Desmoid tumor (patient information)
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Desmoid tumor Microchapters |
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Associate Editor(s)-in-Chief: Sara Mohsin, M.D.[2]Faizan Sheraz, M.D. [3]
Overview
Patient information
Desmoid tumors (also called aggressive fibromatosis) are benign, slowly growing fibroblastic neoplasms with no metastatic potential but a propensity for local recurrence, even after complete surgical resection. Despite being histologically benign, they are locally infiltrative and can cause death through destruction of adjacent vital structures and organs. (See 'Introduction' above.)
●The risk of desmoids is increased in patients with familial adenomatous polyposis (FAP). Given the association with FAP, at some institutions, colonoscopy is recommended for all patients with desmoid tumors, particularly intraabdominal. (See 'FAP and Gardner syndrome' above.)
●Treatment of desmoids can be a clinical challenge, particularly in patients with FAP who tend to develop intraabdominal desmoids at sites of prior surgery. Because of their locally aggressive behavior and tendency to relapse with more aggressive disease, multimodality treatment is often required for these benign lesions and is best delivered within the context of a multidisciplinary team specializing in sarcoma treatment.
●Extraabdominal and abdominal desmoids tend to occur in women during or following pregnancy. If a woman had a desmoid arise during a prior pregnancy and the desmoid was resected, the recurrence risk during future pregnancies is low. If woman had an existing desmoid (pregnancy-associated or predating any pregnancy) that was managed with watchful waiting, that desmoid can grow during a subsequent pregnancy, but not always. The risk to the pregnancy is very low, and the course of the pregnancy may be relatively normal. (See 'Pregnancy' above.)
Treatment — The natural history of desmoid tumors is prolonged, variable, and unpredictable, and it is not clear that any intervention improves survival. (See 'Natural history' above.)
Extraabdominal and abdominal wall tumors
●Observation is an appropriate option in asymptomatic patients who may be reliably followed. If desmoids remain unchanged or shrink, observation may be continued. If the tumor increases in size or becomes symptomatic, if there is imminent risk to adjacent structures, or if the desmoid creates cosmetic concerns, treatment should be pursued. (See 'Initial observation' above.)
●We suggest complete surgical excision as the treatment of choice for a potentially resectable extraabdominal (extremity, trunk, breast) or abdominal wall desmoids in a patient who is medically able to tolerate surgery and if resection can be accomplished without major functional or cosmetic deficit (Grade 2B). Controversy remains as a number of patients will fare well without surgical intervention, so a more conservative approach appears rational for relatively static lesions. (See 'Surgery' above.)
●Primary radiation therapy (RT) is an appropriate option for patients who need treatment but are not good surgical candidates, those who decline surgery, and those for whom surgical morbidity would be excessive. An alternative approach is initiation of systemic treatment in these patients, especially if they are young and there are concerns about the potential for late toxicity from RT. (See 'Radiation therapy' above.)
●We recommend not pursuing adjuvant RT in patients with microscopically negative surgical margins, regardless of tumor size (Grade 1C). We suggest not pursuing adjunctive RT following resection of a primary desmoid tumor with microscopically positive margins (Grade 2C). Since fewer than 50 percent of these patients will develop a recurrence, patients who remain progression-free will be spared the long-term sequelae of RT. (See 'Postoperative RT' above.)
●For gross residual disease of a primary desmoid, we suggest observation or RT rather than more radical resection (Grade 2B). Due to the very high local control rates with RT, additional surgery is warranted only if there is strong confidence in obtaining clear margins, and functional loss is minimal and acceptable. Systemic therapy is also effective in controlling extremity desmoids in a number of patients, precluding the need for further surgery.
●For a recurrent desmoid tumor, our preference is observation or surgical resection. However, systemic therapy or RT alone are reasonable alternative treatments in selected patients who are thought to have a higher morbidity from repeat operation and an increased probability of positive margins. For patients who undergo surgery for a recurrent desmoid, we suggest observation or postoperative RT if the margins are positive (Grade 2C). (See 'Management of recurrent disease' above.)
Intraabdominal desmoid, Gardner syndrome
●For patients who have large intraabdominal desmoid tumors, particularly in the setting of Gardner syndrome, surgery is still a standard approach for resectable tumors. However, the infiltrative nature of the desmoid in this situation often precludes surgery, and the surgical margins are often positive. Medical therapy in lieu of surgery is a viable option for patients with more difficult tumors such as those involving the mesentery, major vessels, or other critical structures. (See 'Intraabdominal desmoids' above and "Desmoid tumors: Systemic therapy".)
●Surgery with or without RT is an appropriate option if there is no response to medical therapy. In this situation, consideration should be given to use of intraoperative electron beam therapy as a component of the treatment [138].
●Management of recurrence of an intraabdominal tumor in patients with Gardner syndrome is challenging because recurrences tend to become more frequent and aggressive with each surgical intervention. Most of these patients are managed with systemic therapy rather than additional local measures. (See 'Management of recurrent disease' above and "Desmoid tumors: Systemic therapy".)
Posttreatment surveillance
●There is no standard protocol for follow-up of patients with desmoid tumors. However, we typically follow patients by clinical examination and radiographic studies (where appropriate) every six months for the first three years, every 12 months to year 6, and then biannually. (See 'Posttreatment surveillance' above.)