Perivascular epithelioid cell tumors (PEComa) in pregnancy with uterine rupture and ongoing abdominal gestation: A case report

Perivascular epithelioid cell tumors (PEComa) represent a rare family of tumors characterized by distinct histology and immunohistochemistry characteristics. Approximately one-quarter of reported cases are gynecologic in origin and associated pregnancies are rare. We report a case of PEComa in pregnancy with initial undiagnosed presentation at 18 weeks of gestation and subsequent presentation and diagnosis at 30 weeks of gestation. Abdominal pain led to the use of magnetic resonance imaging, which raised concerns about placentation abnormality and abdominal pregnancy. Exploratory laparotomy was notable for a 10 cm by 15 cm posterior uterine defect through which the placenta and amniotic sac containing the fetus were extruded. Placenta-like tissue was noted to be invading through the anterior wall of the uterus, which led to concern regarding placenta percreta. A total abdominal hysterectomy and bilateral salpingectomy were then performed, given the complete loss of normal uterine architecture. Pathology returned with findings of placenta accreta and PEComa. Indolent uterine rupture in the setting of PEComa led to an ongoing viable abdominal pregnancy. Uterine PEComa can masquerade as a placenta and lead to obstetrical complications.