MULTIFOCAL CONGENITAL HEMANGIOPERICYTOMAS ASSOCIATED WITH KASABACH-MERRITT SYNDROME

A newborn male with multifocal congenital hemangiopericytomas complica ted by Kasabach-Merritt syndrome was treated by excision of a large he mangiopericytoma in the right mandibular area. After excision of this lesion, the associated consumptive coagulopathy abated. Spontaneous re gression of other subcutaneous tumours over the thighs and left flank was noted by age 6 months; this illustrates the benign course of most congenital hemangiopericytomas. Surgical excision of congenital hemang iopericytoma is indicated when there is a coexisting consumptive coagu lopathy or for the definitive control of large lesions. For smaller le sions and in the absence of Kasabach-Merritt syndrome, the benign natu re and predilection for spontaneous regression of congenital hemangiop ericytomas render observation as the preferred strategy.