Hematologic Cancer

PV QA 2 - Poster Viewing Q&A 2

MO_44_2539 - Local Therapy in the Definitive Management of Castleman Disease

Monday, October 22
10:45 AM - 12:15 PM
Location: Innovation Hub, Exhibit Hall 3

Local Therapy in the Definitive Management of Castleman Disease
T. Beckham1, J. C. Yang2, K. Chau2, A. Noy2, and J. Yahalom2; 1Memorial Sloan Kettering Cancer Center, Department of Radiation Oncology, New York, NY, 2Memorial Sloan Kettering Cancer Center, New York, NY

Purpose/Objective(s): Castleman disease(CD) is a spectrum of rare benign lymphoproliferative disorders of unclear etiology which may present asymptomatically or with a range of symptoms related to systemic effects of inflammatory mediators produced by the involved cells. Unicentric CD presents with a single or limited number of involved sites and without systemic symptoms whereas multicentric CD presents with widespread systemic involvement and typically severe systemic symptoms. A new category, oligocentric CD, has limited nodal involvement with systemic symptoms present. Standard local therapy for patients with unicentric CD is surgical resection or radiotherapy (RT) when complete resection is not feasible. Unfortunately, the efficacy of RT is poorly characterized. Our objective was to review our institutional experience using RT for CD and to evaluate whether RT produced local control rates that were comparable to surgery.

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

Results: 43 patients (28 female and 15 male) were identified with a median age at diagnosis of 40 years (range 14-70 years). Twenty-nine (67.4%) patients received surgery alone, 3 (7.0%) had surgery followed by adjuvant RT, and 6 (14.0%) had RT alone. Thirty-eight patients (88.4%) had a single area of involvement, and 3 patients (7.0%) had limited regional involvement. Two patients (4.6%) had multicentric CD and received RT for consolidation after chemotherapy. The median follow up was 30 months. The 3-year OS, LPFS, and DFFS was 92%, 100%, and 100%, respectively. Local progression was observed in one patient from the surgery alone group, and there was no difference in LPFS between patients managed surgically and those managed with RT alone. No distant failures were observed. The median radiation dose was 3960 cGy (range 3600-5940) in a median of 22 fractions (range 18-33).

Conclusion: Definitive management of limited stage CD is feasible with RT or with surgical resection. Patients with inoperable CD or those unwilling to undergo resection have an excellent prognosis with definitive RT. A dose of 3960 cGy in 22 fractions provides excellent local control.

Author Disclosure: T. Beckham: None. J.C. Yang: Senior Committee Member; ARRO. K. Chau: None. A. Noy: None. J. Yahalom: Chairman; ILROG.

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