Abstract
Castleman's disease is a rare benign lymphoproliferative disorder of unknown etiology. The disease occurs in two clinical forms with different prognoses, treatments and symptoms: a unicentric form (UCD), which is solitary, localized, and a multicentric form characterized by generalized lymphadenopathy and systemic symptoms. This article aims to review the current literature to consolidate the evidence surrounding the curative potential of surgical treatment to the unicentric type. A systematic review of English-language literature was performed and databases (Medline, Pubmed, the Cochrane Database and grey literature) were searched to identify articles pertaining to the treatment of unicentric form of Castleman's disease. Each article was critiqued by two authors using a structured appraisal tool, and stratified according to the level of evidence. After application of inclusion criteria, 14 studies were included. There were no prospective randomized control studies identified. One meta-analysis including 278 patients with UCD reported that resective surgery is safe and should be considered the gold standard for treatment. Seven retrospective studies enhance this standpoint. Radiotherapy (RT) has been used in six studies with controversial results. We conclude that surgical resection appears to be the most effective treatment for Unicentric Castleman's Disease of the thoracic cavity. Radiotherapy can also achieve clinical response and cure in selected patients.
Highlights
Castleman’s disease is a rare benign lymphoproliferative disorder of unknown etiology
Castleman’s disease (CD) is a rare non-clonal lymph proliferative disorder of unknown etiology, which was first described as a pathologic entity in 1954 and later defined by Dr Benjamin Castleman in 1956 [1, 2]
There are 2 different clinical entities: the unicentric type (UCD) in which only one anatomic lymph node affected and the multicentric type characterized by generalized lymphadenopathy, constitutional symptoms, or
Summary
Castleman’s disease is a rare benign lymphoproliferative disorder of unknown etiology. Castleman’s disease (CD) is a rare non-clonal lymph proliferative disorder of unknown etiology, which was first described as a pathologic entity in 1954 and later defined by Dr Benjamin Castleman in 1956 [1, 2]. It is a benign tumorous process of lymphocyte cell lines, whose multiplication leads to excessive expansion of lymph nodes. Three basic histopathologic subtypes have been described: hyaline vascular (HV) (about 70-90% of individuals), plasma cell (PC), and mixed variant (MV) [3, 4] All of these pathologic types present clinically as lymphadenopathy, with or without constitutional symptoms. There are 2 different clinical entities: the unicentric type (UCD) in which only one anatomic lymph node affected and the multicentric type characterized by generalized lymphadenopathy, constitutional symptoms, or-
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