Desmoid Tumor Treatment & Management

Updated: Mar 23, 2021
  • 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. [36]  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. [37]  Since desmoid tumors do not metastasize, many approaches are nonoperative in nature. [38] Since desmoid tumors may stabilize and regress, active observation is an option.


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. [39]

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. [40]

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. [41] Polychemotherapy has been used [42] and can be combined with targeted therapy with imatinib. [43]

Expanded knowledge of familial adenomatosis polyposis–desmoid tumor molecular underpinnings may aid in the development of novel therapeutic strategies. [44]

Magnetic resonance‒guided high-intensity focused ultrasound may prove a safe and effective option for selected desmoid tumors. [45, 46]

Systemic therapy is appropriate if a primary complete resection is not feasible or if there is relapse or progression after resection. [47] There are a number of novel drug therapy candidates for desmoid tumors. [48] Pazopanib, a potent tyrosine kinase inhibitor, represents a promising new therapy for desmoid tumors in adolescent and young adult patients. [49]

Split-course radiotherapy in patients with desmoid tumors was well tolerated with good outcomes. [50]


Excision of Tumor

Aggressive, wide surgical resection is the treatment of choice. [51, 52] 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. [53] 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. [54]  However, there has been a recent tendency to more conservative management. [10] Active surveillance MRI has become a popular option. [55]

Evidence suggests that pregnancy does not adversely affect surgical outcomes. [56]

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. [57]

For tumors that are asymptomatic or nonprogressive, some prefer a wait-and-see approach. [51]


Long-Term Monitoring

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