鼻腔鼻窦血管外皮细胞瘤样肿瘤 1 例报告及易误诊原因分析[1]

A Case Report and Analysis of Easily Misdiagnosed Causes of Sinonasal Hemangiopericytoma-like Tumor

摘要:

摘 要： 目的?探讨鼻腔鼻窦血管外皮瘤样肿瘤（SNHPLT）的临床特征、诊断要点及治疗策略，提高临床医师对该罕见肿瘤的认识，降低误诊率。方法?回顾性分析1例以右侧外鼻渐进性无痛性肿块为主要表现的SNHPLT患者的临床资料。患者为28岁男性，病程1年，术前CT示右侧鼻骨破坏性软组织影。于全身麻醉下行肿块完整切除术，术后标本行病理及免疫组化检查，并定期随访。结果?肿瘤完整切除，术后病理示肿瘤由短梭形细胞构成，间质富含鹿角样血管；免疫组化示CD34（+）、SMA（+）、Ki-67（约1%），确诊为SNHPLT。术后无面形改变、鼻孔狭窄等并发症，呼吸、吞咽、发声功能正常。随访6个月无复发。结论?手术完整切除是SNHPLT的首选治疗方法，病理及免疫组化是确诊的关键。该病总体预后良好，但需长期随访。

Abstract:

Sinonasal Hemangiopericytoma-like Tumor (SNHPLT) is a rare spindle cell neoplasm originating from the nasal cavity and paranasal sinuses, characterized by nonspecific clinical manifestations and a high potential for misdiagnosis. The patient presented with a progressively enlarging, non-tender mass on the external right nasal surface over one year, without associated symptoms such as nasal obstruction, rhinorrhea, headache, or visual disturbances. Following comprehensive preoperative evaluation, complete excision of the right external nasal mass was performed under general anesthesia. The resected specimen was subjected to histopathological examination, and the patient was scheduled for regular postoperative follow-up. This case report aims to elucidate the clinical features, diagnostic criteria, and surgical management strategies for SNHPLT, thereby enhancing clinicians’ awareness of this rare spindle cell tumor and reducing diagnostic inaccuracies.