Treatment Guidelines and Outcome
Wide surgical excision is the mainstay of treatment of sinonasal hemangiopericytomas (Castelnuovo et al. 2003). In order to minimize intraoperative bleeding, a preoperative selective embolization may be effective (Catalano et al. 1996; Serrano et al. 2002), but it should not be routinely indicated (Hekkenberg et al. 1997). The most accepted surgical approaches for sinonasal hemangiopericytomas are the external ones, such as lateral rhinotomy, midfacial degloving, craniofacial resection, infratemporal routes. In recent years, however, also endonasal endoscopic resection for selected lesions has been successfully adopted (Bhattacharyya et al. 1997; Serrano et al. 2002; Castelnuovo et al. 2003).
Radiotherapy should be reserved to patients with advanced and/or unresectable lesions, whereas chemotherapy appears reliable only with the aim of palliation, in presence of systemic spreading. Adjuvant radiotherapy may be also used in presence of incomplete surgical resection (Hervé et al. 1999).
Prognosis appears to be significantly influenced only by tumor stage at diagnosis and completeness of surgical resection (Catalano et al. 1996). Moreover, local recurrence and metastatic potential are significantly lower in paranasal sinuses than in other sites. In the literature review performed at Mount Sinai Hospital, overall recurrence rate, in most cases due to incomplete surgical resection, reached 18% (22 out of 119 cases), distant metastases occurred in 2.5% (3/119), whereas 4 patients (3.3%) died of the disease (Catalano et al. 1996). By comparing these data to those regarding axial-skeletal heman-giopericytomas, it is possible to highlight the different biologic aggressiveness. Non-sinusal lesions, in fact, are characterized by higher local recurrence, distant metastases, and mortality rate (up to 50%, 65%, and 60%, respectively) (Catalano et al. 1996; Hekkenberg et al. 1997). Recurrences may occur even 17 years after treatment, so that follow-up should be prolonged, possibly for a life-long period (Catalano et al. 1996; Hervé et al. 1999).
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