Diagnostic Insights and Surgical Outcomes of Glomangiopericytoma: A Tertiary Center Case Series

血管球瘤的诊断见解和手术结果:一家三级医疗中心病例系列研究

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Abstract

BACKGROUND AND OBJECTIVES: Glomangiopericytoma (GPC) is a rare, low-grade mesenchymal neoplasm that most commonly arises in the nasal cavity and paranasal sinuses. Its rarity, together with overlapping histopathological features shared with other spindle-cell tumors, makes diagnosis challenging, and available data regarding its clinical behavior and optimal management remain limited. This study aimed to present a retrospective case series of GPC from a tertiary referral center, with particular emphasis on clinical presentation, diagnostic evaluation, histopathological characteristics, surgical management, and outcomes. METHODS: Following institutional ethics approval, we conducted a retrospective review of patients treated for GPC between January 2010 and August 2025 at a tertiary care center. Collected data included demographic characteristics, clinical presentation, imaging findings, histopathological features with immunohistochemistry, surgical management, and follow-up outcomes. RESULTS: Eleven patients (six women and five men; mean age, 70.9 years) were included. The most common presenting symptoms were unilateral nasal obstruction, rhinorrhea, headache, and recurrent epistaxis. Tumors predominantly involved the nasal cavity-particularly the posterior septum, sphenoethmoidal recess, and middle turbinate-as well as the ethmoid sinus. Four cases demonstrated locoregional extension, and two required skull base reconstruction. Tumor diameter ranged from 0.8 to 4.0 cm. All patients underwent endoscopic endonasal resection. No recurrences were observed during follow-up, which ranged from 1 month to 15 years (mean, 3.54 years); however, these findings should be interpreted cautiously given the variability in follow-up duration. Immunohistochemistry consistently demonstrated nuclear β-catenin positivity and negative STAT6 expression. CONCLUSION: GPC is a rare and distinct vascular neoplasm characterized by perivascular spindle-cell proliferation and nuclear β-catenin expression. Endoscopic resection was the primary treatment modality in our cohort, and long-term surveillance remains essential because of the potential for delayed recurrence. This case series provides a descriptive overview of the clinical features, diagnostic approaches, and management of GPC.

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