CONGENITAL HEMANGIOPERICYTOMA - AN UNUSUAL VASCULAR NEOPLASM OF INFANCY

Background. Hemangiopericytoma is an uncommon tumor of infants, which originates from the vascular pericytes. Although generally considered to be benign, metastases can occur. Methods. Five cases of congenital hemangiopericytoma were seen in infants; all were found in females. Th e mean age at diagnosis was 7 weeks (range, birth to 10 weeks). The le sions were located in the neck, the parotid, the axilla, and the retro peritoneum. One neck lesion was detected prenatally by ultrasonography . Each lesion was resected. The diagnosis of congenital hemangiopericy toma was established only after histologic examination. Results. No ev idence of recurrence has been found in four of the children. However, intrathoracic and intracranial metastases developed in one child with a neck lesion 28 months after the original resection, and the child wa s treated with chemotherapy, but she died of progressive disease. Conc lusions. We advocate the consideration of congenital hemangiopericytom a in the newborn infant with a vascular mass. Because congenital heman giopericytoma is unresponsive to steroid therapy, unlike other vascula r malformations, resection is the treatment of choice. Long-term posto perative follow-up is essential for the early detection of metastases.