Castleman's Disease: Four Extraordinary Presantations and Review of Literature.

Abstract

Castleman's disease is a rare disorder of the lymphoid tissue with three possible histological variants--the hyalin-vascular type, with a good prognosis, the plasma-cellular type and the mixed type; the latter two are both more aggressive than the hyalin-vascular type. Two clinical types of this disease have already been described: the localized or unifocal type and the multicentric or multifocal type. Localized and multicentric Castleman's disease may be different clinical disorders with overlapping histologic features. Localized disease generally presented with a single enlarged lymph node or widening of the mediastinum, whereas multicentric disease is a systemic lymphoproliferative disorder characterized by lymphadenopathy, hepatosplenomegaly, constitutional symptoms, anemia, hypoalbuminemia, and hypergammaglobulinemia. Unlike the localized type, for which surgical excision is curative regardless of the histologic type, multicentric disease often necessitates aggressive systemic therapy and portends a poorer outcome. The aetiology of Castleman's disease remains unclear. Here, we report four cases of Castleman's disease, with extraordinary presentations. Tumors from retroperitoneum, mesenterium, bladder vicinity and nasopharenx locations were included. All of patients were taken to the surgery with different pre operative diagnosis. There were 1 female and 3 males; ages ranged from 16–62 years old; sizes ranged from 4– 9 cm (mean 5.5 cm). All patients presented with localized disease. One of the patients had previous NHL and lung cancer history. None of the patients demonstrated systemic involvement or signs of the POEMS syndrome. Hystopathologically 3 of the cases were classified as hyalin-vascular type and 1 of the cases were classified as plasma-cellular type. None of the patients had recurrences. Castleman ‘s disease is an uncommon cause of lymphadenopathy, and must be considered after all other causes have been eliminated. We also add the overview of the literature of the cases with extraordinary presentations.