伴发副肿瘤天疱疮的腹盆部Castleman病MRI表现

MRI characteristics of abdominal and pelvic Castleman's disease combined with paraneoplastic pemphigus

目的分析伴发副肿瘤天疱疮的腹盆部Castelman病的临床特点及MRI表现。方法回顾经病理证实的5例伴发副肿瘤天疱疮的腹盆部Castelman病,分析肿瘤部位、形态、大小、数量、边缘、周围结构、平扫及动态增强扫描MRI特点。结果 5例腹盆部Castleman病均为透明血管型,4例位于腹膜后,1例位于右侧髂窝;均为单发肿块,呈卵圆形,边界清楚,T1WI为等或略低信号,T2WI为高信号。动态增强扫描的动脉晚期肿块呈中度强化(n=1)或高度强化(n=4);平衡期及延迟期均呈持续强化。1例动态增强扫描表现为均匀强化;4例不均匀强化;肿瘤内裂隙状或斑片状低信号区在平衡期及延迟期逐渐缩小或消失。结论 MRI对伴发副肿瘤天疱疮腹盆部Castelman病的检出及定性诊断具有重要价值。

Objective To explore the clinical characteristics and MRI manifestations of abdominal Castleman＇s disease combined with paraneoplastic pemphigus （PNP）. Methods Five cases of abdominal Castleman＇s disease combined with PNP were confirmed pathologically. MRI features of all patients, including location, shape, size, number, margin, signal characteristics of tumors and dynamic enhanced MR images were respectively analyzed. Results Hyaline-vascular type Castleman＇s disease was found in abdomen in all 5 cases, 4 in retroperitoneum and 1 in right iliac fossa, all presented as solitary, oval and well-defined mass, iso- or hypo-intense signal on TlWI and hyper-intense signal on T2WI. On dynamic en- hanced MRI, marked enhancement （n=4） or moderate enhancement （n= 1） presented in the late arterial phase. All cases showed persistent enhancement in equilibrium and delayed phases. One tumor homogeneously enhanced on dynamic enhanced MRI, the other 4 showed inhomogeneous enhancement, and intratumoral hypo-intense areas gradually reduced or disappear. Conclusion MR examination plays an important role in the detection and qualitative diagnosis of abdominal tumors associated with PNP Castleman＇s disease.