肝脏血管周上皮样细胞瘤的诊治分析

Experience of diagnosis and treatment of hepatic perivascular epithelioid cell tumor

摘要:

目的 探讨肝脏血管周上皮样细胞瘤（PEComa）的临床特点、诊断与治疗。方法 回顾性分析2002年1月—2020年9月湖南师范大学附属第一医院收治的101例肝脏PEComa患者资料。其中，女性95例，男性6例；年龄27～63岁，中位年龄47岁。肿瘤平均直径4.7 cm。采用χ²检验或Fisher精确概率法和Logistic回归模型分析肝脏PEComa术后复发的影响因素。结果 99例患者行手术治疗，2例患者仅行超声引导下肝肿块穿刺活检。术后病理诊断均为肝脏PEComa。免疫组织化学检测人黑色素瘤标志物（Melan-A、Melanoma）和肌源性标志物SMA均阳性，99例患者血管源性标志物CD34为阳性。随访2～212个月，复发11例。肿瘤最大直径是否≥5 cm、有无初始症状及Ki-67阳性表达率是否>10%与肝脏PEComa复发有关。Logistic回归模型分析显示，肿瘤最大直径≥5 cm［R=4.893（95% CI：1.045，22.903）］和有初始症状［R=6.501（95% CI：1.384，30.551］）为肝脏PEComa复发的独立危险因素（P <0.05）。结论 肝脏PEComa好发于中年女性，影像学表现具有一定的特异性，但确诊率仍较低，确诊依靠病理学检查，外科手术为主要的治疗方式。由于PEComa的恶性潜能，尤其是存在复发的独立危险因素时，术后应密切随访。

关键词:肝脏肿瘤;血管周上皮样细胞瘤;外科治疗;预后

Abstract:

Objective To investigate the clinical features, diagnosis and treatment of liver perivascular epithelioid cell tumor (PEComa).Methods The clinicopathological data of 101 patients with hepatic PEComa admitted to the first affiliated hospital of Hunnan Normal University from January 2002 to September 2020 were studied retrospectively. Chi-square test or Fisher’s exact test and Logistic regression model were performed to analyze the influencing factors of postoperative recurrence of liver PEComa.Results Ninty-nine patients underwent surgical treatment and 2 patients only underwent ultrasound-guided liver mass puncture biopsy. All pathological diagnosis were liver PEComa. Immunohistochemical revealed that Melan-A, Melanoma and SMA were detected in all petients, and 99 patients were positive for CD34. Follow-up time: 2 months to 212 months. Among of them, 11 cases had recurrence. Statistical analysis showed that whether maximum tumor diameter ≥ 5 cm, had initial symptoms or the Ki-67 postive expression rate > 10% were related to liver PEComa recurrence, and maximum tumor diameter ≥5 cm [R=4.893 (95% CI: 1.045, 22.903)] and presence of initial symptoms [R=6.501 (95% CI: 1.384, 30.551)] were independent risk factors for liver PEComa recurrence (P < 0.05).Conclusion Liver PEComa tends to occur in middle-aged women. The Imaging features have some specificity, but the diagnosis rate is still low. Diagnosis of liver PEComa depends on pathological examination. Surgery is the main form of treatment. Patients should be followed closely after surgery ,especially with independent risk factors for recurrence.

Key words:liver neoplasms;perivascular epithelioid cell neoplasms;surgical treatment;prognosis