yolk sac tumor

Rare Disease

MONDO:0005744

Also Known As

endodermal sinus neoplasmendodermal sinus tumorendodermal sinus tumourhepatoid yolk sac tumorhepatoid yolk sac tumouryolk SAC tumor, malignantyolk Sac neoplasmyolk Sac tumoryolk Sac tumor site unspecifiedyolk Sac tumouryolk Sac tumour site unspecifiedyolk sac tumorinfantile embryonal carcinoma

Definition

A non-seminomatous malignant germ cell tumor composed of primitive germ cells. It is the most common malignant germ cell tumor in the pediatric population. It occurs in the infant testis, ovary, sacrococcygeal region, vagina, uterus, prostate, abdomen, liver, retroperitoneum, thorax, and pineal/third ventricle. The tumor mimics the yolk sac of the embryo and produces alpha-fetoprotein (AFP). Treatment includes: surgical resection, radiation, and chemotherapy. This tumor is very responsive to chemotherapy regimens that include cisplatinum.