The enigma of desmoid tumors Conference Paper


Authors: Lewis, J. J.; Boland, P. J.; Leung, D. H. Y.; Woodruff, J. M.; Brennan, M. F.
Title: The enigma of desmoid tumors
Conference Title: 110th Annual Meeting of the Southern Surgical Association
Abstract: Objective: To analyze patients with recurrent extremity desmoids, in whom the surgical therapeutic option was either major amputation or observation. Summary Background Data: The biology and natural history of desmoid tumors are an enigma. These tumors invade surrounding structures and recur locally but do not metastasize. The morbidity of treating these tumors in the context of their relatively benign biology is uncertain. Methods: Between July 1982 and June 1998, the authors treated and prospectively followed 206 patients with extremity desmoid tumors. All patients underwent standardized surgical resection, the surgical goal always being complete resection with negative margins. When tumors recurred, they were evaluated for reresection. Amputation was considered when resection was not possible because of neurovascular or major bone involvement, or in the presence of a functionless, painful extremity. Results: During this period, 22 patients had disease that was not resectable without amputation. This was out of a total of 115 patients with primary disease and 91 patients with recurrent disease. All recurrences were local; in no patient did metastasis develop. In this group of 22 patients with unresectable disease, 7 underwent amputation and 15 did not. These 15 patients were followed, alive with disease, having no surgical resection. Four patients received systemic treatment with tamoxifen and nonsteroidal antiinflammatories, three received systemic cytotoxic chemotherapy, and two received both tamoxifen and chemotherapy. Six patients received no systemic treatment. The range of follow-up was 25 to 92 months. In all patients, there was no or insignificant tumor progression; in three patients who underwent observation alone, there was some regression of tumor. During follow-up, no patient has required subsequent amputation, and no patient has died from disease. Conclusions: In desmoid tumors, aggressive attempts at achieving negative resection margins may result in unnecessary morbidity. Function- and structure-preserving procedures should be the primary goal. In select patients, whose only option is amputation, it may be prudent to observe them with their limb and tumor intact.
Keywords: adolescent; adult; human tissue; middle aged; retrospective studies; major clinical study; histopathology; conference paper; recurrence risk; follow up; follow-up studies; prospective studies; neoplasm recurrence, local; morbidity; tumor biopsy; algorithms; nonsteroid antiinflammatory agent; amputation; tamoxifen; recurrent disease; soft tissue neoplasms; leg; desmoid tumor; fibromatosis, aggressive; arm; humans; human; male; female; priority journal
Journal Title Annals of Surgery
Volume: 229
Issue: 6
Conference Dates: 1998 Dec 6-9
Conference Location: West Palm Beach, FL
ISBN: 0003-4932
Publisher: Lippincott Williams & Wilkins  
Date Published: 1999-06-01
Start Page: 866
End Page: 873
Language: English
DOI: 10.1097/00000658-199906000-00014
PUBMED: 10363901
PROVIDER: scopus
PMCID: PMC1420834
DOI/URL:
Notes: Conference Paper -- Export Date: 16 August 2016 -- Source: Scopus
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MSK Authors
  1. Murray F Brennan
    1059 Brennan
  2. Patrick J Boland
    160 Boland
  3. Jonathan J Lewis
    109 Lewis
  4. Denis Heng Yan Leung
    114 Leung
  5. James M Woodruff
    162 Woodruff