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Context: Sinonasal glomangiopericytoma (GPC) is a rare soft tissue tumor showing perivascular myoid differentiation. We performed a retrospective study to better understand the clinical, histologic, and cytologic features of GPCs.
Design: Our pathology database (2014-2024) was searched for GPC cases. Their clinical and pathologic data were reviewed.
Results: Eight cases from 7 patients were identified (M:F, 3:4; age range, 41-77 years; average, 61 years). The tumor locations were skull base (4), frontal sinus (3), and nasal pharynx (1) (Table). Tumor size ranged from 1.6 to 2.9 cm, with bone destruction in 3 cases. All cases were surgically resected. Three patients had local GPC recurrence at 1 month, 3 months, and 35 years. Frozen sections with touch preparations were performed in 5 of 8 cases. Touch preparations exhibited scant cellularity, with oval to spindle-shaped cells and fine chromatin. Histologic examination demonstrated moderate to high cellularity with oval to spindle-shaped tumor cells arranged around thin-walled, staghorn-shaped vasculature. No cases showed necrosis or mitosis. All cases showed positivity for at least 1 of the immunostains, including vimentin, smooth muscle actin, B-catenin nuclear staining, and Factor XIIla.
Conclusions: Sinonasal GPCis a rare tumor with slight female predominance. The most common location is skull base, followed by frontal sinus. Recurrent GPC can be seen in 38% of patients even after decades; therefore, follow-up is recommended. Touch preparation has limited diagnostic value owing to the low cellularity. The presence of oval to spindle myoid cells and staghorn vessels on histology are the key for diagnosis. Immunostains can rule out other differential diagnoses.
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