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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).

Yolk sac tumor has the pathognomonic Schiller-Duval body, consisting of a central blood vessel surrounded by germ cells. Serum AFP and alpha-1-antitrypsin are elevated. It stains by IHC for AFP, CD10 and CK. It is frequently in association with a benign teratoma.


- Eosinophilic hyaline globules in a yolk sac tumor of ovary

Clinical synopsis

- 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


- Very variable:

  • 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

- Some show:

  • 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:
      • α1-antitrypsin
      • canalicular pattern with polyclonal CEA
  • areas of luteinized stromal cells: sometimes responsible for virilization


- positive:

  • pancreatin

- negative for:

    • keratin 7 (in contrast with endometrioid and clear cell ovarian carcinoma)
    • WT-1 (in contrast with serous ovarian carcinoma)

- Hyaline droplets:

  • heterogeneous chemical composition
  • usually stain for α-fetoprotein
  • may also contain: α1-antitrypsin and basement membrane components (type IV collagen, laminin)


- endodermal component

  • 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


- Invariably aneuploid

Differential diagnosis

- 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.


- PathConsult

See also

- 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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