Congenital cervical fibrosacroma with hydrops fetalis

Congenital fibrosarcoma is a rare soft-tissue tumor of infancy. We report here a case of a congenital cervical fibrosarcoma associated with hydrops fetalis. A 24-yearold female who presented with preterm labor at 31 weeks of gestation was diagnosed by ultrasound scan to have a large neck tumor and hydrops fetalis. An ex utero intrapartum treatment (EXIT) procedure was performed at cesarean section to secure the fetal airway. The neonate died 2 h after delivery because of rapid clinical deterioration associated with hydrops fetalis and pulmonary hypoplasia. Postmortem examination revealed a diagnosis of cervical fibrosarcoma. Although the specific diagnosis of congenital fibrosarcoma may be difficult without histology, the prenatal diagnosis of a fetus with a large neck mass makes the prenatal counseling and the coordinated perinatal management of the airway with an EXIT procedure feasible. Congenital fibrosarcoma is a rare soft-tissue tumor of infancy occurring most commonly at the extremities and more frequently in male than female infants (1). With increasing use of routine antenatal sonography, a few cases of prenatally diagnosed congenital fibrosarcoma have been reported (2–6). We report the prenatal sonographic finding of a congenital fibrosarcoma of the neck associated with hydrops fetalis and pulmonary hypoplasia. Because of the size and position of the tumor, the baby was expected to have severe airway obstruction after birth, which could be difficult to manage. Our case illustrates the importance of prenatal diagnosis for an appropriate and coordinated perinatal management.