Clinicopathologic features of Castleman disease: an analysis of 31 cases

ZHANG Yu; ZHAO Xue-yan; JING Cai-ping; HUO Yuan-yuan; ZHANG Tao-tao; Zhang Rui-yan; LI Gen; ZHANG Guan-jun

doi:10.3969/j.issn.1007-8096.2017.12.006

Abstract：Objective To discuss the clinicopathologic features,diagnosis,differential diagnosis and etiology of Castleman disease.Methods A retrospective analysis of the clinical features,pathological morphology,immunohistochemistry and Epstein-Barr virus was carried out in 31 cases of Castleman disease.Results There were 22 cases of unicenrtic Castleman disease and 9 cases of multicentric Castleman disease.Pathologically,there were 25 cases of hayline vascular types,3 cases cyic of plasmat type and 3 cases of mixed type in all Castleman disease.In this series,there were 11 cases which occurred in retroperitoneum,which was the main site of Castleman disease (35.5％).Immunohistochemistry can assist diagnosis and differential diagnosis.The results of EBER-ISH showed negative signal in 15 cases of Castleman disease.There were 27 cases with complete follow-up data,including 18 cases of unicenrtic type which were healed by surgical resection without recurrence;in all cases of multicentric Castleman disease;1 case recurred,other multicentric cases achieved sustained remission;1 case died of heart disease.Conclusions Castleman disease is a rare lymphoproliferative lesion,and identified diagnosis depends on pathological examination.The accuracy of clinical subtype and histopathogenic type is important for treatment and outcome in the patients.Complete surgical resection is curative for unicenrtic Castleman disease,leading to excellent long-term outcomes.Combination chemotherapy and immunological therapy are usually used in multicentric Castleman disease,and the prognosis of these patients is poor.The use of rituximab is effective on multicentric Castleman disease.