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Tumors of the Ovary
Tumors of Germ Cell Origin
12
Medical
Professional
02/11/2006

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Term
Tumors of germ cell origin
Definition
--25% of ovarian tumors
--account for most ovarian tumors occuring in women younger than 20 yrs old
--Most are benign cystic teratomas
Remainder
Children/young adults
Higher incidence of malignant behavior
Similar to germ cell tumors of testis
Term
Types of ovarian germ cell tumors
Definition
Teratomas
Endodermal sinus (yolk sac) tumor
Embryonal carcinoma
Dysgerminoma
Choriocarcinoma
Mixed tumors
Term
Teratomas
Definition
--characteristically demonstrate tissue elements derived from two or three embryonic layers
--3 dinstinct forms: immature, mature (dermoid cyst), and monodermal
Term
Mature Teratomas
Definition
--hair, teeth, grummas, areas of calcification
--accounts for 90% of germ cell tumors
--BENIGN
--lined by skin, hair, etc
--may arise by reduplication of meiotic maternal chromosomes, giving rise to 46XX cells of maternal origin
Term
Monodermal teratomas
Definition
contains only a single tissue element; for example, the most common is struma ovarii, which consists entirely of thyroid tissue and can be hyperfunctional, resulting in hyperthyroidism
Term
Immature Teratoma
Definition
Solid with fetal or embryonal tissue (especially cartilage and bone)
Younger women (mean = 18)
Diagnosis based on immature neuroepithelium
Grow rapidly with frequent spread/metastases
Stage I/Grade 1 excellent prognosis
Others treated with prophylactice chemotherapy (absent disease >2 yrs good prognosis)
Term
Dysgerminoma
Definition
--MALIGNANT
--homologous to testicular seminoma
--Unusual- 2% of ovarian tumors but half of malignant germ cell tumors
~75% in the second and third decades
Usual unilateral and solid tumors
Large vesicular cell with cleared cytoplasm, well-defined cell borders and centrally placed regular nuclei and background lymphocytes (fried egg appearance)
Extremely radiosensitive
Term
Endodermal sinus (yolk sac) tumor
Definition
Second most common malignant tumor of germ cell origin
Most patients are children or young woman
Differentiation toward yolk sac structure
Rich in AFP and alpha-1-antitrypsin
Conspicuous intracellular and extracellular hyaline droplets, some of which are AFP+
Aggressive tumors
Treated with combination chemotherapy
--SCHILLER DUVAL BODIES!!
Term
Sex Cord-stromal origin tumors
Definition
Ovarian neoplasms derived from the ovarian stroma (derived from sex cords of embryonic gonad)
May emulate feminine (granulosa and theca) or masculine (Sertoli-Leydig) gonads
May be feminizing (granulosa-theca cell tumors) or masculinizing (Leydig cell tumors)
Term
Granulosa-Theca tumors
Definition
--May secrete estrogen
--Precocious puberty in prepubertal girls
--Characterized by Call-Exner bodies, small follicles filled w/ eosinophilic secretion: look like coffee bean nuclei!
Adult women they may be associated w/:
Endometrial hyperplasia and carcinoma
Cystic disease of breast
Generally indolent; 10 year survival 85%
Unilateral usually
Term
Thecoma-fibroma
Definition
These are tumors arising in the ovarian stroma that are composed of either fibroblasts (fibromas) or more plump spindle cells w/ lipid droplets (thecomas).

They are solid, hard, and gray-white
Fibroma: solid tumor consisting of bundles of spindle shaped fibroblasts; associated w/ Meigs syndrome, which is a triad of ovarian fibroma, ascites, and hydrothorax

Thecoma: demonstrates round lipid containing cells in addition to fibroblasts
Term
Sertoli-Leydig tumors
Definition
--is androgen secreting and associated w/ virilism (masculinization)
--Resemble cells of the testis
Commonly produce masculinization or at least defeminization
Peak incidence second and third decades
Solid tumors with gray to golden brown appearance
Generally benign clinical course
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