ovarian yolk sac tumor
Wednesday 10 March 2004
Definition: Malignant germ cell tumor of the ovary developed as a result of differentiation of primitive malignant germ cell elements in the direction of yolk sac or vitelline structures (endodermal sinus tumor).
Usually children and young adults:
- median age 19 years
- ≈25% prepubertal at diagnosis
None present with precocious puberty, amenorrhea, or hirsutism
Vaginal bleeding in 1%
Serum α-fetoprotein level invariably elevated
Chorionic gonadotropin levels normal:
resulting in consistently negative pregnancy tests
Average diameter: 15cm
External surface: smooth, glistening
Cut surface: variegated, partially cystic, often large foci of hemorrhage and necrosis.
The cut surface is remarkably heterogeneous due to extensive hemorrhage, necrosis, and cystic degeneration.
Component of benign cystic teratoma in ≈15% of cases
- reticular or microcystic areas formed by loose meshwork lined by flat or cuboidal cells
- rounded or festooning pseudopapillary processes with central vessels (Schiller–Duval bodies)
- solid ‘undifferentiated’ areas
Mesenchyme-like component has pluripotential properties:
- usually spindle cells in a well-vascularized myxoid background
- may contain heterologous elements such as skeletal muscle
PAS-positive hyaline droplets
- hyaline globules seen in the cytoplasm of the tumor cells
- intracytoplasmic and extracellular
- nearly always present
25% have vesicular structures with eccentric constrictions surrounded by a dense spindle cell stroma: polyvesicular vitelline pattern
- scattered hCG-positive syncytiotrophoblast component
- differentiation toward hepatic, intestinal, and parietal yolk sac structures
- parietal yolk sac structures recognized by thick layers of intercellular basement membrane
- hepatoid component:
- can predominate almost to exclusion of others
- masses, nests, and broad bands of large polyhedral cells with occasional glandular formations and numerous hyaline bodies
- immunohistochemical profile similar to that of hepatocellular carcinoma, including reactivity for:
- canalicular pattern with polyclonal CEA
- areas of luteinized stromal cells: sometimes responsible for virilization
- keratin 7 (in contrast with endometrioid and clear cell ovarian carcinoma)
- WT-1 (in contrast with serous ovarian carcinoma)
- heterogeneous chemical composition
- usually stain for α-fetoprotein
- may also contain: α1-antitrypsin and basement membrane components (type IV collagen, laminin)
- parietovisceral pattern
- glandular pattern
- tubulopapillary pattern (glandulopapillary pattern)
- mucinous carcinoid component (#16096416#)
mucinous carcinoid in endodermal differentiation of ovarian yolk sac tumors (#16096416#)
46XY gonadal dysgenesis (#1346359#)
histiocytic sarcoma (#1346359#)
Recapitulates normal yolk sac elements:
- confirmed by histochemical and ultrastructural studies,4–6 including: recent detection of GATA-4: a transcription factor that regulates differentiation and function of murine yolk sac endoderm
Keratin immunoreactivity of mesenchyme-like component indicates early epithelial differentiation
ovarian endometrioid carcinoma (#15489654#)
ovarian clear cell carcinoma (#15489654#)
tubular and microcystic pattern in ovarian dysgerminoma #15767805#)
ovarian clear cell tumors
ovarian Sertoli-Leydig cell tumor
ovarian dysgerminoma (seminoma)
hepatoid yolk sac tumors and hepatoid ovarian carcinomas of probable surface epithelial origin
- Hepatoid carcinoma of ovary greatly resembles hepatocellular carcinoma, should be distinguished from yolk sac tumor with hepatoid differentiation.
Glandular formations may simulate appearance of endometrioid carcinoma.
Multidrug chemotherapy has dramatically improved survival rates.
Serial determinations of serum α-fetoprotein useful in monitoring course.
Clinical stage is most important prognostic indicator.
Polyvesicular vitelline pattern said to be associated with a good prognosis in pure form.
ovarian germ cell tumors
yolk sac tumor (endodermal sinus tumor)
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Nogales FF, Buritica C, Regauer S, Gonzalez T. Mucinous carcinoid as an unusual manifestation of endodermal differentiation in ovarian yolk sac tumors. Am J Surg Pathol. 2005 Sep;29(9):1247-51. PMID: #16096416#
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