Background

This is the ovarian counterpart for the male testicular seminoma germ cell tumor. Histologically, this tumor is indistinguishable from the seminoma. The most common presentation is an abdominal mass and selected patients may have a gonadoblastoma, a rare tumor usually associated with abnormal gonadal development.

SYNONYMS
INCIDENCE	Most common malignant germ cell tumor of the ovary 1% of all ovarian cancers
AGE RANGE-MEDIAN	5-10% of ovarian cancers in first two decades of life 20-30% of ovarian cancers during pregnancy 80% occur during 2-3rd decades Mean and median in late teens and early 20's 5%<10 years Rare >50 years
GEOGRAPHY	Higher in Japan than in Western countries

 

DISEASE ASSOCIATIONS	CHARACTERIZATION
Phenotypic females with gonadal dysgenesis	Usually arise from gonadoblastomas
Arising in testis of females with androgen insensitivity syndrome
Ataxia-telangiectasia

 

LABORATORY/RADIOLOGIC/ OTHER TESTS	CHARACTERIZATION
Laboratory Markers
Elevated serum hCG	3% of patients
Elevated serum lactic dehydrogenase	95% of patients
Other elevated serum enzymes	Alkaline phosphatase Neuron specific enolase CA125

 

GROSS APPEARANCE/ CLINICAL VARIANTS	CHARACTERIZATION
General	Solid tumors with median diameter of 15 cm Smooth or bosselated external surface Cysts, hemorrhage, and necrosis may be present Bilateral in 10% 10% have microscopic foci of tumor in the grossly normal contralateral ovary

 

HISTOLOGICAL TYPES

CHARACTERIZATION

General

Uniform cells with discrete membranes with a clear glycogen rich cytoplasm Central large nucleus with coarsely clumped chromatin and several nucleoli Arranged in several patterns: Diffuse Insular Trabecular Cord-like Numerous mitotic figures Thin to fibrous bands of stroma with mature lymphocytes (T-cells) Caseation like necrosiis frequent Sarcoidal granulomas in 20% 3% with syncytiotrohoblastic giant cells positive for hCG Occasionally luteinized stromal cells Calcifications may indicate an underlying or previous gonadoblastoma

 

SPECIAL STAINS/IMMUNOPEROXIDASE/ OTHER	CHARACTERIZATION
Special stains	PAS positive diastase sensitive glycogen rich cytoplasm
Immunoperoxidase	Positive for: PLAP Vimentin c-kit (92%) hCG focally within giant cells Some positivity for: LDH NSE Leu-7 Cytokeratin Desmin GFAP

 

DIFFERENTIAL DIAGNOSIS	KEY DIFFERENTIATING FEATURES
Yolk sac tumor-solid pattern
Embryonal carcinoma
Clear cell carcinoma
Granulosa cell tumors
Metastatic carcinomas with clear cell changes

 

PROGNOSIS AND TREATMENT	CHARACTERIZATION
Prognostic Factors	65% present in stage Ia
Treatment	Stage Ia unilateral tumors are treated conservatively Unilateral salpingo-oophorectomy with radiation

Tumors of the Ovary, Maldeveloped Gonads, Fallopian Tube, and Broad Ligament . Atlas of Tumor Pathology. Third Series. Fascicle 23. AFIP. 1998.

Ovary

Seminoma

Last Updated 9/28/2001