Objective Diagnosis of unicentric Castleman Disease (UCD) is not easy before the resection and obtainment of pathological result. We summarized 10-year experience of clinical management for retroperitoneal UCD in PUMCH.
Methods A retrospective analysis of retroperitoneal UCD between December 1st, 2009 and December 31th, 2019 in PUMCH was carried out. Epidemiological data, imaging and laboratorial examinations, and histopathological results after resection were reviewed and analyzed. The long-term follow up was performed in these retroperitoneal UCD patients.
Results 72 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 one patient got pre-operative treatment. The average size of masses was 5.59±2.86cm. 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-FDG PET/CT. Some patients had positive results on somatostatin receptor imaging, but none had positive results on 131 I-MIBG. Some patients presented the elevated level of IL-6, 24h-urinary catecholamine and tumor markers. All the patients received complete resection of masses and 96.00% had hyaline-vascular type pathology except one patient (plasma cell-type). 92.00% 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.
Conclusions Differential diagnosis of retroperitoneal UCD may be difficult according to imaging and laboratorial examinations. Complete resection is the best treatment and could provide a final pathological diagnosis.