Multimodality treatment of mesenteric desmoid tumours
Section snippets
Background and aims
Desmoid tumours, also known as desmoid fibromatoses, are uncommon soft tissue neoplasms. Although they do not metastasise, desmoids often exhibit an infiltrative pattern of spread in an abundant collagen matrix, giving them a dense, fibrotic character.1 As a result, these tumours can produce local tissue destruction leading to significant morbidity and functional loss. Desmoid tumours are a relatively frequent complication of the hereditary cancer predisposition syndrome, Familial Adenomatous
Patient selection and pre-treatment assessment
In a study, approved by the Institutional Review Boards of Brigham and Women’s Hospital (BWH) and Dana-Farber Cancer Institute (DFCI), we reviewed a consecutive series of patients with desmoid tumours of the intestinal mesentery that were referred to the Sarcoma Service at BWH/DFCI between January 2001 and October 2006. These patients were evaluated and treated by a multidisciplinary team including three medical oncologists (JAM, SG and GDD) and two surgical oncologists (MMB and CPR). A total
Description of initial treatment
Summaries of the treatment administered are provided in Fig. 2 and Table 1. Surgery was the first treatment modality for 94% of those with localised tumours, and for 45% of those with infiltrative tumours. Tumour resection with negative margins was achieved in only 7 of 34 (20%) cases for which surgery was used as the first treatment modality, and each of these patients had localised tumour type. In the remaining 27 patients with bulky or infiltrative disease, R1 resections were achieved. For
Discussion
The optimal management strategy for patients with mesenteric desmoid tumours balances the individual patient’s disease risk with the risks and potential benefits of intervention. We found that addition of a relatively well-tolerated systemic chemotherapy, liposomal doxorubicin or vinorelbine, in selected patients with aggressive desmoids can achieve objective responses and disease stabilisation in cases where surgical risks are high. Our overall experience further indicates that some patients
Conflict of interest statement
Dr. Demetri serves as a member of the scientific advisory board of the Desmoid Tumour Research Foundation, as well as having received consulting fees and research support from Novartis, Pfizer, Ariad and Johnson and Johnson.
Dr. George has received consulting fees from Pfizer.
Acknowledgments
Research funded in part by the Virginia and Daniel K. Ludwig Trust for Cancer Research.
References (19)
- et al.
Desmoids in familial adenomatous polyposis are monoclonal proliferations
Br J Cancer
(2000) - et al.
Evidence for genetic predisposition to desmoid tumors in familial adenomatous polyposis independent of the germline APC mutation
Gut
(2004) - et al.
Desmoid tumors – a characterization of patients seen at Mayo Clinic 1976–1999
Fam Cancer
(2006) - et al.
Successful chemotherapeutic modality of doxorubicin plus dacarbazine for the treatment of desmoid tumors in association with familial adenomatous polyposis
J Clin Oncol
(2006) - et al.
Use of doxorubicin and dacarbazine for the management of unresectable intra-abdominal desmoid tumors in Gardner’s cyndrome
Dis Colon Rectum
(1994) - et al.
Combination chemotherapy in adult desmoid tumors
Cancer
(1993) - et al.
Chemotherapy for desmoid tumors in association with familial adenomatous polyposis
Dis Colon Rectum
(1997) - et al.
Extended follow-up of patients treated with cytotoxic chemotherapy for intraabdominal desmoid tumors
Dis Colon Rectum
(2001) - et al.
Therapy of desmoid tumors and fibromatosis using vinorelbine
Am J Clin Oncol
(1999)
Cited by (65)
Fertility preservation and PGT-M in women with familial adenomatous polyposis-associated desmoid tumours
2021, Reproductive BioMedicine OnlineActive surveillance in desmoid-type fibromatosis: A systematic literature review
2020, European Journal of CancerCitation Excerpt :Only part of the studies used and reported disease response based on RECIST [18]. Some included studies selected patients for the AS approach based on the fact that the patients were unable to tolerate chemotherapy or radiotherapy [28], had unresectable asymptomatic mesenteric masses [25] or had masses that were not life-threatening or at risk for mutilation [22]. Moreover, some studies selected patients based on tumour sites (e.g. breast desmoids [21,23]) or were interested in other study end points than the results of the AS approach (e.g. pregnancy status [2], or imaging characteristics [24,31]).
Soft Tissue Tumors of the Abdomen and Retroperitoneum
2020, Surgical Clinics of North AmericaThe management of desmoid tumours: A joint global consensus-based guideline approach for adult and paediatric patients
2020, European Journal of CancerCitation Excerpt :Likewise, Salas et al. showed a benefit of a surveillance approach although they did not perform a true comparison between groups [8]; “very low” according to GRADE. Other reports focussed on specific anatomical sites: mesenteric (FAP and sporadic patients [9]; “very low” according to GRADE), AW [10]; “very low” according to GRADE, extra-abdominal (EA including extremity/girdles, head & neck, and trunk [11]; “very low” according to GRADE) and abdominal (including both IA and AW [12]; “very low” according to GRADE). In both AW and EA sites, an initial non-surgical approach was shown to be safe, although surgery was offered as an option in a few selected cases.
Inherited Colorectal Cancer and the Genetics of Colorectal Cancer
2019, Shackelford's Surgery of the Alimentary Tract: 2 Volume SetManagement of desmoid disease
2018, Seminars in Colon and Rectal Surgery