伴有副肿瘤性天疱疮和肺部异常的局限性Castleman病的临床与病理

目的：分析局限性Castleman病伴有副肿瘤性天疱疮（PNP)和肺浸润的临床、病理和CT表现。方法：4例患者均经手术证实。有完整的临床、实验室、CT影像和病理资料。结果：4例患者临床初诊症状均为PNP,继发性肺部异常包括闭塞性细支气管炎（3例）、多发肺梗塞（1例）和肺脓肿（1例）。CT扫描肿瘤均为单发较大的（直径5－14cm）高强化软组织肿块,病灶位于后腹膜腔3例,前纵隔1例。病理分型,透明血管型3例,中间型1例。PNP于切除肿瘤后2－32周完全消退,而3例闭塞性细支气管炎患者疗效不佳,至今仍有呼吸困难和刺激性咳嗽。结论：PNP和肺部异常为Castlaeman‘s病少见而严重合并症。早期诊断和手术切除肿瘤对患者愈合极为重要。

Localized Castleman disease with paraneoplastic pemphigus and pulmonary involvement: clinical features and histopathology

OBJECTIVE: To investigate the clinical, histopathologic and CT features of localized Castleman disease complicated with paraneoplastic pemphigus (PNP) and pulmonary involvement. METHODS: The clinical, laboratory, histopathologic records and data of computed tomographic (CT) images of 4 patients of Castleman disease with PNP were reviewed. RESULTS: All of the four patients presented with PNP which was confirmed by pathological and immunological studies and also developed lung abnormalities, including bronchiolitis obliterans (n = 3), lung abscess (n = 1) and multiple lung infarction (n = 1), 2 to 5 months after the onset of PNP. Castleman's tumor, which was found incidentally during routine examination, manifested as a solitary and huge mass (5 to 14 cm in size) which markedly enhanced homogeneously on CT in the retroperitoneum (n = 3) and mediastinum (n = 1). Histologically, three cases were of hyaline-vascular type and the other one was of mixed type. Complete remission of PNP occurred 2 weeks to 8 months after surgical resection of the tumor. The condition of two patients with bronchiolitis obliterans failed to be improved. They had been presenting dyspnea and irritable cough 3 to 15 months after surgery. CONCLUSION: PNP and lung abnormalities are rare and severe complications of localized Castleman's disease. Early diagnosis and surgery are very important for prognosis.