目的: 探讨腹膜后伴有奇异形细胞的Castleman病临床特点、病理特征、鉴别诊断,减少误诊。方法: 对我院收治的腹膜后伴有奇异形细胞的Castleman病1例的临床资料进行回顾性分析并文献复习。结果: 患者因腹部胀满不适半年余就诊。行CT检查提示腹膜后巨大肿块,考虑脂肪肉瘤遂行腹膜后肿瘤切除术。术后病理检查示淋巴滤泡数量增多,套区增宽,生发中心萎缩,滤泡间可见大量的浆细胞及少量淋巴细胞的背景中可见散在奇异形大细胞;免疫组织化学染色示:CD3、CD5、CD38、CD20及CD21均(+),CyClinD1(-),Kappa、Lambda均示部分(+)。确诊为腹膜后Castleman病,术后恢复良好出院。随访9个月未见明显复发征象。结论: 伴有奇异形细胞的Castleman病较为罕见,对奇异形细胞认识不足,易误诊为恶性肿瘤,导致过度治疗。其体征及影像学检查缺乏特异性,及时行组织病理学检查是避免误诊的关键。

Objective: To investigate the clinical features, pathological features and differential diagnosis of Castleman’s disease of the retroperitoneum with strange-shaped cells, so as to reduce misdiagnosis. Methods: The clinical data of 1 case of Castleman 's disease of the retroperitoneum with strange-shaped cells admitted to our hospital were retrospectively analyzed and the literature was reviewed. Results: The patient came to the hospital with abdominal distension and discomfort for more than half a year. CT examination showed that a large retroperitoneal mass, and retroperitoneal tumor resection was considered for liposarcoma. Postoperative pathological examination showed that the number of lymphoid follicles increased, the mantle area widened, germinal center atrophy, a large number of plasma cells and a few lymphocytes between follicles in the background could be seen scattered strange-shape large cells. Immunohistochemical staining showed that CD3, CD5, CD38, CD20 and CD21 were all (+), and CyClinD1 (-), Kappa and Lambda were all (+). The patient was diagnosed as Castleman’s disease of the retroperitoneum, and was discharged from hospital with recovered well after surgery. After 9 months of follow-up, no obvious signs of recurrence were found. Conclusion: Castleman's disease with strange-shaped cells is rare, and the lack of understanding of strange-shaped cells can easily be misdiagnosed as malignant tumors, leading to overtreatment. The signs and imaging examinations are lack of specificity. Timely histopathological examination is the key to avoid misdiagnosis.