A giant cord hemangioma with extramedullary hematopoiesis and elevated maternal serum human chorionic gonadotropin: a case report and review of the literature
OBJECTIVE Angiomyxoma is a rare tumor of the umbilical cord and is associated with increased perinatal morbidity and mortality. However, the management of these pregnancies in the third trimester is not clearly defined. We present a case of an angiomyxoma of the umbilical cord diagnosed in the second trimester, and highlight the contribution of color Doppler imaging to the early diagnosis of cord anomalies. CASE REPORT A 29-year-old, gravida 3, para 1, woman had elevated maternal serum alpha-fetoprotein at 17 weeks of gestation. Ultrasonography at 19 weeks showed a placental mass measuring 2 x 1.5 cm over the insertion site of the umbilical cord. The mass slowly enlarged in size, from 2.72 x 1.09 cm at 21 weeks to 3.9 x 3.9 cm at 33 weeks. Beyond the cord lesion, the development of the fetus was unremarkable. At 38 weeks, a normal female infant was delivered by cesarean section due to previous history of cesarean section. A mass measuring 3.2 cm was found near the insertion site of the umbilical cord to the placenta. Pathologic examination showed proliferation of thin-walled vessels embedded in a myxoid stroma, and the endothelial cells were positive for factor VIII-related antigen. CONCLUSION Angiomyxoma is a rare tumor of the umbilical cord and should be considered when using prenatal ultrasound for detection of cystic lesion. Color Doppler imaging can easily and instantly detect perfusion through the umbilical vessels and assess cardiac function. In our case, application of color Doppler imaging for monitoring the relationship between the tumor and the adjacent vessels allowed the fetus to be delivered at term with a favorable outcome.