We present a case of primary yolk sac tumor of the endometrium. This rare tumoroccurred in a 43-year-old woman with a pure primary yolk sac tumor. The tumorresembled yolk sac tumor morphology of the ovary. Tumor cells expressed SALL4,AFP, GPC-3, and AE1/AE3 and were focal positive for PAX8. EMA, ER, and PR, amongothers, were negative. We further analyzed 29 reported cases of this rare tumorin the literature. In total, 17 of 30 patients (57%) had pure endometrial yolksac tumor, and 13 (43%) had a concomitant somatic neoplasm (endometrialadenocarcinoma was the most common). Although the average age was 52 years(range: 24–87 years), patients with pure yolk sac tumor were younger than thosewith concomitant somatic tumors, with a mean age of 44.41 years (24–68 years)versus 61.92 years (28–87 years), P = 0.008. Patients with endometrial yolk sactumor combined with somatic tumor tend to have a slightly higher stage and apoor prognosis.
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