Hemangiopericytoma is a rare vascular tumor featuring the uncontrolled proliferation of pericytes. This tumor tends to develop in subcutaneous tissue and skeletal muscle. The localization of hemangiopericytoma in the orbit is particularly rare. Clinical signs and symptoms are, in order of frequency, proptosis, perception of an intraorbital mass, pain, diplopia, reduction of visus, tumescence, and ecchymoses of the eyelids. Hemangiopericytomas have higher potential of relapse, local invasiveness, and the possibility of producing distant metastases. Surgery must be as radical as possible to avoid incomplete tumor resection and high frequency of relapse together with increased duration of the disease and a higher risk of malignant development. The current report is a study of two cases of hemangiopericytoma in the orbital cavity treated at the Unit of Maxillo-Facial Surgery of the University of "La Sapienza" in Rome. The present data demonstrate that complete en bloc excision of the mass with its capsule is important to avoid the risk of relapse. To this end, an appropriate first surgical treatment should be chosen so as to obtain a wide "surgical light" and complete view of the mass. In the reported cases, the use of a combined anterolateral approach was preferred to attack the tumor from different sides. Indeed, such an approach allows one to obtain optimal exposition of the orbital region and temporal and infratemporal fossae contemporaneously.

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http://dx.doi.org/10.1097/00001665-200401000-00029DOI Listing

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