Other rare uterine cancers: neuroendocrine tumors, yolk sac tumors, choriocarcinoma

Other rare uterine cancers include neuroendocrine carcinoma, yolk sac tumors, and choriocarcinoma. Neuroendocrine carcinomas of the endometrium (NEC) are highly aggressive tumors that generally present with advanced stage disease, with a 5-year survival of 38%. The typical treatment for NEC includes surgical resection and platinum-based chemotherapy with or without radiation therapy, depending on stage at presentation. Yolk sac tumors of the endometrium are very rare with fewer than 20 case reports in the literature; median survival is 28 months. Yolk sac tumors commonly present with abnormal uterine bleeding and an elevated alpha-fetoprotein. Treatment approach is largely extrapolated from germ cell tumors in other locations, and typically includes a combination of surgery and chemotherapy (bleomycin, etoposide, and platinum). Choriocarcinoma is a type of gestational trophoblastic neoplasia that occurs in 1 in 40,000 pregnancies and 1 in 40 hydatiform moles. Choriocarcinoma is uniquely sensitive to chemotherapy, which is the major treatment modality, with single-agent chemotherapy (methotrexate or dactinomycin) utilized in low-risk disease and multiagent chemotherapy used in high-risk disease. Cure rates are greater than 90%.