蕈样肉芽肿34例临床病理特征及预后分析

Clinicopathological characteristics and prognosis of mycosis fungoides:analyses of 34 cases

目的:探讨蕈样肉芽肿的临床病理特征及预后。方法:回顾性分析1996年1月至2016年12月于辽宁省肿瘤医院住院的34例蕈样肉芽肿患者的临床资料,总结患者临床病理特征和预后。随访至2021年6月。采用Kaplan-Meier法进行生存分析,并采用log-rank检验对不同亚组之间患者的总生存(OS)进行比较。结果:34例患者中,男性22例(64.7%),女性12例(35.3%);中位发病年龄56.5岁(25~93岁)。皮疹发生部位主要为周身(23例,67.6%),肿瘤期主要表现为肿物破溃(10例,29.4%)、红肿(7例,20.6%);发病早期误诊20例(58.8%)。TNMB分期Ⅰ期3例(8.8%),Ⅱ期11例(32.4%),Ⅲ期3例(8.8%),Ⅳ期17例(50.0%);病变局限于皮肤和远处转移各17例(50.0%);远处转移患者中,内脏器官受累7例,淋巴结受累5例,骨髓或外周血受累5例;一线化疗32例,一线放疗9例,其中放化疗联合治疗7例。一线化疗32例患者中,完全缓解(CR)14例,部分缓解(PR)12例,有效率81.3%(26/32);接受一线放疗的9例患者中,CR 3例,PR 5例,有效率88.9%(8/9)。中位随访142.5个月(4~300个月),至末次随访,34例中,20例(58.8%)生存,6例(17.6%)死亡,8例(23.5%)失访。生存分析显示,全组中位OS时间未达到;相较于病变局限于皮肤的患者,远处转移患者OS差(P=0.039);远处转移患者中,淋巴结受累者较好,骨髓或外周血受累者次之,内脏器官受累者OS最差(P=0.045)。结论:蕈样肉芽肿早期临床误诊率高,诊断主要依据临床、组织学和病理学特征。早期放化疗有效率高,出现远处转移者预后差,其中淋巴结受累者OS好于骨髓或外周血受累者,内脏器官受累者OS最差。

Objective:To investigate the clinicopathological features and prognosis of mycosis fungoides.Methods:The clinical data of 34 patients with mycosis fungoides hospitalized in Liaoning Cancer Hospital from January 1996 to December 2016 were retrospectively analyzed. The clinicopathological characteristics and prognosis of the patients were summarized. The follow-up was up to June 2021. Kaplan-Meier method was used for survival analysis, and log-rank test was used to compare the overall survival (OS) of patients among different subgroups.Results:Among the 34 patients, 22 (64.7%) were male and 12 (35.3%) were female;the median onset age was 56.5 years (25-93 years). A total of 23 cases (67.6%) presented with the rash on the whole body, and the main manifestations of the rash in the tumor stage were tumor rupture (10 cases, 29.4%) and redness (7 cases, 20.6%);20 cases (58.8%) were misdiagnosed at initial onset. In the terms of TNMB stage, 3 cases were at stage Ⅰ (8.8%), 11 cases were at stage Ⅱ(32.4%), 3 cases were at stage Ⅲ (8.8%), and 17 cases were at stage Ⅳ (50.0%);17 cases (50.0%) had the lesions confined to the skin and 17 cases had distant metastasis. Among the patients with distant metastasis, 7 cases had visceral organs involvement, 5 cases had lymph nodes involvement, and 5 cases had bone marrow or peripheral blood involvement. There were 32 cases treated with first-line chemotherapy alone, 9 cases with radiotherapy alone, and 7 cases in combination with radiotherapy and chemotherapy. Among the 32 patients treated with first-line chemotherapy, 14 cases had complete remission (CR), 12 cases had partial remission (PR), and response rate (RR) was 81.3% (26 cases);among the 9 patients who received radiotherapy, 3 cases had CR, 5 cases had PR, and RR was 88.9% (8 /19). The median follow-up time was 142.5 months;until the last follow-up, 20 (58.8%) cases survived, 6 (17.6%) cases died and 8 (23.5%) cases lost the follow-up. Survival analysis showed that the median OS of the whole group was not reached. Compared with patients whose lesions were confined to the skin, patients with distant metastasis had poorer OS ( P = 0.039). Among patients with distant metastasis, those with lymph node involvement had better OS, followed by those with bone marrow or peripheral blood involvement, those with visceral organ involvement had the poorest OS ( P = 0.045). Conclusions:The clinical misdiagnosis rate of early-stage mycosis fungoides is high, and the diagnosis mainly depends on clinical, histological and pathological characteristics. Radiotherapy and chemotherapy have high efficiency for early-stage disease and the prognosis of patients with distant metastasis is poor. OS in patients with lymph node involvement is better than that in patients with bone marrow or peripheral blood involvement, and OS in patients with visceral organ involvement is the worst.