Castleman Disease in Hepato-pancreaticobiliary Surgery: Friend or Foe?

Abstract Background Castleman disease is a rare lymphoproliferative disorder with low malignant potential. It is associated with risk of lymphoma & follicular dendritic cell tumours. The disease is poorly understood and is well noted in literature, the difficulty in reaching to a diagnosis. While the systemic form of the disease is a subject of Hematological work up, the unifocal disease usually consists of radiologically detected retro-peritoneal or mesenteric masses posing diagnostic challenge to hepato-pancreatico-biliary surgeons. Methods Over a period of 12 years, from 2011 till 2023, in our institution we have identified 4 cases of Castleman disease. Median age of is 60 yo with equal incidence in both sexes. In our series, in all four cases, the diagnosis was incidental while they were being worked up for other conditions. In fact, in 1 case the diagnosis was unexpected after surgery for other reason. Extensive work up including cross-sectional imaging studies failed to reach a diagnosis, therefore, the latter was established by excision. Results All four patients underwent resection that accomplished diagnosis.Three patients underwent surgery for primary excision of the tumour located in various sites of upper abdomen; one para-duodenal, one retro-hepatic and one aorto-caval. In one case, the nodule was a FDG-PET avid mesenteric mass resected in the context of planned Whipple’s procedure for cancer. The Histopathological examination confirmed diagnosis in all cases. Haematology follow-up was recommended in two cases one of which progressed to follicular dendritic cell tumor. All patients were HIV negative and HHV-8 negative. The longest follow-up is 12 years with no evidence of recurrence. Conclusions Castleman’s disease is a rare entity with very low malignant potential. Cases with unifocal disease in upper abdomen are referred to Hepato-pancreato-biliary surgeons and resection is the treatment of choice. The latter allows histopathological analysis establishing the diagnosis. This case series is useful to all HPB surgeons who may come across this diagnostic challenge for this poorly understood pathology.