Abstract
BackgroundCastleman's disease of the pancreas is a very rare condition that may resemble more common disease entities as well as pancreatic cancer.Case presentationHere we report the case of a 58-year-old African American male with an incidentally discovered lesion in the head of the pancreas. The specimen from his pancreaticoduodectomy contained a protuberant, encapsulated mass, exhibiting microscopic features most consistent with localized/unicentric Castleman's disease. These included florid follicular hyperplasia with mantle/marginal zone hyperplasia along with focal progressive transformation of germinal centers admixed with involuted germinal centers.ConclusionTo date, eight cases of Castleman's disease associated with the pancreas have been described in the world literature. We report the first case of unicentric disease situated within the head of the pancreas. In addition, we discuss the diagnostic dilemma Castleman's disease may present to the pancreatic surgeon and review current data on pathogenesis, treatment, and outcome.
Highlights
Castleman's disease of the pancreas is a very rare condition that may resemble more common disease entities as well as pancreatic cancer.Case presentation: Here we report the case of a 58-year-old African American male with an incidentally discovered lesion in the head of the pancreas
Castleman's disease (CD), known as angiofollicular lymph node hyperplasia, remains a rare and poorly understood disease characterized by massive growth of lymphoid tissue
CD was first described as a pathological entity in 1954 and later defined by Castleman et al, in 1956 [11]
Summary
CD is a poorly understood disease that creates both a diagnostic and therapeutic dilemma for surgeons. Complete surgical resection of unicentric disease at the time of presentation is likely to afford the best chance for cure. Radiation therapy has been used with varied success in patients who are poor surgical candidates or in those with unresectable lesions. Long term follow-up is necessary with regard to malignant sequelae. The role of surgery in multicentric disease is limited and should not be considered a realistic treatment option. Systemic therapy in the form of steroids, single or multiple drug chemotherapies have all been used with varied success. A better understanding of the pathogenesis, natural history, and diagnosis of this disorder may lead to improvement over the current modalities available for treatment
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