A prognostic nomogram for prediction of recurrence in desmoid fibromatosis Journal Article


Authors: Crago, A. M.; Denton, B.; Salas, S.; Dufresne, A.; Mezhir, J. J.; Hameed, M.; Gonen, M.; Singer, S.; Brennan, M. F.
Article Title: A prognostic nomogram for prediction of recurrence in desmoid fibromatosis
Abstract: OBJECTIVE:: To construct a postoperative nomogram to estimate the risk of local recurrence for patients with desmoid tumors. BACKGROUND:: The standard management of desmoid tumors is resection, but many recur locally. Other options include observation or novel chemotherapeutics, but little guidance exists on selecting treatment. METHODS:: Patients undergoing resection during 1982-2011 for primary or locally recurrent desmoids were identified from a single-institution prospective database. Cox regression analysis was used to assess risk factors and to create a recurrence nomogram, which was validated using an international, multi-institutional data set. RESULTS:: Desmoids were treated surgically in 495 patients (median follow-up of 60 months). Of 439 patients undergoing complete gross resection, 100 (23%) had recurrence. Five-year local recurrence-free survival was 69%. Eight patients died of disease, all after R2 resection. Adjuvant radiation was not associated with improved local recurrence-free survival. In multivariate analysis, factors associated with recurrence were extremity location, young age, and large tumor size, but not margin. Abdominal wall tumors had the best outcome (5-year local recurrence-free survival rate of 91%). Age, site, and size were used to construct a nomogram with concordance index of 0.703 in internal validation and 0.659 in external validation. Integration of additional variables (R1 margin, sex, depth, and primary vs recurrent presentation) did not importantly improve concordance (internal concordance index of 0.707). CONCLUSIONS:: A postoperative nomogram including only size, site, and age predicts local recurrence and can aid in counseling patients. Systemic therapies may be appropriate for young patients with large, extremity desmoids, but surgery alone is curative for most abdominal wall lesions. Copyright © 2013 Lippincott Williams & Wilkins.
Keywords: adolescent; adult; controlled study; treatment outcome; aged; aged, 80 and over; middle aged; survival analysis; shoulder; young adult; major clinical study; patient selection; cancer radiotherapy; chemotherapy; follow up; follow-up studies; tumor localization; neoplasm recurrence, local; tumor volume; risk factors; risk assessment; nomograms; soft tissue sarcoma; recurrent disease; multivariate analysis; axilla; nomogram; patient counseling; predictive value; desmoid tumor; decision support techniques; fibromatosis; fibromatosis, aggressive; rib; buttock; local recurrence free survival; abdominal wall defect
Journal Title: Annals of Surgery
Volume: 258
Issue: 2
ISSN: 0003-4932
Publisher: Lippincott Williams & Wilkins  
Date Published: 2013-08-01
Start Page: 347
End Page: 353
Language: English
DOI: 10.1097/SLA.0b013e31828c8a30
PROVIDER: scopus
PUBMED: 23532110
PMCID: PMC4096320
DOI/URL:
Notes: --- - "Export Date: 1 August 2013" - "CODEN: ANSUA" - "Source: Scopus"
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MSK Authors
  1. Brian Denton
    11 Denton
  2. Meera Hameed
    281 Hameed
  3. Murray F Brennan
    1059 Brennan
  4. Mithat Gonen
    1028 Gonen
  5. James John Mezhir
    13 Mezhir
  6. Aimee Marie Crago
    106 Crago
  7. Samuel Singer
    337 Singer