Rare Cancers Codexery

Endodermal sinus tumor

A malignant germ cell tumor common in infant testes.

Endodermal sinus tumor is a malignant germ cell cancer. It is the most common testicular tumor in children under three, where it carries a very good prognosis. In adults, it often occurs alongside other germ cell tumors such as teratoma and embryonal carcinoma.

Quick Facts

Also known as
infantile embryonal carcinoma and yolk sac tumor
Secreted tumor marker
alpha-fetoprotein (AFP)
Pathognomonic histological feature
Schiller–Duval bodies
Commonly used chemotherapy agents
  • cisplatin
  • etoposide
  • and bleomycin

Facts from the source article.

Diagnosis

The histology of endodermal sinus tumor is variable but typically includes malignant endodermal cells that secrete alpha-fetoprotein (AFP). AFP can be detected in tumor tissue, serum, cerebrospinal fluid, urine, and, in fetal cases, amniotic fluid. When biopsy and AFP results conflict, the finding indicating EST dictates treatment, because EST may exist as small malignant foci within a larger teratoma that biopsy misses. GATA-4, a transcription factor, may also aid diagnosis. Diagnosis in pregnant women and infants is complicated by their naturally high AFP levels; in pregnant women, maternal serum AFP testing is used, while in infants, tumor marker AFP must be interpreted with age-specific reference tables. EST displays many morphologic patterns, including reticular, endodermal sinus-like, microcystic, papillary, solid, glandular, alveolar, polyvesicular vitelline, enteric, and hepatoid. Schiller–Duval bodies, seen in the endodermal sinus-like pattern, are pathognomonic. Rarely, EST occurs in the vagina.

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