RESUMEN
We report a fetal autopsy case that was diagnosed with a mole coexistent with a live fetus at an early gestation and finally showed coexisting true hermaphroditism of 46,XX/46,XY mosaicism and partial hydatidiform mole, developing metastatic gestational trophoblastic tumors in the lungs of the mother. A 23-year-old Japanese female had a mole coexistent with a fetus and showed a high chorionic gonadotropin titer in urine and serum at 10 weeks of gestation. The fetus was interrupted for gestational toxicosis and genital bleeding at 20 weeks of gestation. A chromosome analysis demonstrated 46,XX and 46,XY mosaicism in both umbilical cord blood and mole samples. Intrapelvic organs contained a testis in the one gonad, and an ovotestis in the other gonad microscopically. The testis had seminiferous tubules containing primitive germ cells, immature Sertoli cells, and cytomegalic Leydig cells. The ovary in the ovotestis had numerous primitive germ cells and a few stromal cells. Cortical cytomegaly and medullary neuroblastoma in situ were seen in the adrenals. The placenta showed focal villous hydrops and focal trophoblast hyperplasia. The patient presented multiple metastatic pulmonary tumors at 1 month after the interruption, and was treated with chemotherapy for the clinical diagnosis of gestational trophoblastic tumor metastases. She responded well and is alive without any symptoms.