A 10-year observational single-center study of retroperitoneal unicentric Castleman disease

Abstract

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 18 F-fluorodeoxyglucose positron emission tomography-computed tomography ( 18 F-FDG PET/CT). Some patients had positive results on somatostatin receptor imaging, but none had positive results on 131 I-metaiiodo-benzylguanidine ( 131 I-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, 18 F-PET/CT, somatostatin receptor imaging and 131 I-MIBG, can be combined for differential analysis. Complete resection is the best treatment and could provide a final pathological diagnosis.

Treatment and Outcome of Castleman Disease: A Retrospective Report of 31 Patients

“… 22 The onset of UCD is often insidious due to the frequent absence of systemic symptoms, so patients with UCD are more likely to have a single lymph node enlargement of more than 5cm. 23 Moreover, 3 patients with UCD had a history of tumor in this study. Likewise, UCD needs to be differentiated from the tumor at the corresponding site.…”

Hyaline vascular-type Castleman’s disease of the retroperitoneum

“…a localized form is a kind of benign tumor [2,3]. As a diagnostic and curative therapeutic method, complete surgical resection is the preferred therapy for the unicentric form [1,4]. After surgery, no recurrence and episodes were noted for our patient in the next several years.…”

Analysis of characteristics of four patients with adrenal unicentric Castleman disease

“…UCD can involve many sites, commonly in the neck, abdomen, and mediastinum ( 8 , 15 ). Retroperitoneum is also a common site of UCD, and the number of the adrenal UCD lesions was found to be about 12% in retroperitoneal UCD ( 7 ). Patients with adrenal UCD ranged in age from 16 to 70 years with an equal sex ratio and localization ( Table 4 ).…”

“…UCD can involve many sites, commonly in the neck, abdomen, and mediastinum (8,15). Retroperitoneum is also a common site of UCD, and the number of the adrenal UCD lesions was found to be about 12% in retroperitoneal UCD (7).…”

“…The CD was divided into unicentric CD (UCD) and multicentric CD (MCD) according to single or multiple lymph node involvement (4,5). It can involve all lymph nodes, typically in the mediastinum, cervical, retroperitoneal, and axillary areas, and around 4% of UCD occurs in the adrenal region (6)(7)(8). The clinical symptoms of CD are related to the number and location of the affected lymph nodes.…”