伴发副肿瘤性天疱疮、副肿瘤性重症肌无力的纵隔滤泡树突状细胞肉瘤MSCT表现及临床病理分析

MSCT Features and Clinicopathological Analysis of Mediastinal Follicular Dendritic Cell Sarcoma with Paraneoplastic Pemphigus and Paraneoplastic Myasthenia Gravis

目的探讨伴发副肿瘤性天疱疮(PNP)、副肿瘤性重症肌无力(MG)的纵隔滤泡树突状细胞肉瘤(FDCS)多层螺旋CT(MSCT)表现及其临床、病理特点,以提高临床对该罕见病的认识,避免误诊和漏诊,辅助临床完善相关诊治,降低病死率。方法回顾性分析2012年1月至2022年12月搜集的2例经本院术后病理确诊的纵隔FDCS伴发PNP和/或MG患者的所有影像、病理及临床资料。结果本组2例均为男性患者,均同时伴发PNP,临床症状主要是口腔溃疡,全身散在分布红色及暗红色皮疹,部分融合成片,其中1例患者同时伴有MG及严重呼吸困难。CT平扫2例病变均发生于后纵隔偏右侧气管隆突下水平,单发实性肿块伴多发囊变坏死,均可见点状或分支状钙化,不同程度推压右肺下叶支气管,增强后不均匀强化,同时合并MG患者的病灶3个月内逐渐增大、囊变坏死区逐渐增多。结论FDCS伴发PNP和/或MG罕见,早期易误诊、漏诊,但其临床表现有一定特异性,且MSCT的某些特征性影像学表现均有助于该病的早期诊断,要充分认识PNP、MG与FDCS的内在联系,做到及时准确诊断以协助临床尽早展开治疗。

Objective To investigate the MSCT manifestations,clinical and pathological features of mediastinal follicular dendritic cell sarcoma with paraneoplastic pemphigus and paraneoplastic myasthenia gravis,in order to improve the clinical understanding of this rare disease,avoid misdiagnosis and missed diagnosis,assist clinical improvement of related diagnosis and treatment,and reduce the mortality.Methods All imaging,pathological and clinical data of 2 patients with mediastinal FDCS accompanied by PNP and/or MG diagnosed pathologically by our hospital from January 2012 to December 2022 were retrospectively analyzed.Results In this group,2 cases were male patients with concurrent PNP,and the main clinical symptoms were oral ulcers,red and dark red rashes scattered throughout the body,some of which were fused into tablets,and one patient was accompanied by MG and severe dyspnea.Plain CT scan showed that the lesions in both cases occurred at the subgrade level of the right tracheal carina in the middle and posterior mediastinum,with single solid mass accompanied by multiple cystic necrosis.Spot-like or branching calcification could be seen in both cases,and the right inferior lobe bronchi were pushed to different degrees,enhancing unevenly and strengthening.Meanwhile,the lesions in the patients with MG were gradually enlarged and the cystic necrosis area was gradually increased within 3 months.Conclusion FDCS accompanied by PNP and/or MG is rare and easy to be misdiagnosed and missed in early stage,but its clinical manifestations have certain specificity,and some characteristic imaging findings of MSCT are conducive to the early diagnosis of this disease.It is necessary to fully understand the internal relationship between PNP,MG and FDCS,so as to achieve timely and accurate diagnosis to assist clinical treatment as early as possible.