Castleman disease. A report of six cases

Abstract

Background and aims: Castleman's disease is a rare disorder situated at the boundary between reactive and neoplastic conditions. The pathogenesis is a subject of debate and the limited number of cases renders the study of the disease difficult. In our paper we present a series of six cases of Castleman disease with emphasis on the clinical presentation, pathology examination and the use of immunohistochemistry in the final diagnosis of the cases. Patients and method: The classification of the disease was based on clinical, imaging and pathological assessment. Specimens were obtained by surgical excision and were routinely processed for the pathology examination. Results: All cases were unicentric disease. Two cases were locally extensive. The clinical symptoms were related mostly to compression effects. Five case were of the hyaline-vascular type and one was included in the plasma cell variant. One case showed angiomyoid differentiation. Conclusions: We strongly believe that by understanding the pathogenesis of the precursor lesions we will gain better understanding of the pathways that lead to neoplasia and that Castleman disesase is a very interesting "natural experiment" illustrating the progression from chronic antigen stimulation to reactive lymphoid hyperplasia and finally to overt lymphoid neoplasia.