Single Institution Experience With Sinonasal Glomangiopericytoma: A Case Series.

Introduction

Sinonasal glomangiopericytoma (GPC) is an uncommon neoplasm, accounting for less than 0.5% of all sinonasal tumors. It is characterized as having low malignant potential, with complete surgical excision representing optimal treatment. Presenting symptoms are typically due to mass effect and vascularity of this tumor, often including unilateral nasal obstruction or epistaxis. Accounts of this tumor in the literature remain sparse.

Methods

Single institution retrospective review.

Results

Six cases of sinonasal GPC were identified from a review of the electronic medical records spanning from 2009 through 2021. Age at diagnosis ranged from 48 to 67 years, with a gender distribution of 5 males and 1 female. Most subjects presented with unilateral sinonasal obstruction of variable duration. Each underwent endoscopic resection of the mass with negative margins, and no adjuvant therapy was pursued. Pathologic specimens demonstrated a vascular patterned tumor with spindled cells surrounding vessels and were positive for smooth muscle actin and negative for cytokeratin. Active post-surgical follow-up ranged from 11 months to 10 years. All patients were without endoscopic evidence of recurrence, and 2 had post-operative imaging which showed no evidence of disease.

Conclusions

This review of 6 cases of sinonasal GPC represents the largest known series of this rare pathology in the literature to date. Based on our experience, and in agreement with the available literature, this disease is reliably managed with complete surgical excision. Adjuvant therapy can be avoided in otherwise uncomplicated cases. Although rare, GPC should be considered in the differential diagnosis of all vascular sinonasal tumors.