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鼻腔血管球性血管外皮细胞瘤1例罕见病例报告:结合近期文献的病例报告

A rare case of glomangiopericytoma in the nasal cavity: A case report in light of recent literature.

作者信息

Almarri Firas K, Alnatheer Abdullah M, Abuhaimed Muath K, Albathi Abeer A, Alqahtani Abdulmalik Q, Tatwani Tariq

机构信息

College of Medicine, Imam Mohammed Ibn Saud Islamic University, Riyadh, Saudi Arabia.

Department of Otorhinolaryngology - Head and Neck Surgery, Prince Sultan Military Medical City, Riyadh, Saudi Arabia.

出版信息

Ann Med Surg (Lond). 2022 Apr 28;77:103685. doi: 10.1016/j.amsu.2022.103685. eCollection 2022 May.

Abstract

INTRODUCTION AND IMPORTANCE

Glomangiopericytoma (GPC) is a rare sinonasal tumor that behaves benignly with a long overall survival rate. It accounts for fewer than 0.5% of all sinonasal tumors.

CASE PRESENTATION

We report the case of a 64-year-old man who presented with recurrent episodes of epistaxis. Rhinoscopy revealed a left posterior nasal septal mass with active oozing. Computed tomography (CT) showed a well-defined soft tissue lesion in the left nasal cavity measuring 1.95 × 1.51 cm. Complete endoscopic resection was successfully performed. Histopathological findings favored the diagnosis of GPC as it revealed tumor cells positive for smooth muscle actin and β-catenin with immunopositivity for CD34.

CLINICAL DISCUSSION

Presenting symptoms of GPC are predominated by epistaxis and nasal obstruction. Since CT and MRI merely lead to a presumptive diagnosis, histopathological findings are indispensable. Complete surgical excision of GPC remains the treatment of choice with excellent prognosis, especially when immunohistochemistry is positive for actin and CD34 immunostaining is negative.

CONCLUSION

GPC is a rare indolent tumor of pericytes that has a macroscopic appearance of a nasal polyp, which may result in uncertainty in the initial diagnosis. In most cases, GPC warrants only local excision. This case report adds to the literature and helps galvanize the developing clinical guidelines for diagnosis and treatment.

摘要

引言与重要性

血管外皮细胞瘤(GPC)是一种罕见的鼻窦肿瘤,通常表现为良性,总体生存率较高。它在所有鼻窦肿瘤中所占比例不到0.5%。

病例介绍

我们报告一例64岁男性患者,反复出现鼻出血。鼻镜检查发现左后鼻中隔有一肿物且有活动性渗血。计算机断层扫描(CT)显示左侧鼻腔有一个边界清晰的软组织病变,大小为1.95×1.51厘米。成功实施了完整的内镜下切除术。组织病理学检查结果支持GPC的诊断,因为肿瘤细胞平滑肌肌动蛋白和β-连环蛋白呈阳性,CD34免疫阳性。

临床讨论

GPC的主要症状为鼻出血和鼻塞。由于CT和磁共振成像(MRI)仅能做出推测性诊断,组织病理学检查结果必不可少。完整手术切除GPC仍是首选治疗方法,预后良好,尤其是当免疫组化肌动蛋白阳性且CD34免疫染色阴性时。

结论

GPC是一种罕见的周细胞惰性肿瘤,肉眼外观类似鼻息肉,这可能导致初始诊断存在不确定性。在大多数情况下,GPC仅需局部切除。本病例报告丰富了文献,并有助于推动诊断和治疗临床指南的制定。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8a6e/9142678/36842bec0c52/gr1.jpg

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