Desmoid Tumor Treatment & Management

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD   more...
 
Updated: Mar 29, 2011
 

Treatment Options for Nonsurgical Patients

Primary surgery with negative surgical margins is the most successful primary treatment modality for desmoid tumors. Positive margins after surgery reflect a high risk for recurrence.[22]

In those patients who refuse surgery or are not surgical candidates the following options may be considered:

  • Radiation therapy may be used as a treatment for recurrent disease or as primary therapy to avoid mutilating surgical resection. It may be used postoperatively, preoperatively, or as the sole treatment.[23]
  • Pharmacologic therapy with antiestrogens and prostaglandin inhibitors may also be used.
  • In cases of recurrent extra-abdominal desmoid tumors in which surgery is contraindicated or in cases of recurrence, a chemotherapeutic regimen of doxorubicin, dacarbazine, and carboplatin may be effective. Intra-abdominal desmoid tumors as a part of Gardener syndrome may respond to systemic doxorubicin, and ifosfamide can be useful for patients with complications from the tumor.[24] Polychemotherapy has been used[25] and can be combined with targeted therapy with imatinib.[26]

A selection of completed, recruiting, and active clinical trials follows:

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Excision of Tumor

Aggressive, wide surgical resection is the treatment of choice. Complete surgical excision of desmoid tumors is the most effective method of cure. This sometimes necessitates removal of most of an anterior compartment of a leg. Extensive cases may require excision plus adjuvant treatment including chemotherapy and repeat surgery.[27] In selected patients, radical resection with intraoperative margin evaluation by frozen sections followed by immediate mesh reconstruction may be a safe and effective procedure providing definitive cure yet minimizing functional limitations.[28]

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Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH  Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, University of Medicine and Dentistry of New Jersey-New Jersey Medical School

Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi

Disclosure: Nothing to disclose.

Coauthor(s)

Matthew J Trovato, MD  Fellow, Division of Plastic Surgery, University of Medicine and Dentistry of New Jersey, New Jersey Medical School

Disclosure: Nothing to disclose.

Peter C Lambert  Department of Chemistry, Wesleyan University

Disclosure: Nothing to disclose.

Specialty Editor Board

Neil Shear, MD  Professor and Chief of Dermatology, Professor of Medicine, Pediatrics and Pharmacology, University of Toronto Faculty of Medicine; Head of Dermatology, Sunnybrook Women's College Health Sciences Center and Women's College Hospital, Canada

Neil Shear, MD is a member of the following medical societies: American Academy of Dermatology, American Society for Clinical Pharmacology and Therapeutics, Canadian Dermatology Association, Canadian Medical Association, Ontario Medical Association, and Royal College of Physicians and Surgeons of Canada

Disclosure: Nothing to disclose.

David F Butler, MD  Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic, Northside Clinic

David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa

Disclosure: Nothing to disclose.

Jeffrey Meffert, MD  Assistant Clinical Professor of Dermatology, University of Texas School of Medicine at San Antonio

Jeffrey Meffert, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, Association of Military Dermatologists, and Texas Dermatological Society

Disclosure: Nothing to disclose.

Catherine M Quirk, MD  Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania

Catherine M Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD  Director, Ackerman Academy of Dermatopathology, New York

Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology

Disclosure: Nothing to disclose.

Additional Contributors

The authors and editors of eMedicine gratefully acknowledge the contributions of previous Chief Editor, William D. James, MD, to the development and writing of this article.

References
  1. Brueckl WM, Ballhausen WG, Fortsch T, et al. Genetic testing for germline mutations of the APC gene in patients with apparently sporadic desmoid tumors but a family history of colorectal carcinoma. Dis Colon Rectum. Jun 2005;48(6):1275-81. [Medline].

  2. Sturt NJ, Gallagher MC, Bassett P, et al. Evidence for genetic predisposition to desmoid tumours in familial adenomatous polyposis independent of the germline APC mutation. Gut. Dec 2004;53(12):1832-6. [Medline].

  3. Caspari R, Olschwang S, Friedl W, et al. Familial adenomatous polyposis: desmoid tumours and lack of ophthalmic lesions (CHRPE) associated with APC mutations beyond codon 1444. Hum Mol Genet. Mar 1995;4(3):337-40. [Medline].

  4. Shields CJ, Winter DC, Kirwan WO, Redmond HP. Desmoid tumours. Eur J Surg Oncol. Dec 2001;27(8):701-6. [Medline].

  5. Klemmer S, Pascoe L, DeCosse J. Occurrence of desmoids in patients with familial adenomatous polyposis of the colon. Am J Med Genet. Oct 1987;28(2):385-92. [Medline].

  6. Lee JC, Thomas JM, Phillips S, Fisher C, Moskovic E. Aggressive fibromatosis: MRI features with pathologic correlation. AJR Am J Roentgenol. Jan 2006;186(1):247-54. [Medline].

  7. Raynham WH, Louw JH. Desmoid tumours in familial polyposis of the colon. S Afr J Surg. Jul-Sep 1971;9(3):133-40. [Medline].

  8. Gaches C, Burke J. Desmoid tumour (fibroma of the abdominal wall) occurring in siblings. Br J Surg. Jul 1971;58(7):495-8. [Medline].

  9. Lopez R, Kemalyan N, Moseley HS, Dennis D, Vetto RM. Problems in diagnosis and management of desmoid tumors. Am J Surg. May 1990;159(5):450-3. [Medline].

  10. Agrawal PS, Jagtap SM, Mitra SR. Extra-abdominal desmoid tumour of the leg. Singapore Med J. Jan 2008;49(1):e6-7. [Medline].

  11. Macgill AA, Milione VR, Sullivan LG. Extra-abdominal desmoid fibromatosis in the foot: a case study. J Am Podiatr Med Assoc. Jan-Feb 2011;101(1):70-4. [Medline].

  12. Shi B, Zhu Y, Xu Z, Liu Y, Zheng B, Qi T. Aggressive fibromatosis in the urological system. Report of two adult patients and review of the literature. Urol Int. 2007;78(1):93-6. [Medline].

  13. Neri HA, Villagra EJ, Alvarez AC, et al. Ethmoidal desmoid tumor in a pediatric patient. Otolaryngol Head Neck Surg. Jan 2007;136(1):137-8. [Medline].

  14. Muller M, Dessogne P, Baron M, Picquenot JM, Riopel C, Diologent B, et al. [Desmoid tumor of the breast in a 9 years old little girl.]. Ann Pathol. Feb 2011;31(1):41-45. [Medline].

  15. Pajares B, Galera I, Ribelles N, Polo M. Insidious mastalgia hiding a desmoid tumour of the breast. Clin Transl Oncol. Jan 2010;12(1):63-5. [Medline].

  16. Gurbuz AK, Giardiello FM, Petersen GM, et al. Desmoid tumours in familial adenomatous polyposis. Gut. Mar 1994;35(3):377-81. [Medline].

  17. Lotfi AM, Dozois RR, Gordon H, et al. Mesenteric fibromatosis complicating familial adenomatous polyposis: predisposing factors and results of treatment. Int J Colorectal Dis. 1989;4(1):30-6. [Medline].

  18. Wilcken N, Tattersall MH. Endocrine therapy for desmoid tumors. Cancer. Sep 15 1991;68(6):1384-8. [Medline].

  19. Waddell WR. Treatment of intra-abdominal and abdominal wall desmoid tumors with drugs that affect the metabolism of cyclic 3',5'-adenosine monophosphate. Ann Surg. Mar 1975;181(3):299-302. [Medline].

  20. Dhingra K. Antiestrogens--tamoxifen, SERMs and beyond. Invest New Drugs. 1999;17(3):285-311. [Medline].

  21. Jakowski JD, Mayerson J, Wakely PE Jr. Fine-needle aspiration biopsy of the distal extremities: a study of 141 cases. Am J Clin Pathol. Feb 2010;133(2):224-31. [Medline].

  22. Buitendijk S, van de Ven CP, Dumans TG, et al. Pediatric aggressive fibromatosis: a retrospective analysis of 13 patients and review of literature. Cancer. Sep 1 2005;104(5):1090-9. [Medline].

  23. El-Haddad M, El-Sebaie M, Ahmad R, et al. Treatment of aggressive fibromatosis: the experience of a single institution. Clin Oncol (R Coll Radiol). Dec 2009;21(10):775-80. [Medline].

  24. Bhama PK, Chugh R, Baker LH, Doherty GM. Gardner's syndrome in a 40-year-old woman: successful treatment of locally aggressive desmoid tumors with cytotoxic chemotherapy. World J Surg Oncol. Dec 17 2006;4:96. [Medline].

  25. Constantinidou A, Jones RL, Scurr M, Al-Muderis O, Judson I. Advanced aggressive fibromatosis: Effective palliation with chemotherapy. Acta Oncol. Aug 30 2010;[Medline].

  26. Knechtel G, Stoeger H, Szkandera J, Dorr K, Beham A, Samonigg H. Desmoid tumor treated with polychemotherapy followed by imatinib: a case report and review of the literature. Case Rep Oncol. Aug 6 2010;3(2):287-93. [Medline].

  27. Ramirez RN, Otsuka NY, Apel DM, Bowen RE. Desmoid tumor in the pediatric population: a report of two cases. J Pediatr Orthop B. May 2009;18(3):141-4. [Medline].

  28. Bertani E, Chiappa A, Testori A, et al. Desmoid tumors of the anterior abdominal wall: results from a monocentric surgical experience and review of the literature. Ann Surg Oncol. Jun 2009;16(6):1642-9. [Medline].

  29. Mendenhall WM, Zlotecki RA, Morris CG, Hochwald SN, Scarborough MT. Aggressive fibromatosis. Am J Clin Oncol. Apr 2005;28(2):211-5. [Medline].

  30. Huang PW, Tzen CY. Prognostic factors in desmoid-type fibromatosis: a clinicopathological and immunohistochemical analysis of 46 cases. Pathology. Feb 2010;42(2):147-50. [Medline].

  31. Meazza C, Bisogno G, Gronchi A, Fiore M, Cecchetto G, Alaggio R, et al. Aggressive fibromatosis in children and adolescents: the Italian experience. Cancer. Jan 1 2010;116(1):233-40. [Medline].

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Bland fibrocytic cells of a desmoid tumor growing in a haphazard-to-storiform manner and producing collagen (hematoxylin-eosin, original magnification X100).
Desmoid tumor spindle cells invading skeletal muscle (hematoxylin-eosin, original magnification X100).
Dermoid tumor spindle cells surrounding and destroying skeletal muscle cells (hematoxylin-eosin, original magnification X100)
 
 
 
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