MULTIFOCAL CONGENITAL HEMANGIOPERICYTOMAS ASSOCIATED WITH KASABACH-MERRITT SYNDROME

A newborn male with multifocal congenital hemangiopericytomas complicated by Kasabach-Merritt syndrome was treated by excision of a large hemangiopericytoma in the right mandibular area. After excision of this lesion, the associated consumptive coagulopathy abated. Spontaneous regression 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 hemangiopericytoma is indicated when there is a coexisting consumptive coagulopathy or for the definitive control of large lesions. For smaller lesions and in the absence of Kasabach-Merritt syndrome, the benign nature and predilection for spontaneous regression of congenital hemangiopericytomas render observation as the preferred strategy.