Castleman’s Disease: A Unicentric Case of Abdominal Origin and Literature Review

Abstract

Castleman’s disease (CD) is a rare lymphoproliferative disorder, which may be unicentric or multicentric. We present the case of a young man with the hyaline vascular (HV) variant of unicentric CD, with a review of cases of CD in the abdominal cavity. A 38-year-old man presented with post-meal epigastric pain and lactose intolerance that had started two months earlier. A solitary 7 cm retroperitoneal mass was detected using computed tomography (CT) scan. The mass was surgically resected and histological examination revealed CD of the HV variant. The symptoms resolved following the operation and the patient was free of disease at 1-year follow-up. A comprehensive literature review yielded reports of a further 214 patients with CD in the abdominal cavity. The female: male ratio was 1.64:1 (133 females, 81 males). The multicentric type of the disease was reported in 9/194 (4.6%) and the unicentric type in 185/194 (95.4%). The HV variant of the disease was reported in 168/191 patients (87.9%), the plasma cell variant in 13/191 (6.8%), the mixed variant in 9/191 (4.7%) and the stroma rich variant in 1/191 (0.5%). Regarding the location, 106/187 (56.6%) of the lesions were found in the retroperitoneal space, 34/187 (18.2%) in the mesentery, 8/187 (4.1%) in the liver and biliary tract, 12/187 (6.3%) adjacent to the pancreas and 17/187 (9%) in an unspecified region of the abdomen. Although further research is required to identify the exact etiology of the disease, the HV variant of CD, which is the most common, can be effectively treated with surgical resection. The plasma cell type of CD is a more serious condition and the high risk associated with misdiagnosis and delay in its treatment renders high clinical suspicion and early identification of this variant of the utmost importance.