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Desmoid Tumor Treatment & Management

  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
 
Updated: Jun 06, 2016
 

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.[33]

In those patients who refuse surgery or are not surgical candidates, the options below 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.[34]

Pharmacologic therapy with antiestrogens and prostaglandin inhibitors may also be used. Pharmacologic agents result in objective response rates of approximately 40-50%; the duration of response is variable.[35]

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 Gardner syndrome may respond to systemic doxorubicin, and ifosfamide can be useful for patients with complications from the tumor.[36] Polychemotherapy has been used[37] and can be combined with targeted therapy with imatinib.[38]

Expanded knowledge of familial adenomatosis polyposis–desmoid tumor molecular underpinnings may aid in the development of novel therapeutic strategies.[39]

Magnetic resonance‒guided high-intensity focused ultrasound may prove a safe and effective option for selected desmoid tumors.[40, 41]

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Excision of Tumor

Aggressive, wide surgical resection is the treatment of choice.[42, 43] 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.[44] 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.[45]

Evidence suggests that pregnancy does not adversely affect surgical outcomes.[46]

Lesions involving the extremities and deep soft tissues of the trunk have a higher risk of recurrence, as do Gardner syndrome–associated lesions in other locations.[47]

For tumors that are asymptomatic or nonprogressive, some prefer a wait-and-see approach.[42]

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Long-Term Monitoring

After surgery, MRI may be useful for monitoring desmoid tumor recurrence.

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Contributor Information and Disclosures
Author

Robert A Schwartz, MD, MPH Professor and Head of Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, Rutgers New Jersey Medical School; Visiting Professor, Rutgers University School of Public Affairs and Administration

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

Disclosure: Nothing to disclose.

Coauthor(s)

Peter C Lambert, MS St George's University School of Medicine, Grenada

Disclosure: Nothing to disclose.

Specialty Editor Board

David F Butler, MD Section Chief of Dermatology, Central Texas Veterans Healthcare System; Professor of Dermatology, Texas A&M University College of Medicine; Founding Chair, Department of Dermatology, Scott and White Clinic

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

Disclosure: Nothing to disclose.

Jeffrey Meffert, MD Associate 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, Texas Dermatological Society

Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD Professor and Chairman, Department of Dermatology and Dermatologic Surgery, Medical University of South Carolina College of Medicine

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

Disclosure: Nothing to disclose.

Additional Contributors

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: Canadian Medical Association, Ontario Medical Association, Royal College of Physicians and Surgeons of Canada, Canadian Dermatology Association, American Academy of Dermatology, American Society for Clinical Pharmacology and Therapeutics

Disclosure: Nothing to disclose.

Acknowledgements

Matthew J Trovato, MD Fellow, Division of Plastic Surgery, Rutgers New Jersey Medical School

Disclosure: Nothing to disclose.

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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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