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Title: Successful Treatment of High Risk and Recurrent Pediatric Desmoids Using Radiation as a Component of Multimodality Therapy

Journal Article · · International Journal of Radiation Oncology, Biology and Physics
 [1];  [2];  [3];  [4];  [5];  [1];  [6]; ; ;  [5]
  1. Department of Radiation Oncology, University of California, San Francisco, CA (United States)
  2. Department of Radiation Oncology, Indiana University, Indianapolis, IN (United States)
  3. Department of Biostatistics, University of California, San Francisco, CA (United States)
  4. Kaiser Permanente Medical Group, Santa Clara, CA (United States)
  5. Department of Pediatrics, University of California, San Francisco, CA (United States)
  6. Department of Orthopedic Surgery, University of California, San Francisco, CA (United States)

Purpose: To evaluate the role of radiation therapy (RT) as a component of multimodality therapy for pediatric desmoids. Methods and Materials: Twenty-one children diagnosed between 1987 and 2005 were identified. Median age at start of treatment was 13 years (range, 2-21). Primary therapy consisted of resection alone (10), resection + external beam radiation therapy (EBRT) (5), resection + chemotherapy (CT; 3), EBRT alone (1), and CT alone (2). Results: The median follow-up from start of treatment is 75.7 months (range, 16-162). Examining patients with gross total resections (GTRs) (-) margins and those who had GTRs (+) margins followed by EBRT, only 2 of 7 failed primary treatment. Conversely, 13 of 14 patients with other primary treatments failed locally. Of the 15 patients who recurred, only 1 patient had a GTR (-) margins. Seven of these patients had salvage therapy that did not include RT, and of these only 2 have no evidence of disease (NED) at last follow-up. In contrast, the remaining 8 patients received RT as a component of their final salvage therapy and 7 of these are NED at last follow-up. At last follow-up, no patient has died, although toxicities of therapy have occurred. Conclusions: Local control is difficult to achieve in pediatric patients with desmoids. In the setting in which negative surgical margins cannot be achieved, RT plays a key role in achieving NED status. Even after multiple recurrences, successful salvage is achievable, particularly when high-dose focal therapy is incorporated.

OSTI ID:
21282010
Journal Information:
International Journal of Radiation Oncology, Biology and Physics, Vol. 75, Issue 1; Other Information: DOI: 10.1016/j.ijrobp.2008.10.072; PII: S0360-3016(08)03865-0; Copyright (c) 2009 Elsevier Science B.V., Amsterdam, The Netherlands, All rights reserved; Country of input: International Atomic Energy Agency (IAEA); ISSN 0360-3016
Country of Publication:
United States
Language:
English

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