SINONASAL HEMANGIOPERICYTOMA - CASE-REPORT AND LITERATURE-REVIEW

Hemangiopericytoma (HPC) is a rare vascular tumor that can arise in an y organ system, but occurs most frequently in skeletal muscle. We pres ent a case of a primary sphenoid sinus HPC unusual for its spontaneous remission after biopsy. There have been approximately 55 cases of sin onasal HPC reported in the literature, representing less than 5% of al l lesions. In general, HPC behaves aggressively, demonstrating greater than 50% local recurrence and 10% metastatic disease. Although extend ed surgical resection is traditionally considered the most effective t herapy for all HPC, critical literature review does not support this m ethod of treatment for HPC occurring in the paranasal sinuses and skul l base. Long-term follow-up indicates that the majority of sinonasal H PC have a benign clinical course regardless of treatment. As a result, we dd not recommend extended resection as the initial therapy for sin onasal HPC. Instead, a period of observation coupled with serial MRI o r CT scans should be used to detect tumor progression. These indolent tumors should undergo extended resection only after progression has be en confirmed. In addition to the case report, a synopsis of the review ed literature and a summary of treatment recommendations are also pres ented.