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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.


Commonly Used Terms

Ovary

Seminoma



Last Updated 9/28/2001

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