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

Journal of Rhinology Pub Date : 2026-03-01 Epub Date: 2026-03-31 DOI:10.18787/jr.2025.00056
Abdulaziz Rajeh Alanzi, Young Ha Lee, Myeong Sang Yu
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引用次数: 0

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.

血管外皮细胞瘤的诊断和手术结果:三级中心病例系列。
背景和目的:肾小球外皮细胞瘤(GPC)是一种罕见的低级别间充质肿瘤,最常见于鼻腔和鼻窦。它的罕见性,加上与其他梭状细胞肿瘤有重叠的组织病理特征,使得诊断具有挑战性,并且关于其临床行为和最佳治疗的可用数据仍然有限。本研究旨在回顾来自三级转诊中心的GPC病例系列,特别强调临床表现,诊断评估,组织病理学特征,手术处理和结果。方法:在获得机构伦理批准后,我们对一家三级医疗中心2010年1月至2025年8月期间接受GPC治疗的患者进行了回顾性研究。收集的资料包括人口统计学特征、临床表现、影像学表现、免疫组织化学组织病理学特征、手术处理和随访结果。结果:纳入11例患者(6女5男,平均年龄70.9岁)。最常见的症状是单侧鼻塞、鼻漏、头痛和反复鼻出血。肿瘤主要累及鼻腔,尤其是后隔、蝶筛隐窝和鼻甲中部以及筛窦。4例表现为局部延伸,2例需要颅底重建。肿瘤直径0.8 ~ 4.0 cm。所有患者均行鼻内窥镜切除。随访1个月~ 15年,平均3.54年,无复发;然而,考虑到随访时间的可变性,这些发现应谨慎解释。免疫组化一致显示细胞核β-catenin阳性和STAT6阴性表达。结论:GPC是一种罕见而独特的血管肿瘤,以血管周围梭形细胞增生和细胞核β-catenin表达为特征。内镜切除是本研究队列的主要治疗方式,由于可能延迟复发,长期监测仍然是必要的。本病例系列提供了GPC的临床特征、诊断方法和管理的描述性概述。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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