Desmoid Tumor Treatment & Management

Updated: Mar 26, 2020
  • Author: Robert A Schwartz, MD, MPH; Chief Editor: Dirk M Elston, MD  more...
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Approach Considerations

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. [35]  Surgery plus the administration of nonsteroidal anti-inflammatory medication, hormonal therapy, and cytotoxic chemotherapy is sometimes used, the latter being the most effective pharmacological approach. [36]


Treatment Options for Nonsurgical Patients

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. [37]

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. [38]

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. [39] Polychemotherapy has been used [40] and can be combined with targeted therapy with imatinib. [41]

Expanded knowledge of familial adenomatosis polyposis–desmoid tumor molecular underpinnings may aid in the development of novel therapeutic strategies. [42]

Magnetic resonance‒guided high-intensity focused ultrasound may prove a safe and effective option for selected desmoid tumors. [43, 44]

Systemic therapy is appropriate if a primary complete resection is not feasible or if there is relapse or progression after resection. [45] There are a number of novel drug therapy candidates for desmoid tumors. [46] Pazopanib, a potent tyrosine kinase inhibitor, represents a promising new therapy for desmoid tumors in adolescent and young adult patients. [47]

Split-course radiotherapy in patients with desmoid tumors was well tolerated with good outcomes. [48]


Excision of Tumor

Aggressive, wide surgical resection is the treatment of choice. [49, 50] 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. [51] 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. [52]  However, there has been a recent tendency to more conservative management. [9] Active surveillance MRI has become a popular option. [53]

Evidence suggests that pregnancy does not adversely affect surgical outcomes. [54]

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. [55]

For tumors that are asymptomatic or nonprogressive, some prefer a wait-and-see approach. [49]


Long-Term Monitoring

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