Excellent outcomes with surgery or radiotherapy in the management of Castleman disease including a case of oligocentric disease Journal Article


Authors: Beckham, T. H.; Yang, J. C.; Chau, K. W.; Noy, A.; Yahalom, J.
Article Title: 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. © 2020 Elsevier Inc. Optimal management of Castleman disease (CD) is poorly understood. We reviewed the outcomes of local therapy in managing CD at our institution. Surgery and radiotherapy were both effective for local control and are curative for unicentric disease. Consolidative local therapy after systemic therapy may offer a new management option for patients with multicentric CD. © 2020 Elsevier Inc.
Keywords: adolescent; adult; cancer chemotherapy; clinical article; treatment response; aged; cancer surgery; excision; overall survival; fatigue; intensity modulated radiation therapy; systemic therapy; cancer radiotherapy; outcome assessment; positron emission tomography; follow up; lymph node dissection; c reactive protein; progression free survival; multiple cycle treatment; local therapy; maintenance therapy; cyclophosphamide; histology; rash; interleukin 6; muscle weakness; joint swelling; adjuvant radiotherapy; lymph node biopsy; induction chemotherapy; erythrocyte sedimentation rate; night sweat; hepatosplenomegaly; rare diseases; cervical lymphadenopathy; angiofollicular lymph node hyperplasia; giant lymph node hyperplasia; lymphoproliferative disorder; human; male; female; article; mediastinum lymphadenopathy; siltuximab; distant failure free survival; local progression free survival
Journal Title: Clinical Lymphoma, Myeloma and Leukemia
Volume: 20
Issue: 10
ISSN: 2152-2650
Publisher: Elsevier Inc.  
Date Published: 2020-10-01
Start Page: 685
End Page: 689
Language: English
DOI: 10.1016/j.clml.2020.05.002
PUBMED: 32522439
PROVIDER: scopus
PMCID: PMC7541423
DOI/URL:
Notes: Article -- Export Date: 2 November 2020 -- Source: Scopus
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  1. Joachim Yahalom
    634 Yahalom
  2. Ariela Noy
    369 Noy
  3. Karen Chau
    30 Chau
  4. Thomas Hendrix Beckham
    18 Beckham