Pediatric desmoid tumor: Retrospective analysis of 63 cases Journal Article


Authors: Faulkner, L. B.; Hajdu, S. I.; Kher, U.; La Quaglia, M.; Exelby, P. R.; Heller, G.; Wollner, N.
Article Title: Pediatric desmoid tumor: Retrospective analysis of 63 cases
Abstract: Purpose: This study was conducted to evaluate clinica]prognostic factors predictive of the probability of recurrence of desmoid tumor (DT). Patients and Methods: Sixty-three patients with histologically confirmed diagnosis of DT were retrospectively studied. Median age at diagnosis was 13 years. Patient distribution by site was as follows: 61% extremities, 18% head and neck, 13% trunk (including 5% intraabdominal), and 8% multicentric lesions. All patients had partial or complete resections; 20 patients also received radiotherapy and/or chemotherapy. Results: At a median follow-up time of 6 years since first treatment, the overall actuarial probability of having one or more recurrences was 75%. Age, sex, site, size, or number of previous recurrences had no significant impact on the likelihood of recurrence. The only factor associated with an increased proportion of recurrence-free patients was a negative margin of resection (70% v 15% with positive margins; P = .006). Of the four patients with more than 3 years follow-up since chemotherapy, two recurred, and of the 11 patients with the same follow-up after radiotherapy, four recurred, including two of five patients who received a dose of 50 Gy or more. No deaths directly related to tumor invasion were observed. Conclusion: A surgical approach aiming at clear margins is presently the best treatment option. When this cannot be accomplished without severe disfigurement or function impairment, partial resection is an acceptable alternative, but one associated with a high risk of regrowth. Whether adjuvant strategies should be used in this situation remains to be addressed.
Keywords: adolescent; adult; cancer chemotherapy; child; preschool child; school child; cancer surgery; major clinical study; cancer recurrence; cancer radiotherapy; cytogenetics; infant; tamoxifen; malignant transformation; desmoid tumor; gardner syndrome; prognosis; human; male; female; priority journal; article
Journal Title: Journal of Clinical Oncology
Volume: 13
Issue: 11
ISSN: 0732-183X
Publisher: American Society of Clinical Oncology  
Date Published: 1995-11-01
Start Page: 2813
End Page: 2818
Language: English
DOI: 10.1200/jco.1995.13.11.2813
PUBMED: 7595743
PROVIDER: scopus
DOI/URL:
Notes: Article -- Export Date: 28 August 2018 -- Source: Scopus
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MSK Authors
  1. Glenn Heller
    291 Heller
  2. Philip Exelby
    3 Exelby