Treatment of unicentric and multicentric Castleman disease and the role ofradiotherapy

BACKGROUND. Although surgery is considered standard therapy for unicentric Castleman disease, favorable responses to radiotherapy also have been docum ented. The authors undertook this study to analyze the clinical factors, tr eatment approaches, and outcomes of patients with unicentric or multicentri c Castleman disease, and to report die outcomes of patients with unicentric Castleman disease treated with radiotherapy. METHODS. The authors reviewed the medical records of 22 patients who had re ceived a histologic diagnosis of Castleman disease at the University of Tex as M. D. Anderson Cancer Center between 1988 and 1999. One patient with a c oncurrent histopathologic diagnosis of nonsecretory multiple myeloma was ex cluded from the study. In all patients, the diagnosis of Castleman disease was based on the results of lymph node biopsies. Disease was categorized as being either unicentric or multicentric and further subdivided into hyalin e vascular, plasma cell, or mixed variant histologic types. Clinical variab les and outcomes were analyzed according to treatment, which consisted of s urgery, chemotherapy, or radiotherapy. RESULTS. Records from 21 patients were analyzed: 12 had unicentric disease, and 9 had multicentric disease. The mean follow-up time for the entire ser ies was 51 months (median, 40 months). Four patients with unicentric diseas e were treated with radiotherapy alone: 2 remain alive and symptom free, 2 died of causes unrelated to Castleman disease and had no evidence of diseas e at last follow-up. Eight patients with unicentric disease were treated wi th complete or partial surgical resection, and all are alive and asymptomat ic. All nine patients with multicentric disease were treated with combinati on chemotherapy: five are alive with no evidence of disease, and four are a live with progressive disease. CONCLUSIONS. Surgery results in excellent rates of cure in patients with un icentric Castleman disease; radiotherapy can also achieve clinical response and cure in selected patients. Multicentric Castleman disease is a more ag gressive clinical entity and is most effectively treated with combination c hemotherapy, whereas the role of radiotherapy in its treatment remains uncl ear. Cancer 2001;92:670-6. (C) 2001 American Cancer Society.