Castleman disease. A report of six cases.

Abstract

Abstract

Backgroud 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 case of Castleman disease with emphasis on clinical presentation, pathology examination and the use of immunohistochemistry in the final diagnosis of the cases.

Patients and method: Classification of the disease was based on clinical, imaging and pathological assesment. Specimens were obtained by surgical excission 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.

Discussion: The histological aspect is highly sugestive of a chronic reactive process although some authors include the disease in the lymphproliferative disorders category. Chronic viral stimulation, especially Herpes virus 8 (HHV-8, Kaposi sarcoma virus) infection, the IL-6 signaling cascade, angiogenesis and clonal rearangements play a role in the pathogensis of CD.

Conclusions: We strongly belive that by understanding the pathogenesis of the precursor lessions we will gain better understanding of the  pathways that lead to neoplasia and that Castleman disesase is a very interesting „natural experiment” ilustrating the progression from chronic antigen stimulation to reactive lymphoid hyperplasia and finally to overt lymphoid neoplasia.