymph node metastasis.  M0: No distant metastasis. (AJCC 6th and 7th eds.)


Ovarian Mucinous Carcinoid Tumor

A rare carcinoid tumor that arises from the ovary and is characterized by the presence of glands that are lined by cells that contain intracytoplasmic mucin or resemble goblet cells.  Pools of mucin, containing glands, may also be present.  It may have an aggressive clinical course and it can metastasize to other anatomic sites.


ovarian Sertoli-Leydig cell tumor

A benign or malignant sex cord-stromal tumor arising from the ovary. It is characterized by the presence of neoplastic Leydig cells. Signs and symptoms include hirsutism, menorrhagia and metrorrhagia. It may be associated with trisomy 8.


Well Differentiated Ovarian Steroid Cell Tumor

An ovarian steroid cell tumor not otherwise specified, and characterized by the absence of nuclear atypia, mitotic figures, necrosis, and hemorrhage.


Refractory Ovarian Clear Cell Adenocarcinoma

Ovarian clear cell adenocarcinoma that is resistant to treatment.


Ovarian Wolffian Tumor

An epithelial neoplasm of Wolffian (mesonephric) origin arising from the ovarian hilum. Most tumors behave in a benign fashion.


Ovarian Stromal-Leydig Cell Tumor

A rare, benign and well circumscribed stromal tumor of the ovary.  It is characterized by the presence of a fibrotic stroma with clusters of Leydig cells.  The Leydig cells contain crystals of Reinke.  Patients may present with virilization.


Stage IIA Ovarian Cancer AJCC V6 and v7

Stage IIA includes: T2a, N0, M0.  T2a: Extension and/or implants on uterus and/or tube(s). No malignant cells in ascites or peritoneal washings.  N0: No regional lymph node metastasis.  M0: No distant metastasis. (AJCC 6th and 7th eds.)


non-gestational ovarian choriocarcinoma

A malignant germ cell tumor that arises from the ovary and is composed of cytotrophoblasts, syncytiotrophoblasts, and extravillous trophoblasts. The prognosis is less favorable than gestational choriocarcinoma.


Stage IB Ovarian Germ Cell Tumor AJCC v6 and v7

Stage IB includes: (T1b, N0, M0). T1b: Tumor limited to both ovaries; capsules intact, no tumor on ovarian surface.  No malignant cells in ascites or peritoneal washings.  N0: No regional lymph node metastasis.  M0: No distant metastasis. (AJCC 6th and 7th Eds.)


Borderline Ovarian Clear Cell Tumor/Atypical Proliferative Ovarian Clear Cell Tumor with Intraepithelial Carcinoma

A non-invasive clear cell adenofibromatous neoplasm that arises from the ovary. It is characterized by the presence of atypical neoplastic clear or hobnail epithelial cells and intraepithelial carcinoma.


FIGO Stage IIIA1(i) Ovarian Cancer

Ovarian cancer with positive retroperitoneal lymph nodes only. Metastasis equal or less than 10 mm. (FIGO, 2014)


malignant non-dysgerminomatous germ cell tumor of ovary

A malignant germ cell tumor other than dysgerminoma that arises from the ovary.


Malignant Ovarian Luteinized Thecoma

A thecoma of the ovary that contains lutein cells and may metastasize to other anatomic sites. It is usually characterized by nuclear atypia and mitotic activity.


ovarian angiosarcoma

A malignant vascular neoplasm arising from the ovary.


Stage II Ovarian Polyembryoma AJCC v6 and v7

Stage II includes: T2, N0, M0. T2: Tumor involves one or both ovaries with pelvic extension and/or implants. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 6th and 7th eds.)


Stage I Borderline Ovarian Surface Epithelial-Stromal Tumor

Stage I includes: T1, N0, M0. T1: Tumor limited to ovaries (one or both).  N0: No regional lymph node metastasis.  M0: No distant metastasis.  (AJCC 6th and 7th eds.)


Stage IIIA Ovarian Germ Cell Tumor AJCC v6 and v7

Stage IIIA includes: T3a, N0, M0. T3a: Microscopic peritoneal metastasis beyond pelvis (no macroscopic tumor). N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 6th and 7th eds.)


Recurrent Ovarian Carcinosarcoma

The reemergence of ovarian carcinosarcoma after a period of remission.


Ovarian germ cell choriocarcinoma stage II


Recurrent BRCA- Associated Ovarian Carcinoma

The reemergence of BRCA-associated ovarian carcinoma after a period of remission.


ovarian endometriosis

A non-neoplastic disorder characterized by the growth of endometrial tissue in the ovaries. It results in the development of blood filled ovarian cysts (chocolate cysts), and creation of scars and adhesions.


Borderline Ovarian Serous Tumor/Atypical Proliferative Ovarian Serous Tumor

A non-invasive serous neoplasm that arises from the ovary and shows greater cellular proliferation and cytologic atypia as compared to benign ovarian serous tumors, but less as compared to low-grade ovarian serous carcinoma.


ovarian gynandroblastoma

A rare neoplasm arising from the ovary. Although it may occur at any age, it is more often seen in young females. Morphologically, it is characterized by a mixture of two cellular populations: well differentiated Sertoli cells and granulosa cells, with the latter constituting at least ten percent of the neoplasm. The vast majority of cases are stage I lesions at presentation and produce either estrogenic or androgenic manifestations. Although it may present as a massive ovarian tumor, it usually follows a benign clinical course. Very rare case reports of testicular lesions morphologically resembling gynandroblastomas are in fact variants of juvenile granulose cell tumor, or Sertoli cell tumor, or a combination of both.


Stage IIIB Ovarian Cancer AJCC v6 and v7

Stage IIIB includes: T3b, N0, M0.  T3b: Macroscopic peritoneal metastasis beyond pelvis 2 cm or less in greatest dimension. N0: No regional lymph node metastasis.  M0: No distant metastasis. (AJCC 6th and 7th eds.)


Refractory Extrarenal Rhabdoid Tumor of the Ovary

An extrarenal rhabdoid tumor of the ovary that is resistant to treatment.


Refractory Ovarian Squamous Cell Carcinoma

Ovarian squamous cell carcinoma that is resistant to treatment.


Benign epithelial tumor of ovary

A non-metastasizing tumor that originates from the surface epithelium of the ovary.  It is composed of epithelial cells and stroma.  Representative examples include serous cystadenoma, mucinous cystadenoma, clear cell adenofibroma, and benign Brenner tumor.


ovarian adenosarcoma

A biphasic neoplasm that arises from the ovary and is characterized by the presence of mullerian-type epithelial tissue in a mesenchymal sarcomatous stroma. The presence of a high grade sarcomatous component is associated with recurrences and metastases.


Stage IV Ovarian Polyembryoma AJCC v6 and v7

Stage IV includes: Any T, Any N, M1. M1: Distant metastasis (excludes peritoneal metastasis). (AJCC 6th and 7th eds.)


Ovarian germ cell choriocarcinoma stage I

Stage I includes: T1, N0, M0. T1: Tumor limited to ovaries (one or both). N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 6th and 7th eds.)


Ovarian Smooth Muscle Tumor of Uncertain Malignant Potential

A smooth muscle neoplasm that arises from the ovary and cannot be reliably diagnosed as benign or malignant, because of the presence of ambiguous morphologic findings.


Poorly Differentiated Ovarian Sertoli-Leydig Cell Tumor

A Sertoli-Leydig tumor of the ovary characterized by the presence of a sarcomatoid stroma which contains primitive gonadal stromal cells. It may behave in a malignant fashion and metastasize to other anatomic sites.


Malignant Ovarian Thecoma

A thecoma of the ovary which may metastasize to another anatomic site. It is usually characterized by nuclear atypia and mitotic activity. Malignant thecomas are rare.


Ovarian Mucinous Cystic Tumor Associated with Pseudomyxoma Peritonei

The presence of abundant mucoid or gelatinous, cellular or acellular material in the pelvis and abdominal cavity (pseudomyxoma peritonei) that is associated with the presence of an ovarian mucinous cystic tumor.  In the vast majority of cases, the presence of pseudomyxoma peritonei is the result of metastasis from a primary appendiceal or gastrointestinal tumor site, and the ovarian mucinous tumor is metastatic rather than primary.


Ovarian Cancer by AJCC v8 Stage

A term that refers to the staging of ovarian cancer according to the American Joint Committee on Cancer, 8th edition. This staging system applies to ovarian carcinomas. Nonepithelial primary ovarian cancers may be staged using this classification but should be reported separately. (from AJCC 8th Ed.)


Ovarian Signet Ring Stromal Tumor

A rare, benign sex cord-stromal tumor that arises from the ovary. It is characterized by the presence of round cells with a single cytoplasmic vacuole and an eccentric nucleus resembling signet ring cells. Mucin stains are negative.


Stage I Ovarian Dysgerminoma AJCC v6 and v7

Stage I includes: (T1, N0, M0). T1: Tumor limited to ovaries (one or both).  N0: No regional lymph node metastasis.  M0: No distant metastasis. (AJCC 6th and 7th Eds.)


Recurrent Ovarian Adenocarcinoma

The reemergence of ovarian adenocarcinoma after a period of remission.


Ovarian Seromucinous Cystadenoma

A non-metastasizing cystic mixed epithelial neoplasm that arises from the ovary. It is characterized by the presence of more than one epithelial cell type, most often serous and endocervical-type mucinous.


Ovarian Smooth Muscle Neoplasm

A benign, intermediate, or malignant neoplasm that arises from the ovary and is composed of neoplastic cells with smooth muscle differentiation.


Ovarian Low Grade Endometrioid Stromal Sarcoma

A rare invasive mesenchymal tumor that arises from the ovary.  It is characterized by the absence of marked pleomorphism and nuclear atypia in the neoplastic mesenchymal cells.  It may have a favorable clinical outcome.


Ovarian Sertoli Cell Tumor with Lipid Storage

A Sertoli cell tumor that arises from the ovary and is characterized by the presence of cells that are distended by the presence of intracytoplasmic lipid.


pediatric ovarian germ cell tumor

A germ cell tumor that arises from the ovary and occurs in children.


Ovarian Mesonephric-Like Adenocarcinoma

An exceedingly rare adenocarcinoma that arises from the ovary and displays mesonephric differentiation. Some tumors are thought to derive from mesonephric duct (Wolffian duct) remnants. Others may have a Mullerian duct lineage.


Recurrent Malignant Ovarian Granulosa Cell Tumor

The reemergence of malignant ovarian granulosa cell tumor after a period of remission.


Refractory Ovarian Transitional Cell Carcinoma

Ovarian transitional cell carcinoma that is resistant to treatment.


Benign Ovarian Seromucinous Tumor

A non-metastasizing mixed epithelial neoplasm that arises from the ovary. It is characterized by the presence of more than one epithelial cell type, most often serous and endocervical-type mucinous. This category includes seromucinous cystadenoma and seromucinous adenofibroma.


Ovarian Carcinoid Tumor

A well differentiated neuroendocrine neoplasm that arises from the ovary.  Histologically, it is classified as insular, trabecular, mucinous, or stromal carcinoid.  Clinical manifestations of carcinoid syndrome occur in approximately thirty percent of patients with insular carcinoid tumor.  Carcinoid syndrome is rare in other types of primary ovarian carcinoid tumor.  With the exception of mucinous carcinoid tumor, the prognosis is favorable.  Primary ovarian mucinous carcinoid tumor has a more aggressive clinical course and it can metastasize to other anatomic sites.


Borderline Ovarian Endometrioid Adenofibroma

An epithelial neoplasm that arises from the ovary characterized by the presence of glandular or cystic spaces which contain atypical glandular epithelial cells resembling endometrial cells. The surrounding ovarian stroma is fibrotic. There is no evidence of stromal invasion.


Stage IIA Ovarian Germ Cell Tumor AJCC v6 and v7

Stage IIA includes: T2a, N0, M0. T2a: Extension and/or implants on uterus and/or tube(s). No malignant cells in ascites or peritoneal washings. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 6th and 7th eds.)


FIGO Stage IC1 Ovarian Cancer

Stage IC ovarian cancer with surgical spill. (FIGO, 2014)


ovarian primitive germ cell tumor

A malignant tumor that arises from the ovary and is characterized by the presence of malignant germ cell components but lacks a teratoma component.


Ovarian Cancer by FIGO Stage

A category of staging terms for ovarian cancer according to the International Federation of Gynecology and Obstetrics (FIGO), 2014. AJCC ovarian cancer stage terms from the 6th and 7th editions that are synonymous to the FIGO ovarian cancer staging classification of 2014 are included as preferred terms.


Metastatic Ovarian Endometrioid Adenocarcinoma

Ovarian endometrioid adenocarcinoma that has spread from its original site of growth to another anatomic site.


Platinum-Refractory Ovarian Carcinoma

Ovarian carcinoma that progresses during platinum therapy or within one month of completing the last platinum therapy.


liposarcoma of the ovary

A malignant adipose tissue neoplasm of the ovary.


Borderline Ovarian Seromucinous Tumor/Atypical Proliferative Ovarian Seromucinous Tumor with Microinvasion

A low grade ovarian epithelial neoplasm characterized by the presence of atypical neoplastic serous and mucinous cells. Microinvasion of the ovarian stroma is present.


Benign germ cell tumor of ovary

A non-metastasizing germ cell tumor that arises from the ovary.  The vast majority of cases are benign dermoid cysts.


Ovarian Sertoli Cell Tumor

A rare, typically benign ovarian neoplasm composed of Sertoli cells. Patients may present with pseudoprecocity, menometrorrhagia, amenorrhea, hirsutism, and hoarseness.  Rarely it may invade the ovarian stroma and extend beyond the ovary.


FIGO Stage IIIC Ovarian Cancer

Ovarian cancer with macroscopic, extrapelvic, peritoneal metastasis greater than 2 cm +/- positive retroperitoneal lymph nodes. Includes extension to capsule of liver/spleen. (FIGO, 2014)


childhood choriocarcinoma of the ovary

A non-gestational choriocarcinoma that arises from the ovary and occurs in children.


Metastatic Ovarian Small Cell Carcinoma, Hypercalcemic Type

A primary small cell carcinoma of the ovary that is associated with hypercalcemia and has metastasized to other anatomic sites.


Borderline Ovarian Endometrioid Tumor/Atypical Proliferative Ovarian Endometrioid Tumor

An epithelial neoplasm that arises from the ovary characterized by the presence of glandular or cystic spaces which contain atypical glandular epithelial cells resembling endometrial cells. The surrounding ovarian stroma is often fibrotic. There is no evidence of stromal invasion.


Borderline Ovarian Serous Tumor/Atypical Proliferative Ovarian Serous Tumor with Microinvasion

A low grade ovarian epithelial neoplasm characterized by the presence of neoplastic serous epithelial cells, atypia, and microinvasion of the ovarian stroma.


Ovarian Cellular Fibroma

A benign neoplasm arising from soft tissue of the ovary. It is characterized by the presence of spindle-shaped fibroblasts and increased cellularity.


Borderline Ovarian Seromucinous Tumor/Atypical Proliferative Ovarian Seromucinous Tumor

A low grade, non-invasive ovarian epithelial neoplasm characterized by the presence of atypical neoplastic serous and mucinous cells.


Benign Ovarian Serous Tumor

A non-metastasizing neoplasm that arises from the ovary and is characterized by the presence of neoplastic epithelium that resembles the epithelium of the fallopian tube.  It includes serous cystadenoma, serous adenofibroma, serous cystadenofibroma, and surface papilloma.


Moderately Differentiated Ovarian Sertoli-Leydig Cell Tumor

A Sertoli-Leydig cell tumor of the ovary characterized by the presence of spindle-shaped gonadal stromal cells and Sertoli cells, some of which are atypical. Leydig cells are also present forming clusters at the periphery of the cellular aggregates. Metastases have been reported in a minority of patients.


Recurrent Borderline Ovarian Surface Epithelial-Stromal Tumor

The reemergence of borderline ovarian epithelial tumor after a period of remission.


Ovarian Adenomatoid Tumor

A rare benign mesothelial tumor that arises from the ovary. It is characterized by the presence of gland-like structures.


Ovarian Sex Cord-Stromal Tumor Associated with Peutz-Jeghers Syndrome

A sex cord-stromal tumor that arises from the ovary in a patient diagnosed with Peutz-Jeghers syndrome.


Stage III Ovarian Polyembryoma AJCC v6 and v7

Stage III includes: T3, N0, M0. T3: Tumor involves one or both both ovaries with microscopically confirmed peritoneal metastasis outside pelvis. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 6th and 7th eds.)


Juvenile Bilateral Ovarian Granulosa Cell Tumor

A granulosa cell tumor that involves both ovaries. It affects females in the first three decades of life.


Refractory Ovarian Adenocarcinoma

Ovarian adenocarcinoma that is resistant to treatment.


Stage III Ovarian Dysgerminoma AJCC v6 and v7


Ovarian Fibromatosis and Massive Edema

A rare benign lesion characterized by tumor-like enlargement of the ovaries due to fibroblastic proliferation with collagen deposition (fibromatosis) or stromal accumulation of edema fluid (massive edema). It presents in premenopausal patients, with menstrual abnormalities, abdominal pain, or androgenic manifestations. Most cases are unilateral. (WHO 2020)


luteoma of pregnancy

A non-neoplastic and self-limited condition that occurs during pregnancy. It is characterized by proliferation of luteinized stromal cells that replace the normal ovarian stromal cells. It is usually manifested with bilateral multinodular ovarian masses. Treatment is not required.


Ovarian Sertoli-Leydig Cell Tumor with Heterologous Elements

A Sertoli-Leydig cell tumor that arises from the ovary. It is characterized by the presence of epithelial and/or mesenchymal components and neoplasms that arise from these components.


Microsatellite Stable Ovarian Carcinoma

Ovarian carcinoma characterized by the absence of microsatellite instability.


Ovarian Trabecular Carcinoid Tumor

A carcinoid tumor that arises from the ovary and is characterized by the presence of columnar cells with abundant cytoplasm forming ribbons.  The prognosis is excellent.


Mucinous adenofibroma of borderline malignancy

A low malignant potential adenofibroma of the ovary It is characterized by an atypical epithelial hyperplasia. The epithelial cells contain intracytoplasmic mucin. There is no evidence of stromal destructive invasion.


Stage IC Ovarian Cancer AJCC v6 and v7

Stage IC includes: T1c, N0, M0. T1c: Tumor limited to one or both ovaries with any of the following: capsule ruptured, tumor on ovarian surface, malignant cells in ascites or peritoneal washings.  N0: No regional lymph node metastasis.  M0: No distant metastasis. (AJCC 6th and 7th eds.)


Refractory Malignant Ovarian Brenner Tumor

Malignant ovarian Brenner tumor that is resistant to treatment.


Stage IIB Ovarian Cancer AJCC v6 and v7

Stage IIB includes: T2b N0, M0.  T2b: Extension and/or implants on other pelvic tissues.  No malignant cells in ascites or peritoneal washings.  N0: No regional lymph node metastasis.  M0: No distant metastasis. (AJCC 6th and 7th eds.)


ovarian granulosa cell tumor

A granulosa-stromal cell tumor that arises from the ovary. It is characterized by the presence of granulosa cells that comprise at least ten percent of the cellular population. The granulosa cells are often found in a background that contains theca and fibrous cells. There are two major subtypes recognized, adult and juvenile granulosa cell tumor. Clinically, patients may present with an abdominal mass. Symptoms depend on the patient's age. The most important indicator of prognosis is tumor stage. Age over forty years at the time of the initial diagnosis, large tumor size, bilaterality, cellular atypia, and increased mitotic activity are factors indicating a potentially aggressive clinical course and relative poor prognosis.


Clear cell adenofibroma of borderline malignancy

An epithelial neoplasm with low malignant potential affecting the ovary. It is characterized by the presence of atypical glands and/or cystic spaces lined with clear or hobnail cells.


Stage IB Ovarian Cancer AJCC v6 and v7

Stage IB includes: T1b, N0, M0. T1b: Tumor limited to both ovaries; capsules intact, no tumor on ovarian surface.  No malignant cells in ascites or peritoneal washings.  N0: No regional lymph node metastasis.  M0: No distant metastasis. (AJCC 6th and 7th eds.)


Borderline Ovarian Serous Cystadenofibroma

A neoplasm of low malignant potential arising from the ovary. It is characterized by the presence of cystic spaces which are lined by atypical serous epithelial cells. The surrounding ovarian stroma is fibrotic. There is no evidence of stromal invasion.


Hilus cell tumor

A benign Leydig cell tumor which arises in the hilar area of the ovary.


Benign Brenner Tumor

A benign neoplasm that arises from the ovary and is composed of nests of transitional cells in a fibrotic stroma. There is no evidence of invasion.


Platinum-Sensitive Ovarian Carcinoma

Ovarian carcinoma that has a documented response to platinum-based chemotherapy.


Unresectable Ovarian Carcinoma

Ovarian carcinoma that is not amenable to surgical resection.


Borderline Ovarian Clear Cell Cystadenofibroma

An epithelial neoplasm with low malignant potential affecting the ovary. It is characterized by the presence of prominent cystic spaces lined with atypical clear or hobnail cells. There is no evidence of stromal invasion.


ovarian Wilms tumor

An embryonal neoplasm arising from the ovary with morphologic features resembling Wilms tumor of the kidney. It occurs during the reproductive age and may present as a rapidly growing adnexal mass.


Recurrent Ovarian Cystadenocarcinoma

The reemergence of ovarian cystadenocarcinoma after a period of remission.


Stage II Ovarian Yolk Sac Tumor AJCC v6 and v7

Stage II includes: T2, N0, M0. T2: Tumor involves one or both ovaries with pelvic extension and/or implants. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 6th and 7th eds.)


Borderline Ovarian Serous Adenofibroma

A low malignant potential adenofibroma arising from the ovary. It is characterized by an atypical epithelial hyperplasia. The epithelial cells are of serous type. There is no evidence of stromal destructive invasion.


Stage IA Ovarian Cancer AJCC v8

Stage IA includes: T1a, N0, M0. T1a: Ovarian cancer with tumor limited to one ovary (capsule intact); no malignant cells in ascites or peritoneal washings. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 8th Ed.)


Childhood Malignant Ovarian Germ Cell Tumor

A malignant germ cell tumor in children arising from the ovary.  It includes the dysgerminoma, yolk sac tumor, choriocarcinoma, embryonal carcinoma and immature teratoma.


Borderline Ovarian Clear Cell Tumor

An epithelial neoplasm with low malignant potential affecting the ovary.  It is characterized by the presence of clear or hobnail cells.  In some cases, the cells may display nuclear atypia and prominent nucleoli.  When such cells are present, they remain confined to the glands.  There is no evidence of stromal invasion.


Congenital Ovarian Failure

Ovarian failure, the cause of which is present at birth.


Stage I Ovarian Cancer AJCC v8

Stage I includes: T1, N0, M0. T1: Ovarian cancer with tumor limited to ovaries (one or both). N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 8th Ed.)


Stage IIIA Ovarian Cancer AJCC v8

Stage IIIA includes: IIIA1: T1/2, N1, M0; IIIA2: T3a, N0/N1, M0. T1: Ovarian cancer with tumor limited to ovaries (one or both). T2: Ovarian cancer with tumor involving one or both ovaries with pelvic extension below pelvic brim. T3a: Ovarian cancer with microscopic extrapelvic (above the pelvic brim) peritoneal involvement with or without positive retroperitoneal lymph nodes. N0: No regional lymph node metastasis. N1: Positive retroperitoneal lymph nodes only (histologically confirmed). M0: No distant metastasis. (AJCC 8th Ed.)


Ovarian Stromal Tumor with Minor Sex Cord Elements

A rare, benign sex cord-stromal tumor of the ovary characterized by the presence of a fibrothecomatous stroma and scattered sex cord elements. Although it is usually hormonally inactive, cases associated with endometrial hyperplasia or adenocarcinoma have been reported.


Sertoli-Leydig cell tumor, retiform

A Sertoli-Leydig cell tumor characterized by the presence of spaces that resemble rete testis (retiform elements).  These spaces form anastomosing patterns and comprise at least ten percent but less than ninety percent of the tumor.  When the retiform elements comprise ninety percent or more of the tumor, the term retiform Sertoli-Leydig cell tumor is used.  A minority of patients may have an aggressive clinical course.


Childhood Ovarian Nongerminomatous Germ Cell Tumor

A nongerminomatous germ cell tumor that arises in the ovary and occurs in children.


Ovarian Mixed Germ Cell-Sex Cord-Stromal Tumor

A neoplasm that arises from the ovary and is characterized by the presence of germ cell and sex cord-stromal tissues that are intimately admixed.  If there is no malignant germ cell component present, the clinical course is benign.


Malignant Ovarian Steroid Cell Tumor

A steroid tumor of the ovary which has an aggressive clinical course and metastasizes to other anatomic sites. It is usually of large size and is characterized by nuclear atypia, increased mitotic activity, hemorrhage, and necrosis. Sometimes, patients have abdominal metastases at presentation.


Ovarian Insular Carcinoid Tumor

A carcinoid tumor that arises from the ovary and is characterized by the presence of round cells with abundant eosinophilic cytoplasm forming cribriform patterns.  Clinical manifestations of carcinoid syndrome occur in approximately thirty percent of patients.  The prognosis is favorable.


Stage IV Borderline Ovarian Surface Epithelial-Stromal Tumor

Stage IV includes: Any T, Any N, M1.  M1: Distant metastasis (excludes peritoneal metastasis). (AJCC 6th and 7th eds.)


Ovarian Mixed Germ Cell-Sex Cord-Stromal Tumor, Unclassified

An ovarian mixed germ cell-sex cord-stromal tumor in which the neoplastic cellular infiltrate does not have the distinctive appearance of a gonadoblastoma.


Unresectable Ovarian Endometrioid Adenocarcinoma

Ovarian endometrioid adenocarcinoma that is not amenable to surgical resection.


ovarian neuroendocrine neoplasm

An epithelial neoplasm with neuroendocrine differentiation that arises from the ovary. It includes carcinoid tumor, small cell carcinoma pulmonary type, and large cell neuroendocrine carcinoma.


Ovarian Leiomyoma

A benign smooth muscle neoplasm arising from the ovary. It is usually characterized by the presence of spindle cells with cigar-shaped nuclei, interlacing fascicles, and a whorled pattern. Variants with increased cellularity, increased mitotic activity, myxoid changes, or epithelioid morphology have also been reported.


ovarian benign neoplasm

A non-metastasizing neoplasm that arises from the ovary. Representative examples include serous cystadenoma, mucinous cystadenoma, clear cell adenofibroma, benign Brenner tumor, thecoma, and fibroma.


Refractory Ovarian Mucinous Adenocarcinoma

Ovarian mucinous adenocarcinoma that is resistant to treatment.


Ovarian Torsion

Twisting of the ovary resulting in the cutoff of the blood supply to the ovary. It may develop in an enlarged or normal ovary and in the vast majority of cases is unilateral. Signs and symptoms include acute lower abdominal pain, nausea, vomiting, and fever.


Ovarian Signet Ring Cell Carcinoma

An extremely rare adenocarcinoma that arises from the ovary. It is characterized by the presence of signet ring malignant epithelial cells.


Ovarian Leydig Cell Tumor

An ovarian Leydig cell tumor for which the site of origin (ovarian hilus or ovarian stroma) cannot be determined.


Recurrent Ovarian Seromucinous Carcinoma

The reemergence of ovarian seromucinous carcinoma after a period of remission.


Stage IIC Ovarian Carcinoma

Stage IIC includes: T2c, N0, M0.  T2c: Pelvic extension and/or implants (T2a or T2b) with malignant cells in ascites or peritoneal washing.  N0: No regional lymph node metastasis.  M0: No distant metastasis. (AJCC 6th and 7th eds.)


Recurrent Platinum-Resistant Ovarian Carcinoma

The reemergence of platinum-resistant ovarian carcinoma after a period of remission.


Recurrent Ovarian Germ Cell Tumor

The reemergence of ovarian germ cell tumor after a period of remission.


Stage IA Ovarian Cancer

Stage IA includes: T1a, N0, M0.  T1a: Tumor limited to one ovary; capsule intact, no tumor on ovarian surface.  No malignant cells in ascites or peritoneal washings.  N0: No regional lymph node metastasis.  M0: No distant metastasis. (AJCC 6th and 7th eds.)


Borderline Ovarian Endometrioid Tumor/Atypical Proliferative Ovarian Endometrioid Tumor with Microinvasion

A low grade ovarian epithelial neoplasm characterized by the presence of glandular or cystic spaces which contain atypical glandular epithelial cells resembling endometrial cells. Microinvasion of the ovarian stroma is present.


Ovarian Cancer by AJCC v6 and v7 Stage

A category of staging terms for ovarian cancer according to the American Joint Committee on cancer (AJCC) 6th and 7th editions. International Federation of Gynecology and Obstetrics (FIGO) ovarian cancer staging terms prior to 2014 are included in this category if synonymous with the AJCC terms.


Stage II Ovarian Dysgerminoma AJCC v6 and v7

Stage II includes: T2, N0, M0. T2: Tumor involves one or both ovaries with pelvic extension and/or implants. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 6th and 7th eds.)


Refractory Ovarian Undifferentiated Carcinoma

Ovarian undifferentiated carcinoma that is resistant to treatment.


Metastatic Ovarian Undifferentiated Carcinoma

Ovarian undifferentiated carcinoma that has spread from its original site of growth to another anatomic site.


Recurrent Ovarian Endometrioid Adenocarcinoma

The reemergence of ovarian endometrioid adenocarcinoma after a period of remission.


Stage IV Ovarian Germ Cell Tumor AJCC v6 and v7

Stage IV includes: Any T, Any N, M1. M1: Distant metastasis (excludes peritoneal metastasis). (AJCC 6th and 7th eds.)


ovarian germ cell tumor

A neoplasm that arises from the ovary and originates from germ cells. Representative examples include teratoma, embryonal carcinoma, yolk sac tumor, and dysgerminoma.


Ovarian stromal hyperplasia

A non-neoplastic disorder that usually affects postmenopausal women.  It is characterized by proliferation of ovarian stromal cells without evidence of atypia.  There is usually bilateral ovarian involvement.  The ovaries may or may not be enlarged.


FIGO Stage IC2 Ovarian Cancer

Stage IC ovarian cancer with capsule rupture before surgery or tumor on ovarian surface. (FIGO, 2014)


ovary leiomyosarcoma

An aggressive malignant smooth muscle neoplasm, arising from the ovary. It is characterized by a proliferation of neoplastic spindle cells.


Lipid cell tumor of ovary

An ovarian tumor in which the vast majority of the cells (more than 90% of the tumor cells) resemble steroid hormone-secreting cells. It usually presents with androgenic manifestations. Approximately one-third of the cases follow a malignant clinical course.


Metastatic Microsatellite Stable Ovarian Carcinoma

Microsatellite stable ovarian carcinoma that has spread from its original site of growth to another anatomic site.


Borderline Ovarian Endometrioid Tumor

A rare, low grade ovarian epithelial neoplasm characterized by the presence of glandular or cystic spaces which contain atypical glandular epithelial cells resembling endometrial cells.


FIGO Stage IC3 Ovarian Cancer

Stage IC ovarian cancer with malignant cells in the ascites or peritoneal washings. (FIGO, 2014)


Stage IV Ovarian Dysgerminoma AJCC v6 and v7


Stage IC Ovarian Cancer AJCC v8

Stage IC includes: T1c, N0, M0. T1c: Ovarian cancer with tumor limited to one or both ovaries, with any of the following: surgical spill, capsule ruptured before surgery or tumor on ovarian surface, or malignant cells in ascites or peritoneal washings. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 8th Ed.)


Ovarian Sclerosing Stromal Tumor

A benign ovarian stromal tumor characterized by the presence of cellular areas which contain fibroblasts and round cells. The cellular areas are separated by sclerotic or edematous hypocellular tissue. Symptoms include abdominal discomfort and menstrual abnormalities.


Stage IA Ovarian Germ Cell Tumor AJCC v6 and v7

Stage IA includes: (T1a, N0, M0). T1a: Tumor limited to one ovary; capsule intact, no tumor on ovarian surface.  No malignant cells in ascites or peritoneal washings.  N0: No regional lymph node metastasis.  M0: No distant metastasis. (AJCC 6th and 7th Eds.)


Metastatic Platinum-Resistant Ovarian Carcinoma

Platinum-resistant ovarian carcinoma that has spread from its original site of growth to another anatomic site.


FIGO Stage IVB Ovarian Cancer

Ovarian cancer with hepatic and/or splenic parenchymal metastasis, metastasis to extra-abdominal organs (including inguinal lymph nodes and lymph nodes outside of the abdominal cavity). (FIGO, 2014)


Benign Ovarian Clear Cell Tumor

A non-metastasizing neoplasm that arises from the ovary.  It is characterized by a predominance of clear and hobnail benign epithelial cells.


Recurrent Ovarian Clear Cell Adenocarcinoma

The reemergence of ovarian clear cell adenocarcinoma after a period of remission.


Metastatic Ovarian Mucinous Adenocarcinoma

Ovarian mucinous adenocarcinoma that has spread from its original site of growth to another anatomic site.


Ovarian Steroid Cell Tumor, Not Otherwise Specified

A steroid cell tumor that arises from the ovary and is defined by morphologic features that do not fit into the categories of Leydig cell tumor and stromal luteoma.  These features include solid proliferations of polygonal neoplastic cells with eosinophilic and granular cytoplasm.  Grossly, these tumors usually present as large and well circumscribed masses and are usually associated with androgenic manifestations.  Approximately one third of the cases have a malignant clinical course.


Stage IB Ovarian Cancer AJCC v8

Stage IB includes: T1b, N0, M0. T1b: Ovarian cancer with tumor limited to one or both ovaries (capsules intact); no tumor on ovarian surface; no malignant cells in ascites or peritoneal washings. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 8th Ed.)


Refractory Ovarian Carcinoma

Ovarian carcinoma that is resistant to treatment.


Stage I Ovarian Polyembryoma AJCC v6 and v7

Stage I includes: (T1, N0, M0). T1: Tumor limited to ovaries (one or both).  N0: No regional lymph node metastasis.  M0: No distant metastasis. (AJCC 6th and 7th Eds.)


Ovarian Granulosa-Stromal Cell Tumor

A group of sex cord-stromal tumors that arise from the ovary.  These tumors are characterized by the presence of granulosa cells, stromal cells, and/or theca cells.  This group includes granulosa cell tumor and tumors of the thecoma/fibroma group.


ovarian melanoma

A melanoma (disease) that involves the ovary.


Refractory Ovarian Endometrioid Adenocarcinoma

Ovarian endometrioid adenocarcinoma that is resistant to treatment.


Ovarian Neuroectodermal-Type Tumor

A rare ovarian malignant tumor with neuroectodermal differentiation. It is characterized either by a small round cell proliferation or by neuronal or glial differentiation.


Sertoli-Leydig cell tumor, intermediate differentiation, with heterologous elements

An ovarian Sertoli-Leydig cell tumor of intermediate differentiation, characterized by the presence of a heterologous component, usually consisting of cartilage, skeletal muscle, or rhabdomyosarcoma.


malignant germ cell tumor of ovary

Malignant germ cell tumor of ovary is a rare ovarian cancer arising from germ cells in the ovary, frequently unilateral at diagnosis which characteristically presents during adolescence with pelvic mass, fever, vaginal bleeding and acute abdomen.


Borderline Ovarian Serous Tumor-Micropapillary Variant/Non-Invasive Low Grade Ovarian Serous Carcinoma

A non-invasive serous neoplasm that arises from the ovary and shows micropapillary and/or cribriform architectural patterns.  It is composed of round epithelial cells with scant cytoplasm and moderate nuclear atypia.


Stage IV Ovarian Yolk Sac Tumor AJCC v6 and v7

Stage IV includes: Any T, Any N, M1. M1: Distant metastasis (excludes peritoneal metastasis). (AJCC 6th and 7th eds.)


Poorly Differentiated Ovarian Sertoli-Leydig Cell Tumor, Variant with Heterologous Elements

An ovarian poorly differentiated Sertoli-Leydig cell tumor. It is characterized by the presence of a heterologous component, usually consisting of cartilage, skeletal muscle, or rhabdomyosarcoma.


Well Differentiated Ovarian Sertoli-Leydig Cell Tumor

A Sertoli-Leydig tumor of the ovary characterized by the presence of Sertoli cells in tubules without evidence of significant nuclear atypia or mitotic activity. Primitive gonadal stromal cells are not present. It usually follows a benign clinical course.


ovarian endometrioid adenofibroma

A benign neoplasm of the ovary characterized by the presence of glandular structures with endometrial-type well-differentiated cells in a fibrotic stroma.


Stage IIIA2 Ovarian Cancer AJCC v8

Stage IIIA2 includes: T3a, N0/N1, M0. T3a: Ovarian cancer with microscopic extrapelvic (above the pelvic brim) peritoneal involvement with or without positive retroperitoneal lymph nodes. N0: No regional lymph node metastasis. N1: Positive retroperitoneal lymph nodes only (histologically confirmed). M0: No distant metastasis. (AJCC 8th Ed.)


Stage I Ovarian Yolk Sac Tumor AJCC v6 and v7


Ovarian Retiform Sertoli-Leydig Cell Tumor, Variant with Heterologous Elements

A retiform Sertoli-Leydig cell tumor that arises from the ovary. It is characterized by the presence of an epithelial and/or mesenchymal heterologous component.


undifferentiated sarcoma of ovary

An aggressive high grade sarcoma that arises from the ovary.  It is characterized by the presence of marked pleomorphism and nuclear atypia in the neoplastic mesenchymal cells.  The prognosis is poor.


Stage IIIA1 Ovarian Cancer AJCC v8

Stage IIIA1 includes: T1/2, N1, M0. T1: Ovarian cancer with tumor limited to ovaries (one or both). T2: Ovarian cancer with tumor involving one or both ovaries with pelvic extension below pelvic brim. N1: Positive retroperitoneal lymph nodes only (histologically confirmed). M0: No distant metastasis. (AJCC 8th Ed.)


Stage IIC Ovarian Germ Cell Tumor AJCC v6 and v7

Stage IIC includes: T2c, N0, M0. T2c: Pelvic extension and/or implants (T2a or T2b) with malignant cells in ascites or peritoneal washing. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 6th and 7th eds.)


Ovarian Leydig Cell Tumor, Non-Hilar Type

A Leydig cell tumor that arises from the ovarian stroma.


Benign thecoma

A thecoma of the ovary that does not metastasize to other anatomic sites.


Borderline Ovarian Mucinous Tumor, Intestinal Type

A neoplasm of low malignant potential arising from the ovary.  It is characterized by the proliferation of neoplastic mucinous epithelial cells that resemble intestinal epithelial cells.  There is no evidence of stromal invasion.


ovarian sarcoma

A rare, aggressive malignant mesenchymal neoplasm that arises from the ovary. The prognosis is poor.


FIGO Stage III Ovarian Cancer

Ovarian cancer involving 1 or both ovaries with cytologically or histologically confirmed spread to the peritoneum outside the pelvis and/or metastasis to the retroperitoneal lymph nodes. (FIGO, 2014)


FIGO Stage II Ovarian Cancer

Ovarian cancer involving one or both ovaries with pelvic extension (below the pelvic brim) or primary peritoneal cancer. (FIGO, 2014)


Stage I Ovarian Embryonal Carcinoma AJCC v6 and v7

Stage I includes: (T1, N0, M0). T1: Tumor limited to ovaries (one or both).  N0: No regional lymph node metastasis.  M0: No distant metastasis. (AJCC 6th and 7th Eds.)


Advanced Ovarian Carcinosarcoma

Ovarian carcinosarcoma that has spread extensively to other anatomic sites or is no longer responding to treatment.


Recurrent Extrarenal Rhabdoid Tumor of the Ovary

The reemergence of extrarenal rhabdoid tumor of the ovary after a period of remission.


Extrarenal Rhabdoid Tumor of the Ovary

An extrarenal rhabdoid tumor affecting the ovary.


Stage III Ovarian Embryonal Carcinoma AJCC v6 and v7

Stage III includes: T3, N0, M0. T3: Tumor involves one or both both ovaries with microscopically confirmed peritoneal metastasis outside pelvis. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 6th and 7th eds.)


adenocarcinofibroma

A carcinoma arising from the ovary. It is characterized by the presence of malignant epithelial cells in a fibrotic stroma. Histologic variants include clear cell, serous, mucinous, and endometrioid adenocarcinofibroma.


Stage III Borderline Ovarian Surface Epithelial-Stromal Tumor

Stage III includes: T3, N0, M0.  T3: Tumor involves one or both ovaries with microscopically confirmed peritoneal metastasis outside pelvis.  N0: No regional lymph node metastasis.  M0: No distant metastasis. (AJCC 6th and 7th eds.)


Adult Type Ovarian Granulosa Cell Tumor

A granulosa cell tumor that affects middle aged or postmenopausal women.  Patients may present with uterine bleeding.


Stage II Ovarian Germ Cell Tumor AJCC v6 and v7

Stage II includes: T2, N0, M0. T2: Tumor involves one or both ovaries with pelvic extension and/or implants. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 6th and 7th eds.)


Recurrent Ovarian Mucinous Adenocarcinoma

The reemergence of ovarian mucinous adenocarcinoma after a period of remission.


Acquired Ovarian Failure

Ovarian failure, the cause of which is not present at birth.


serous adenocarcinofibroma of ovary

A malignant neoplasm of the ovary with an invasive epithelial component and a fibrotic stroma. The epithelial component is characterized by the presence of malignant epithelial cells of serous type, forming glandular, papillary, and solid patterns.


Stage IV Ovarian Carcinoma

A carcinoma that arises from the ovary and has metastasized to another anatomic site.


Ovarian Non-Neoplastic Disorder

A non-neoplastic disorder that affects the ovary.  Representative examples include endometriosis and polycystic ovarian disease.


Borderline Ovarian Mucinous Tumor, Endocervical-Like

A neoplasm of low malignant potential arising from the ovary.  It is characterized by the proliferation of neoplastic epithelial cells that resemble endocervical epithelial cells.  There is no evidence of stromal invasion.


Gonadotropin-Resistant Ovary Syndrome

Ovarian dysfunction due to a defect at the receptor or post receptor level, or due to the presence of antibodies against gonadotropin receptors, resulting in deficient gonadotropin signaling that causes elevated concentrations of follicle stimulating hormone and/or luteinizing hormone. A distinguishing feature is the presence of normal numbers of ova.


Borderline Ovarian Seromucinous Tumor

A low grade ovarian epithelial neoplasm characterized by the presence of atypical neoplastic serous and mucinous cells.


BRCA-Associated Ovarian Carcinoma

Ovarian carcinoma caused by deleterious germline mutation in a gene of the BRCA family.


Solid Pseudopapillary Neoplasm of the Ovary

An exceedingly rare solid pseudopapillary tumor that occurs in ectopic pancreatic tissue in the ovary.


Thecoma, luteinized

A variant of ovarian thecoma characterized by the presence of lutein cells. It is associated with a lower frequency of estrogenic manifestations compared to typical thecomas. In a minority of cases androgenic manifestations are present.


Ovarian Tumor of the Thecoma/Fibroma Group

A group of neoplasms that arise from the ovary and represent a spectrum of tumors ranging from neoplasms that are composed entirely of fibroblasts to those that are composed predominantly of theca cells.


Ovarian Luteinized Thecoma Associated with Sclerosing Peritonitis

A rare usually bilateral luteinized thecoma that arises from the ovary and is associated with sclerosing peritonitis. It occurs mostly in premenopausal women. The ovarian tumor is benign but patients may develop intestinal obstruction due to the sclerosing peritonitis.


Ovarian germ cell choriocarcinoma stage III

Stage III includes: T3, N0, M0. T3: Tumor involves one or both both ovaries with microscopically confirmed peritoneal metastasis outside pelvis. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 6th and 7th eds.)


Stage IIA Ovarian Cancer AJCC v8

Stage IIA includes: T2a, N0, M0. T2a: Ovarian cancer with tumor extension and/or implants on the uterus and/or fallopian tube(s) and/or ovaries. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 8th Ed.)


Unresectable Ovarian Undifferentiated Carcinoma

Ovarian undifferentiated carcinoma that is not amenable to surgical resection.


FIGO Stage IIIA1 Ovarian Cancer

Ovarian cancer with positive retroperitoneal lymph nodes only. (FIGO, 2014)


Platinum-Resistant Ovarian Carcinoma

Ovarian carcinoma that progresses between one and six months of completing the last platinum therapy.


Stage IIIA Ovarian Carcinoma

Ovarian cancer with positive retroperitoneal lymph nodes and/or microscopic metastasis beyond the pelvis. (FIGO, 2014)


Stage III Ovarian Yolk Sac Tumor AJCC v6 and v7


FIGO Stage IIIA1(ii) Ovarian Cancer

Ovarian cancer with positive retroperitoneal lymph nodes only. Metastasis greater than 10 mm. (FIGO, 2014)


Refractory Ovarian Seromucinous Carcinoma

Ovarian seromucinous carcinoma that is resistant to treatment.


ovarian sertoli-stromal cell tumor

A sex cord-stromal tumor that arises from the ovary and is composed entirely of, or in various combinations of, Sertoli cells, Leydig cells, and fibroblast-like cells.


Stage II Ovarian Cancer AJCC v8

Stage II includes: T2, N0, M0. T2: Ovarian cancer with tumor involving one or both ovaries with pelvic extension below pelvic brim. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 8th Ed.)


Benign Ovarian Luteinized Thecoma

A non-metastasizing thecoma of the ovary that is characterized by the presence of lutein cells.


ovarian malignant mesothelioma

A rare malignant mesothelial neoplasm that usually involves both the ovarian surface and the ovarian stroma. In most cases there is bilateral ovarian involvement.


Ovarian Seromucinous Adenofibroma

A non-metastasizing mixed epithelial neoplasm that arises from the ovary. It is characterized by the presence of more than one epithelial cell type, most often serous and endocervical-type mucinous and prominent fibrous stroma..


benign endometrioid tumor of ovary

A non-metastasizing neoplasm that arises from the ovary characterized by the presence of glands or cysts lined by benign endometrial-type cells.  It includes endometrioid adenofibroma, endometrioid cystadenofibroma, and endometrioid cystadenoma.


Stage IIIC Ovarian Germ Cell Tumor AJCC v6 and v7

Stage IIIC includes: (T3c, N0, M0); (Any T, N1, M0). T3c: Peritoneal metastasis beyond pelvis more than 2cm in greatest dimension and/or regional lymph node metastasis. N0: No regional lymph node metastasis. N1: Regional lymph node metastasis. M0: No distant metastasis. (AJCC 6th and 7th eds.)


Proliferating Brenner Tumor

A transitional cell neoplasm of low malignant potential arising from the ovary.  It is characterized by the presence of neoplastic transitional cells with atypical features without evidence of stromal invasion.


Typical Ovarian Thecoma

An ovarian thecoma characterized by the presence of cells with uniform nuclei and abundant vacuolated cytoplasm.  Mitoses are not present or, when present, are rare.


Borderline Ovarian Mucinous Tumor/Atypical Proliferative Ovarian Mucinous Tumor with Intraepithelial Carcinoma

A low grade ovarian epithelial neoplasm characterized by the presence of neoplastic mucinous epithelial cells, atypia, and the presence of intraepithelial carcinoma.


Unresectable Ovarian Mucinous Adenocarcinoma

Ovarian mucinous adenocarcinoma that is not amenable to surgical resection.


choriocarcinoma of ovary

A choriocarcinoma arising from the ovary. When it appears before puberty is of germ cell origin. In children and young adults signs and symptoms include precocious pseudopuberty and vaginal bleeding. Serum human chorionic gonadotropin is elevated. Germ cell derived ovarian choriocarcinoma should be differentiated from primary or metastatic gestational choriocarcinoma affecting the ovary. The prognosis of germ cell derived choriocarcinoma is less favorable and requires more aggressive chemotherapy treatment regimens compared to gestational choriocarcinoma.


Ovarian Dedifferentiated Carcinoma

An aggressive carcinoma arising from the ovary.  Most patients present with advanced disease.  Microscopically, it is characterized by the presence of an undifferentiated carcinomatous component and a second component of either endometrioid carcinoma or, rarely, serous carcinoma.


Borderline Ovarian Endometrioid Cystadenofibroma

An epithelial neoplasm that arises from the ovary characterized by the presence of cystic spaces which are lined by atypical glandular epithelial cells resembling endometrial cells. The surrounding ovarian stroma is fibrotic. There is no evidence of stromal invasion.


Ovarian Sex Cord Tumor with Annular Tubules

An ovarian sex cord-stromal tumor characterized by the presence of Sertoli cells forming annular tubules. It may be associated with Peutz-Jeghers syndrome. Cases associated with Peutz-Jeghers syndrome have followed a benign clinical course. Cases which are not associated with Peutz-Jeghers syndrome have been reported having a clinically malignant course.


Stage III Ovarian Germ Cell Tumor AJCC v6 and v7

Stage III includes: T3, N0, M0. T3: Tumor involves one or both both ovaries with microscopically confirmed peritoneal metastasis outside pelvis. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 6th and 7th eds.)


Stage III Ovarian Cancer AJCC v8

Stage III includes: IIIA1: T1/2, N1, M0; IIIA2: T3a, N0/N1, M0; IIIB: T3b, N0/N1, M0; IIIC: T3c, N0/N1, M0. T1: Ovarian cancer with tumor limited to ovaries (one or both). T2: Ovarian cancer with tumor involving one or both ovaries with pelvic extension below pelvic brim. T3a: Ovarian cancer with microscopic extrapelvic (above the pelvic brim) peritoneal involvement with or without positive retroperitoneal lymph nodes. T3b: Ovarian cancer with macroscopic peritoneal metastasis beyond pelvis 2 cm or less in greatest dimension with or without metastasis to the retroperitoneal lymph nodes. T3c: Ovarian cancer with macroscopic peritoneal metastasis beyond the pelvis more than 2 cm in greatest dimension with or without metastasis to the retroperitoneal lymph nodes (includes extension of tumor to capsule of liver and spleen without parenchymal involvement of either organ). N0: No regional lymph node metastasis. N1: Positive retroperitoneal lymph nodes only (histologically confirmed). M0: No distant metastasis. (AJCC 8th Ed.)


Stage II Ovarian Embryonal Carcinoma AJCC v6 and v7

Stage II includes: T2, N0, M0. T2: Tumor involves one or both ovaries with pelvic extension and/or implants. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 6th and 7th eds.)


Ovarian Retiform Sertoli-Leydig Cell Tumor

A Sertoli-Leydig cell tumor of the ovary in which anastomosing, slit-like spaces resembling rete testis constitute at least 90% of the tumor elements. It may present with estrogenic and less often androgenic manifestations. It usually follows a benign clinical course.


Borderline Ovarian Mucinous Tumor/Atypical Proliferative Ovarian Mucinous Tumor

A non-invasive neoplasm that arises from the ovary and is composed of gastrointestinal-type, mucin-containing epithelial cells.  It shows greater cellular proliferation and cytologic atypia as compared to benign ovarian mucinous tumors.


Unresectable Platinum-Resistant Ovarian Carcinoma

Platinum-resistant ovarian carcinoma that is not amenable to surgical resection.


Ovarian germ cell choriocarcinoma stage IV

Stage IV includes: Any T, Any N, M1. M1: Distant metastasis (excludes peritoneal metastasis). (AJCC 6th and 7th eds.)


Borderline Ovarian Clear Cell Tumor/Atypical Proliferative Ovarian Clear Cell Tumor

A non-invasive clear cell adenofibromatous neoplasm that arises from the ovary. It is characterized by the presence of atypia in the glandular epithelium.


Stage IIIA Choroidal and Ciliary Body Melanoma AJCC v8

Stage IIIA includes: (T2c-d, N0, M0); (T3b-c, N0, M0); (T4a, N0, M0). T2c: Tumor size category 2 without ciliary body involvement but with extraocular extension less than or equal to 5 mm in largest diameter. T2d: Tumor size category 2 with ciliary body involvement and extraocular extension less than or equal to 5 mm in largest diameter. T3b: Tumor size category 3 with ciliary body involvement. T3c: Tumor size category 3 without ciliary body involvement but with extraocular extension less than or equal to 5 mm in largest diameter. T4a: Tumor size category 4 without ciliary body involvement and extraocular extension. N0: No regional lymph node metastasis. M0: No distant metastasis.  (AJCC 8th ed.)


mosaic trisomy 16

Mosaic trisomy 16 is a rare chromosomal anomaly syndrome with a highly variable phenotype ranging from minor anomalies with normal development to intrauterine growth retardation, abnormal skin pigmentation, craniofacial and body asymmetry, cardiac (e.g. ventricular septal defect) and genital (e.g. hypospadias, cryptorchidism) anomalies, scoliosis and hearing loss to neonatal death. Additional features observed include skeletal malformations (e.g. clino/polydactyly, talipes), mild facial dysmorphism, and developmental delay.


Grade 2 Urinary Frequency, CTCAE

Limiting instrumental ADL; medical management indicated


Postneoadjuvant Therapy Stage IIIB Esophageal Squamous Cell Carcinoma AJCC v8

Stage IIIB includes: (T3, N1, M0); (T0-3, N2, M0); (T4a, N0, M0). T0: No evidence of primary tumor. T1: Tumor invades the lamina propria, muscularis mucosae, or submucosa. T2: Tumor invades the muscularis propria. T3: Tumor invades adventitia. T4a: Tumor invades the pleura, pericardium, azygos vein, diaphragm, or peritoneum. N0: No regional lymph node metastasis. N1: Tumor with metastasis in one or two regional lymph nodes. N2: Tumor with metastasis in three to six regional lymph nodes. M0: No distant metastasis. (AJCC 8th ed.)


Gastric Cardia Squamous Cell Carcinoma

A squamous cell carcinoma that arises from the gastric cardia.


Grade 2 Other Intra-Operative Injury, CTCAE


Grade 1 Other Intra-Operative Injury, CTCAE


Grade 3 Other Intra-Operative Injury, CTCAE


Grade 4 Other Intra-Operative Injury, CTCAE


Partington syndrome

A rare neurological condition that is primarily characterized by mild to moderate intellectual disability and dystonia of the hands. Other signs and symptoms may include dysarthria, behavioral abnormalities, recurrent seizures and/or an unusual gait (style of walking). Partington syndrome usually occurs in males; when it occurs in females, the signs and symptoms are often less severe. It is caused by changes (mutations) in the ARX gene and is inherited in an X-linked recessive manner. Treatment is based on the signs and symptoms present in each person.


hydroxynaphthoquinone

Any naphthoquinone in which the naphthaoquinone moiety is substituted by at least one hydroxy group.


Any member of the class of 1,4-naphthoquinones in which the naphthoquinone moiety is substituted by at least one hydroxy group.


glucocorticoid deficiency 4

Any familial glucocorticoid deficiency in which the cause of the disease is a mutation in the NNT gene.


Grade 3 Hypotension, CTCAE

Medical intervention indicated; hospitalization indicated


Stage IV Esophageal Squamous Cell Carcinoma AJCC v7

Stage IV includes: Any T, Any N, M1, Any G, Tumor location: Any.  M1: Distant metastasis.  Tumor location: Location of the primary cancer site is defined by the position of the upper (proximal) edge of the tumor in the esophagus.  (AJCC 7th ed.)


Esophageal Well Differentiated Squamous Cell Carcinoma

An esophageal squamous cell carcinoma characterized by the presence of prominent keratinization and low mitotic activity.


Clinical Stage IV Esophageal Squamous Cell Carcinoma AJCC v8

Stage IV includes: IVA: (T4, N0-2, M0); (Any T, N3, M0); IVB: (Any T, Any N, M1). T4: Tumor invades adjacent structures. N0: No regional lymph node metastasis. N1: Tumor with metastasis in one or two regional lymph nodes. N2: Tumor with metastasis in three to six regional lymph nodes. N3: Tumor with metastasis in seven or more regional lymph nodes. M0: No distant metastasis. M1: Distant metastasis. (AJCC 8th ed.)


Postneoadjuvant Therapy Stage I Esophageal Squamous Cell Carcinoma AJCC v8

Stage I includes: T0-2, N0, M0. T0: No evidence of primary tumor. T1: Tumor invades the lamina propria, muscularis mucosae, or submucosa. T2: Tumor invades the muscularis propria. N0: No regional lymph node metastasis. M0: No distant metastasis. (AJCC 8th ed.)


Cervical Esophagus Squamous Cell Carcinoma

A squamous cell carcinoma that arises from the cervical esophagus.


Clinical Stage II Esophageal Squamous Cell Carcinoma AJCC v8

Stage II includes: (T2, N0-1, M0); (T3, N0, M0). T2: Tumor invades the muscularis propria. T3: Tumor invades adventitia. N0: No regional lymph node metastasis. N1: Tumor with metastasis in one or two regional lymph nodes. M0: No distant metastasis. (AJCC 8th ed.)


Stage II Esophageal Squamous Cell Carcinoma AJCC v7

Stage II includes: IIA (T2-3, N0, M0, G1, GX, Tumor location: Upper, middle); (T2-3, N0, M0, G2-3, Tumor location: Lower, X); IIB (T2-3, N0, M0, G2-3, Tumor location: Upper, middle); (T1-2, N1, M0, Any G, Tumor location: Any).  T2: Tumor invades muscularis propria. T3: Tumor invades adventitia. T1: Tumor invades lamina propria, muscularis mucosae, or submucosa.  N0: No regional lymph node metastasis.  N1: Metastasis in 1-2 regional lymph nodes.  M0: No distant metastasis.  G1: Well differentiated.  GX: Grade cannot be assessed-stage grouping as G1.  G2: Moderately differentiated.  G3: Poorly differentiated.  Tumor location: Location of the primary cancer site is defined by the position of the upper (proximal) edge of the tumor in the esophagus.  (AJCC 7th ed.)


Pathologic Stage 0 Esophageal Squamous Cell Carcinoma AJCC v8

Stage 0 includes: Tis, N0, M0, GN/A, Tumor location: Any. Tis: High-grade dysplasia, defined as malignant cells confined to the epithelium by the basement membrane. N0: No regional lymph node metastasis. M0: No distant metastasis. GN/A: Grade non-applicable. (AJCC 8th ed.)


Stage IB Esophageal Squamous Cell Carcinoma AJCC v7

Stage IB includes: (T1, N0, M0, G2-3, Tumor location: Any); (T2-3, N0, M0, G1, GX, Tumor location: Lower, X). T1: Tumor invades lamina propria, muscularis mucosae, or submucosa.  T2: Tumor invades muscularis propria. T3: Tumor invades adventitia. N0: No regional lymph node metastasis.  M0: No distant metastasis.  G1: Well differentiated.  GX: Grade cannot be assessed-stage grouping as G1.  G2: Moderately differentiated.  G3: Poorly differentiated.  Tumor location: Location of the primary cancer site is defined by the position of the upper (proximal) edge of the tumor in the esophagus.  (AJCC 7th ed.)


Pathologic Stage IB Esophageal Squamous Cell Carcinoma AJCC v8

Stage IB includes: (T1a, N0, M0, G2-3, Tumor location: Any); (T1b, N0, M0, G1-3, Tumor location: Any); (T1b, N0, M0, GX, Tumor location: Any); (T2, N0, M0, G1, Tumor location: Any). T1a: Tumor invades the lamina propria or muscularis mucosae. T1b: Tumor invades the submucosa. T2: Tumor invades the muscularis propria. N0: No regional lymph node metastasis. M0: No distant metastasis. G1: Well-differentiated. GX: Grade cannot be assessed. G2: Moderately differentiated. G3: Poorly differentiated, undifferentiated. (AJCC 8th ed.)


Stage IIIC Esophageal Squamous Cell Carcinoma AJCC v7

Stage IIIC includes: (T4a, N1-2, M0, Any G, Tumor location: Any); (T4b, Any N, M0, Any G, Tumor location: Any); (Any T, N3, M0, Any G, Tumor location: Any).  T4a: Resectable tumor invading pleura, pericardium, or diaphragm. T4b: Unresectable tumor invading other adjacent structures, such as aorta, vertebral body, trachea, etc. N1: Metastasis in 1-2 regional lymph nodes.  N2: Metastasis in 3-6 regional lymph nodes.  N3: Metastasis in seven or more regional lymph nodes.  M0: No distant metastasis.  Tumor location: Location of the primary cancer site is defined by the position of the upper (proximal) edge of the tumor in the esophagus.  (AJCC 7th ed.)


Postneoadjuvant Therapy Stage III Esophageal Squamous Cell Carcinoma AJCC v8

Stage III includes: IIIA: (T0-2, N1, M0); IIIB: (T3, N1, M0); (T0-3, N2, M0); (T4a, N0, M0). T0: No evidence of primary tumor. T1: Tumor invades the lamina propria, muscularis mucosae, or submucosa. T2: Tumor invades the muscularis propria. T3: Tumor invades adventitia. T4a: Tumor invades the pleura, pericardium, azygos vein, diaphragm, or peritoneum. N0: No regional lymph node metastasis. N1: Tumor with metastasis in one or two regional lymph nodes. N2: Tumor with metastasis in three to six regional lymph nodes. M0: No distant metastasis. (AJCC 8th ed.)


Stage I Esophageal Squamous Cell Carcinoma AJCC v7

Stage I includes: IA (T1, N0, M0, G1, GX, Tumor location: Any); IB (T1, N0, M0, G2-3, Tumor location: Any); (T2-3, N0, M0, G1, GX, Tumor location: Lower, X). T1: Tumor invades lamina propria, muscularis mucosae, or submucosa.  T2: Tumor invades muscularis propria. T3: Tumor invades adventitia. N0: No regional lymph node metastasis.  M0: No distant metastasis.  G1: Well differentiated.  GX: Grade cannot be assessed-stage grouping as G1.  G2: Moderately differentiated.  G3: Poorly differentiated.  Tumor location: Location of the primary cancer site is defined by the position of the upper (proximal) edge of the tumor in the esophagus.  (AJCC 7th ed.)


Esophageal Squamous Cell Carcinoma by AJCC v7 Stage

A term that refers to the staging of esophageal squamous cell carcinoma according to the American Joint Committee on Cancer, 7th edition.


Pathologic Stage IA Esophageal Squamous Cell Carcinoma AJCC v8

Stage IA includes: (T1a, N0, M0, G1, Tumor location: Any); (T1a, N0, M0, GX, Tumor location: Any). T1a: Tumor invades the lamina propria or muscularis mucosae. N0: No regional lymph node metastasis. M0: No distant metastasis. G1: Well-differentiated. GX: Grade cannot be assessed. (AJCC 8th ed.)


Clinical Stage IVB Esophageal Squamous Cell Carcinoma AJCC v8

Stage IVB includes: Any T, Any N, M1. M1: Distant metastasis. (AJCC 8th ed.)