巨淋巴结增生症及其合并副肿瘤天疱疮的临床、病理和CT征象分析

Giant lymph node hyperlasia and its combined paraneoplastic pemphigus： the clinical, histo-pathology and CT findings

目的研究巨淋巴结增生症及其合并副肿瘤天疱疮（PNP）的临床、病理、CT特点。方法总结分析经手术病理证实的19例巨淋巴结增生症患者的临床、病理、影像及术后随访资料。结果不伴有PNP及肺部异常的14例巨淋巴结增生症患者，大部分无症状，极少数有下腹部不适或隐痛。伴发PNP及肺部异常的患者，其临床表现、CT及病理学特点等都较特殊。病理分型：透明血管型18例，混合型1例。CT扫描：18例表现为直径2．5～15cm的单发结节或巨大软组织肿块，1例表现为左颈部多发肿大的淋巴结，直径1．5～5cm；17例边缘光滑，10例密度均匀；7例钙化，均表现为中央区多发散在斑点状分支状钙化，其中5例伴周围散在多发斑点、条状钙化，1例同时伴有周边壳状及环状钙化；形态呈圆柱状或椭球形及球形；增强扫描动脉期及延迟期持续显著强化且与动脉几乎同步强化。结论CT扫描是诊断巨淋巴结增生症的有效方法，特别是CT动态增强和延迟扫描，在明确诊断、指导手术及评价预后方面具有重要价值。对于伴发PNP及肺部异常的病例，早期诊断和手术切除体内巨淋巴结增生症瘤样病变为治愈的关键。

Objective To explore the CT findings, the clinicopathological features of giant lymph node hyperlasia and its combined paraneoplastic pemphigus. Methods The clinical features,the imaging and the follow-up data of 19 patients surgically confirmed as giant lymph node hyperlasia were analyzed. Results Clinically, the majority of patients not accompanied with paraneoplastic pemphigus abnormal pulmonary, showed asymptomatic, and only a very small number had lower abdominal discomfort or pain.The patients associated with paraneoplastic pemphigus and abnormal pulmonary manifested the special clinical manifestations, CT finding, pathological characteristics. Histopathologically, the hyaline-vascular type was found in 18 cases and mixed type in 1 case. CT scanning showed that the lesions in 18 patients appeared as larger（2.5-15 cm in diameter）, solitary, cylindrical soft-tissue masses with marked enhancement. One case was presented as multiple enlarge lymph node at left neck （1.5-5 cm in diameter）. Seventeen of 19 cases were smooth at the edge, and ten cases were uniform in density. The calcification was characterized of an arborizing and （or） flocculent pattern and central location in 7 cases, of which, 1 case circumferential distribution and 5 cases scattered with multiple spots or strip. Shapes were cylindrical and spherical or elliptical. All patients with giant lymph node hyperlasia showed marked enhancement after contrast administration at arterial phase and delay scan. Conclusion CT scanning is an effective method in diagnosis, guiding surgery and evaluating prognosis of giant lymph node hyperlasia, especially dynamic contrast-enhanced and delayed CT scanning. It is the critical way for patients complicated with paraneoplastic pemphigus and abnormal pulmonary cases, to diagnose early and resect tumor-like lesions in vivo.