The management of unicentric and multicentric Castleman's disease - A report of 16 cases and a review of the literature

BACKGROUND. Castleman's disease (CD), or angiofollicular lymph node hyperpl asia, creates both diagnostic and therapeutic dilemmas for most physicians. For patients with this rare and poorly understood disease, the optimal the rapy is unknown. The authors report their experience during the years 1986- 1997 with this uncommon clinicopathologic entity. METHODS. Sixteen patients with a histologic diagnosis of CD were identified in the pathology database. Unicentric disease was defined as a solitary ma ss. Multicentric disease compromised patients with widespread lymphadenecto my. Clinical, radio logic, and laboratory data were analyzed to evaluate tr eatment response. RESULTS. The study group consisted of 16 patients classified into 3 clinico pathologic groups: hyaline-vascular, plasma cell, and "mixed." Of those pat ients who underwent complete surgical excision of a unicentric hyaline-vasc ular CD mass (n = 8), all remain symptom free without clinical or radiograp hic recurrence. Two patients remain asymptomatic following partial resectio n or radiation therapy for an unresectable unicentric hyaline-vascular CD m ass. Two patients with multicentric hyaline-vascular CD are currently in co mplete remission following adjuvant therapy. Multicentric plasma cell CD wa s present in a single patient. This patient (who underwent surgical and sys temic therapy) died of disease within 4 months of presentation. Three patie nts with unicentric hyaline-vascular/plasma cell-CD remain symptom free fol lowing either complete resection or observation. CONCLUSIONS. The authors recommend surgical resection for patients with the unicentric variant of CD. Surgical removal of a unicentric mass of hyaline -vascular or hyaline-vascular/plasma cell type is curative. Partial resecti on, radiotherapy, or observation alone may avoid the need for excessively a ggressive therapy. Patients with multicentric disease, either hyaline-vascu lar or plasma cell type, do not benefit from surgical management and should be candidates for multimodality therapy, the nature of which has yet to be defined. Cancer 1999;85:706-17, (C) 1999 American Cancer Society.