Glomangiopericytoma, a rare sinonasal hemangiopericytoma with particular characteristics

Abstract

Introduction: Glomangiopericytoma (GPC) is an extremely rare paranasal sinuses and nasal cavity vascular neoplasm introduced and differentiated from the conventional hemangiopericytoma in 1998 by Granter et al. Up to now, to the best of our knowledge less than 250 confirmed cases have been reported in the literature. However, the exact etiology is unknown but some risk factors including trauma, hypertension, long term steroid use and pregnancy have been suggested as predisposing factors. Nasal obstruction is the most common presentation followed by intermittent epistaxis, pain, proptosis and epiphora.

Case presentation: Here we describe a case of right sided glomangiopericytoma that was completely resected with safe margins by a Weber-Ferguson approach and underwent adjuvant radiotherapy with no evidence of recurrence one year after surgery.

Discussion: Although glomangiopericytoma is very rare but it should be considered in case of confronting a unilateral vascular mass especially in pregnant female with a history of hypertension, trauma or long-term steroid usage. Definite diagnosis is based on immunohistochemistry and preoperative imaging is mandatory as endoscopic approach should be kept for small sized tumors with definitely identified origin.

Conclusion: Glomangiopericytoma is a rare tumor classified as a low-grade borderline malignancy tumor. Complete excision and long term follow up due to high rate of recurrence is required.