鼻腔鼻窦血管外皮瘤样肿瘤1例及文献复习  

Sinonasal hemangiopericytoma-like tumor:A case report and literature review

作　　者：王佳琪 叶任静 史雅文[1] 殷敏[1] WANG Jiaqi;YE Renjing;SHI Yawen;YIN Min(Department of Otorhinolaryngology,The First Affiliated Hospital,Nanjing Medical University,Nanjing 210029,China)

机构地区：[1]南京医科大学第一附属医院江苏省人民医院耳鼻咽喉科,南京210029

出　　处：《中国中西医结合耳鼻咽喉科杂志》2025年第2期108-110,共3页Chinese Journal of Otorhinolaryngology in Integrative Medicine

摘　　要：目的探讨鼻腔鼻窦血管外皮瘤样肿瘤(sinonasal hemangiopericytoma-like tumor,SHPCL)的临床特征、诊断及治疗策略,提高临床医师对这一罕见疾病的认识。方法回顾性分析1例74岁男性SHPCL患者的临床资料。患者以“左侧鼻腔反复出血1年伴嗅觉减退”为主诉入院,经鼻内镜、鼻窦CT及MR检查提示左侧鼻腔鼻窦占位性病变。行鼻内镜下鼻窦肿瘤切除术,术后通过病理形态学及免疫组化(SMA灶性阳性、β-Catenin核浆强阳性等)明确诊断,并对其术后恢复及随访资料进行总结。结果术中完整切除肿瘤,术后病理示梭形细胞肿瘤,结合免疫组化特征(SMA灶+、β-Catenin核浆++),确诊为SHPCL。术后未接受辅助放化疗,随访3年复查鼻内镜及CT均未见肿瘤复发或转移征象,预后良好。结论SHPCL临床罕见,确诊需依赖组织病理学及免疫组化联合分析。完整手术切除是根治该肿瘤的关键,其生物学行为多呈惰性,预后良好,但需长期规律随访以早期发现潜在复发。Objective To analyze the clinical characteristics,diagnosis,and treatment strategies of sinonasal hemangioper-icytoma-like tumor(SHPCL)and enhance clinical awareness of this rare disease.Methods A retrospective analysis was conducted on a 74-year-old male patient with SHPCL.The patient presented with recurrent left-sided epistaxis and hyposmia for one year.Preoperative evaluations including nasal endoscopy,sinus CT,and MR imaging revealed a sinonasal mass.The tumor was completely resected via endoscopic sinus surgery.Postoperative histopathological examination and immunohistochemistry(focal SMA+,strong nuclear/cytoplasmicβ-Catenin++,etc.)confirmed the diagnosis.Follow-up data were analyzed to evaluate outcomes.Results The tumor was completely excised.Histopathology showed a spindle cell tumor,and immunohistochemical findings(focal SMA+,β-Catenin nuclear/cytoplasmic++)supported the diagnosis of SHPCL.No adjuvant therapy was administered.Follow-up over 3 years revealed no recurrence or metastasis on nasal endoscopy and CT imaging,indicating favorable prognosis.Conclusion SHPCL is an extremely rare tumor requiring combined histopathological and immunohistochemical confirmation.Complete surgical resection is the cornerstone of treatment,with indolent biological behavior and excellent prognosis.Long-term regular follow-up is essential to detect potential recurrence.

关 键 词：鼻腔鼻窦肿瘤 血管外皮瘤样肿瘤 免疫组织化学 

分 类 号：R739.62[医药卫生—肿瘤]