Excellent Outcomes with Surgery or Radiotherapy in the Management of Castleman Disease Including a Case of Oligocentric Disease

Abstract

Background: Castleman disease (CD) is a rare polyclonal lymphoproliferative disorder of unclear etiology. Standard therapy for unicentric CD is surgical resection. Radiotherapy can be used; however, its efficacy is poorly characterized.

Patients and methods: We reviewed patients with histologically confirmed CD undergoing definitive local therapy at our institution between 1990 and 2017. Overall survival was determined from the date of diagnosis. Local progression-free survival and distant failure-free survival were determined from the date of first definitive therapy. The Kaplan-Meier method was used to analyze survival.

Results: Forty-four patients (29 female and 15 male) were identified with a median age at diagnosis of 40 years (range, 14-70 years). Thirty-five (80%) patients received surgery alone, 3 (7%) had surgery followed by radiotherapy, and 6 (14%) had radiotherapy alone. Thirty-nine (89%) patients had a single area of involvement, and 3 (7%) patients had limited regional involvement. Two (5%) patients had multicentric CD and received consolidative radiotherapy. The 3-year overall, local progression-free, and distant failure-free survival were 92%, 100%, and 100%, respectively. No distant failures were observed. The median radiation dose was 3960 cGy (range, 3600-5940 cGy) in 22 fractions (range, 18-33 fractions).

Conclusions: Unicentric CD is readily amenable to cure with local therapy. Surgical excision is preferred, but radiation appears to be an effective alternative for patients when surgery is high risk or not feasible. Patients with oligo- or multi-centric CD may experience prolonged disease-free survival with consolidative radiotherapy after partial response to systemic therapy.

Keywords: Angiofollicular lymph node hyperplasia; Giant lymph node hyperplasia; Local therapy; Lymphoproliferative disorder; Rare diseases.