Department of Urology, Peking Union Medical College Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, Beijing, China.
Medicine (Baltimore). 2021 Mar 12;100(10):e25088. doi: 10.1097/MD.0000000000025088.
Diagnosis of unicentric Castleman disease (UCD) is not easy before the resection and obtainment of pathological result. We retrospectively summarized 10-year experience of clinical evaluation and management for retroperitoneal UCD in Peking Union Medical College Hospital (PUMCH) between December 1, 2009 and December 31, 2019. Seventy two UCD patients with pathological diagnosis after resection were screened out. Among them 25 patients had retroperitoneal UCD. The average age of the 25 patients was 43.80 ± 12.79, and 52.00% were male. No patients had systemic symptoms, and 1 patient got preoperative treatment. The average size of masses was 5.59 ± 2.86 cm. The UCD sites included kidney, adrenal area, perinephric area, pancreas, peripancreatic area, area of descending part of duodenum, periaortic area or beside iliac artery, and others. The masses presented different degree of enhancement on CT scans and hypoecho or isoecho on ultrasound. Increased metabolism could be found on 18F-fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG PET/CT). Some patients had positive results on somatostatin receptor imaging, but none had positive results on 131I-metaiiodo-benzylguanidine (131I-MIBG). Some patients presented the elevated level of interleukin-6 (IL-6), 24hour-urinary catecholamine and tumor markers. All the patients received complete resection of masses and 96.00% had hyaline-vascular type pathology except 1 patient (plasma cell-type). Ninety two percent patients received a long-term follow-up with an average follow-up time of 35.48 ± 33.90 months. No patients died or experienced relapse during follow-up. Differential diagnosis of retroperitoneal UCD may be difficult according to imaging and laboratorial examinations. Differential diagnosis with pheochromocytomas/paragangliomas should be taken into special consideration. Different imaging examinations, such as CT/MRI, 18F-PET/CT, somatostatin receptor imaging and 131I-MIBG, can be combined for differential analysis. Complete resection is the best treatment and could provide a final pathological diagnosis.
诊断局灶型Castleman 病（UCD）并不容易，需要在切除并获得病理结果之前进行。我们回顾性总结了 2009 年 12 月 1 日至 2019 年 12 月 31 日北京协和医院（PUMCH）10 年来经切除术后病理诊断为 UCD 的患者的临床评估和治疗经验。共筛选出 72 例有病理诊断的 UCD 患者，其中 25 例为腹膜后 UCD。25 例患者的平均年龄为 43.80±12.79 岁，52.00%为男性。无系统性症状，1 例患者接受了术前治疗。肿块的平均大小为 5.59±2.86cm。UCD 部位包括肾脏、肾上腺区、肾周区、胰腺、胰周区、十二指肠降段区、腹主动脉旁或髂动脉旁及其他部位。CT 扫描显示肿块呈不同程度强化，超声呈低回声或等回声。18F-氟脱氧葡萄糖正电子发射断层扫描-计算机断层扫描（18F-FDG PET/CT）显示代谢增加。一些患者的生长抑素受体显像呈阳性，但 131I-间碘苄胍（131I-MIBG）显像均为阴性。部分患者白细胞介素-6（IL-6）、24 小时尿儿茶酚胺和肿瘤标志物水平升高。所有患者均行肿块完全切除术，除 1 例（浆细胞型）外，其余均为透明血管型病理（96.00%）。92.00%的患者接受了长期随访，平均随访时间为 35.48±33.90 个月。随访期间无患者死亡或复发。根据影像学和实验室检查，腹膜后 UCD 的鉴别诊断可能较为困难。应特别考虑与嗜铬细胞瘤/副神经节瘤的鉴别诊断。CT/MRI、18F-PET/CT、生长抑素受体显像和 131I-MIBG 等不同影像学检查可结合进行鉴别分析。完全切除是最佳治疗方法，可提供最终的病理诊断。
