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.^[40]

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.^[41]

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.^[42]Polychemotherapy has been used^[43]and can be combined with targeted therapy with imatinib.^[44]

Expanded knowledge of familial adenomatosis polyposis–desmoid tumor molecular underpinnings may aid in the development of novel therapeutic strategies.^[45]

Magnetic resonance‒guided high-intensity focused ultrasound may prove a safe and effective option for selected desmoid tumors.^{[46, 47]}

Systemic therapy is appropriate if a primary complete resection is not feasible or if there is relapse or progression after resection.^[48]There are a number of novel drug therapy candidates for desmoid tumors.^[49]Pazopanib, a potent tyrosine kinase inhibitor, represents a promising new therapy for desmoid tumors in adolescent and young adult patients.^[50] Another option is sorafenib.^[51]

Split-course radiotherapy in patients with desmoid tumors was well tolerated with good outcomes.^[52]

Excision of Tumor

Aggressive, wide surgical resection is the treatment of choice.^{[53, 54]} 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.^[55] 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.^[56] However, there has been a recent tendency to more conservative management.^[17] Active surveillance MRI has become a popular option.^[57]

Evidence suggests that pregnancy does not adversely affect surgical outcomes.^[58]

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.^[59]

For tumors that are asymptomatic or nonprogressive, some prefer a wait-and-see approach.^[53]

Long-Term Monitoring

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

Medication

Zhao CX, Dombrowski ND, Perez-Atayde AR, Robson CD, Afshar S, Janeway KA, et al. Desmoid tumors of the head and neck in the pediatric population: Has anything changed?. Int J Pediatr Otorhinolaryngol. 2021 Jan. 140:110511. [QxMD MEDLINE Link].
Raynham WH, Louw JH. Desmoid tumours in familial polyposis of the colon. S Afr J Surg. 1971 Jul-Sep. 9(3):133-40. [QxMD MEDLINE Link].
Gaches C, Burke J. Desmoid tumour (fibroma of the abdominal wall) occurring in siblings. Br J Surg. 1971 Jul. 58(7):495-8. [QxMD MEDLINE Link].
Lopez R, Kemalyan N, Moseley HS, Dennis D, Vetto RM. Problems in diagnosis and management of desmoid tumors. Am J Surg. 1990 May. 159(5):450-3. [QxMD MEDLINE Link].
Jeong WS, Oh TS, Sim HB, Eom JS. Desmoid tumor following augmentation mammoplasty with silicone implants. Arch Plast Surg. 2013 Jul. 40(4):470-2. [QxMD MEDLINE Link]. [Full Text].
Buitendijk S, van de Ven CP, Dumans TG, et al. Pediatric aggressive fibromatosis: a retrospective analysis of 13 patients and review of literature. Cancer. 2005 Sep 1. 104(5):1090-9. [QxMD MEDLINE Link].
Inoue Y, Ishida H, Ueno H, Kobayashi H, Yamaguchi T, Konishi T, et al. The treatment of desmoid tumors associated with familial adenomatous polyposis: the results of a Japanese multicenter observational study. Surg Today. 2017 Mar 1. [QxMD MEDLINE Link].
Fiore M, Crago A, Gladdy R, Kasper B. The Landmark Series: Desmoid. Ann Surg Oncol. 2021 Mar. 28 (3):1682-1689. [QxMD MEDLINE Link].
Pawluś A, Szymańska K, Kaczorowski K, Sokołowska-Dąbek D, Olchowy C, Markiewicz BD, et al. A Rare Case of Aggressive Fibromatosis Infiltrating Dorsal Muscles in a 6-Year-Old Patient - CT, MRI and Elastography Evaluation. Pol J Radiol. 2015. 80:164-7. [QxMD MEDLINE Link].
Wu C, Amini-Nik S, Nadesan P, Stanford WL, Alman BA. Aggressive fibromatosis (desmoid tumor) is derived from mesenchymal progenitor cells. Cancer Res. 2010 Oct 1. 70(19):7690-8. [QxMD MEDLINE Link].
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. 2005 Jun. 48(6):1275-81. [QxMD MEDLINE Link].
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. 2004 Dec. 53(12):1832-6. [QxMD MEDLINE Link].
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. 1995 Mar. 4(3):337-40. [QxMD MEDLINE Link].
Cavallini A, Rotelli MT, Lippolis C, Piscitelli D, Digennaro R, Covelli C, et al. Human microRNA expression in sporadic and FAP-associated desmoid tumors and correlation with beta-catenin mutations. Oncotarget. 2017 Mar 19. [QxMD MEDLINE Link].
Wang WL, Nero C, Pappo A, Dina Lev DL, Lazar AJ, Lopez-Terrada DH. CTNNB1 Genotyping and APC Screening in Pediatric Desmoid Tumors: A Proposed Alogrithm. Pediatr Dev Pathol. 2012 Feb 28. [QxMD MEDLINE Link].
Mullen JT, Delaney TF, Rosenberg AE, Le L, Iafrate AJ, Kobayashi W, et al. ß-Catenin Mutation Status and Outcomes in Sporadic Desmoid Tumors. Oncologist. 2013 Aug 19. [QxMD MEDLINE Link].
Desmoid Tumor Working Group. The management of desmoid tumours: A joint global consensus-based guideline approach for adult and paediatric patients. Eur J Cancer. 2020 Mar. 127:96-107. [QxMD MEDLINE Link].
Gurbuz AK, Giardiello FM, Petersen GM, et al. Desmoid tumours in familial adenomatous polyposis. Gut. 1994 Mar. 35(3):377-81. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Wilcken N, Tattersall MH. Endocrine therapy for desmoid tumors. Cancer. 1991 Sep 15. 68(6):1384-8. [QxMD MEDLINE Link].
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. 1975 Mar. 181(3):299-302. [QxMD MEDLINE Link].
Dhingra K. Antiestrogens--tamoxifen, SERMs and beyond. Invest New Drugs. 1999. 17(3):285-311. [QxMD MEDLINE Link].
Shields CJ, Winter DC, Kirwan WO, Redmond HP. Desmoid tumours. Eur J Surg Oncol. 2001 Dec. 27(8):701-6. [QxMD MEDLINE Link].
Klemmer S, Pascoe L, DeCosse J. Occurrence of desmoids in patients with familial adenomatous polyposis of the colon. Am J Med Genet. 1987 Oct. 28(2):385-92. [QxMD MEDLINE Link].
Lee JC, Thomas JM, Phillips S, Fisher C, Moskovic E. Aggressive fibromatosis: MRI features with pathologic correlation. AJR Am J Roentgenol. 2006 Jan. 186(1):247-54. [QxMD MEDLINE Link].
Huang PW, Tzen CY. Prognostic factors in desmoid-type fibromatosis: a clinicopathological and immunohistochemical analysis of 46 cases. Pathology. 2010 Feb. 42(2):147-50. [QxMD MEDLINE Link].
Meazza C, Bisogno G, Gronchi A, Fiore M, Cecchetto G, Alaggio R, et al. Aggressive fibromatosis in children and adolescents: the Italian experience. Cancer. 2010 Jan 1. 116(1):233-40. [QxMD MEDLINE Link].
Dufresne A, Paturel M, Alberti L, Philippon H, Duc A, Decouvelaere AV, et al. Prediction of desmoid tumor progression using miRNA expression profiling. Cancer Sci. 2015 May. 106 (5):650-5. [QxMD MEDLINE Link].
Quintini C, Ward G, Shatnawei A, Xhaja X, Hashimoto K, Steiger E, et al. Mortality of intra-abdominal desmoid tumors in patients with familial adenomatous polyposis: a single center review of 154 patients. Ann Surg. 2012 Mar. 255(3):511-6. [QxMD MEDLINE Link].
Colombo C, Miceli R, Lazar AJ, Perrone F, Pollock RE, Le Cesne A, et al. CTNNB1 45F mutation is a molecular prognosticator of increased postoperative primary desmoid tumor recurrence: An independent, multicenter validation study. Cancer. 2013 Jul 31. [QxMD MEDLINE Link].
Agrawal PS, Jagtap SM, Mitra SR. Extra-abdominal desmoid tumour of the leg. Singapore Med J. 2008 Jan. 49(1):e6-7. [QxMD MEDLINE Link].
Macgill AA, Milione VR, Sullivan LG. Extra-abdominal desmoid fibromatosis in the foot: a case study. J Am Podiatr Med Assoc. 2011 Jan-Feb. 101(1):70-4. [QxMD MEDLINE Link].
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. [QxMD MEDLINE Link].
Kohno J, Sumiyoshi T, Tsutsumi N, Maeno A, Okubo K, Mitsumori K, et al. [Scrotal desmoid tumor in a patient with familial adenomatous polyposis]. Hinyokika Kiyo. 2015 Jan. 61 (1):27-31. [QxMD MEDLINE Link].
Neri HA, Villagra EJ, Alvarez AC, et al. Ethmoidal desmoid tumor in a pediatric patient. Otolaryngol Head Neck Surg. 2007 Jan. 136(1):137-8. [QxMD MEDLINE Link].
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. 2011 Feb. 31(1):41-45. [QxMD MEDLINE Link].
Pajares B, Galera I, Ribelles N, Polo M. Insidious mastalgia hiding a desmoid tumour of the breast. Clin Transl Oncol. 2010 Jan. 12(1):63-5. [QxMD MEDLINE Link].
Mekhail FG, Montgomery JR, Spicer PJ. Imaging findings of a biopsy-proven desmoid tumor of the axilla in a young female. Radiol Case Rep. 2022 Apr. 17 (4):1050-1053. [QxMD MEDLINE Link]. [Full Text].
Jakowski JD, Mayerson J, Wakely PE Jr. Fine-needle aspiration biopsy of the distal extremities: a study of 141 cases. Am J Clin Pathol. 2010 Feb. 133(2):224-31. [QxMD MEDLINE Link].
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). 2009 Dec. 21(10):775-80. [QxMD MEDLINE Link].
Mendenhall WM, Zlotecki RA, Morris CG, Hochwald SN, Scarborough MT. Aggressive fibromatosis. Am J Clin Oncol. 2005 Apr. 28(2):211-5. [QxMD MEDLINE Link].
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. 2006 Dec 17. 4:96. [QxMD MEDLINE Link].
Constantinidou A, Jones RL, Scurr M, Al-Muderis O, Judson I. Advanced aggressive fibromatosis: Effective palliation with chemotherapy. Acta Oncol. 2010 Aug 30. [QxMD MEDLINE Link].
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. 2010 Aug 6. 3(2):287-93. [QxMD MEDLINE Link].
Colombo C, Foo WC, Whiting D, Young ED, Lusby K, Pollock RE, et al. FAP-related desmoid tumors: a series of 44 patients evaluated in a cancer referral center. Histol Histopathol. 2012 May. 27(5):641-9. [QxMD MEDLINE Link].
Ghanouni P, Dobrotwir A, Bazzocchi A, Bucknor M, Bitton R, Rosenberg J, et al. Magnetic resonance-guided focused ultrasound treatment of extra-abdominal desmoid tumors: a retrospective multicenter study. Eur Radiol. 2016 May 5. [QxMD MEDLINE Link].
Avedian RS, Bitton R, Gold G, Butts-Pauly K, Ghanouni P. Is MR-guided High-intensity Focused Ultrasound a Feasible Treatment Modality for Desmoid Tumors?. Clin Orthop Relat Res. 2016 Mar. 474 (3):697-704. [QxMD MEDLINE Link].
Sparber-Sauer M, Seitz G, von Kalle T, Vokuhl C, Leuschner I, Scheer M, et al. Systemic therapy of aggressive fibromatosis in children and adolescents: Report of the Cooperative Weichteilsarkom Studiengruppe (CWS). Pediatr Blood Cancer. 2018 Jan 5. [QxMD MEDLINE Link].
Mercier KA, Al-Jazrawe M, Poon R, Acuff Z, Alman B. A Metabolomics Pilot Study on Desmoid Tumors and Novel Drug Candidates. Sci Rep. 2018 Jan 12. 8 (1):584. [QxMD MEDLINE Link].
Agresta L, Kim H, Turpin BK, Nagarajan R, Plemmons A, Szabo S, et al. Pazopanib therapy for desmoid tumors in adolescent and young adult patients. Pediatr Blood Cancer. 2018 Jan 31. [QxMD MEDLINE Link].
Johns MS, Merritt WT 3rd, Rhodes L, Ford CN, Thompson M, Lee WM, et al. A cost analysis of sorafenib for desmoid tumors. J Oncol Pharm Pract. 2023 Apr. 29 (3):663-668. [QxMD MEDLINE Link].
Luo J, Jin K, Qian S, Ma X, Pan Z, Yao W, et al. Single institution experience of split course radiotherapy in patients with desmoid tumors. Onco Targets Ther. 2019. 12:1741-1748. [QxMD MEDLINE Link].
Oudot C, Defachelles AS, Minard-Colin V, Olschwang S, Fourcade L, Helfre S, et al. [Desmoid tumors in children: current strategy]. Bull Cancer. 2013 May. 100(5):518-28. [QxMD MEDLINE Link].
Soto-Miranda MA, Sandoval JA, Rao B, Neel M, Krasin M, Spunt S, et al. Surgical Treatment of Pediatric Desmoid Tumors. A 12-Year, Single-Center Experience. Ann Surg Oncol. 2013 Jul 10. [QxMD MEDLINE Link].
Ramirez RN, Otsuka NY, Apel DM, Bowen RE. Desmoid tumor in the pediatric population: a report of two cases. J Pediatr Orthop B. 2009 May. 18(3):141-4. [QxMD MEDLINE Link].
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. 2009 Jun. 16(6):1642-9. [QxMD MEDLINE Link].
Ben Haj Amor M, Ploton L, Ceugnart L, Taïeb S. [Magnetic resonance imaging of desmoid-type fibromatosis: Current evaluation criteria]. Bull Cancer. 2020 Mar. 107 (3):359-363. [QxMD MEDLINE Link].
Cates JM. Pregnancy does not increase the local recurrence rate after surgical resection of desmoid-type fibromatosis. Int J Clin Oncol. 2014 Aug 16. [QxMD MEDLINE Link].
Cates JM, Stricker TP, Sturgeon D, Coffin CM. Desmoid-type fibromatosis-associated Gardner fibromas: prevalence and impact on local recurrence. Cancer Lett. 2014 Oct 28. 353(2):176-81. [QxMD MEDLINE Link].

Media Gallery

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)

of 3

Desmoid Tumor Treatment & Management

Treatment Options for Nonsurgical Patients

Excision of Tumor

Long-Term Monitoring

References

Tables

Contributor Information and Disclosures

What would you like to print?