Elsevier

European Journal of Cancer

Volume 44, Issue 16, November 2008, Pages 2404-2410
European Journal of Cancer

Multimodality treatment of mesenteric desmoid tumours

https://doi.org/10.1016/j.ejca.2008.06.038Get rights and content

Abstract

Background

Desmoid tumours are rare neoplasms characterised by clonal proliferation of myofibroblasts that do not metastasise, but often exhibit an infiltrative pattern and functional impairment. When desmoids arise in the intestinal mesentery, surgical resection is seldom possible without life-altering loss of intestinal function.

Methods

Retrospective review of the clinical management of 52 consecutive patients treated for desmoids of the intestinal mesentery from January 2001 to August 2006. A multidisciplinary treatment plan was developed based on primary disease extent, tumour behaviour and resectability. Patients with stable but unresectable disease were observed without treatment. Patients with resectable disease underwent surgery, and patients with unresectable progressing disease received chemotherapy, most commonly liposomal doxorubicin, followed by surgery if chemotherapy rendered the disease resectable.

Results

At a median follow-up of 50.0 months (range 4.6–212), 50 patients (96%) have either no recurrence or radiographically stable disease. No patient requires total parenteral nutrition.

Conclusion

These data indicate that the extent of disease; tumour behaviour and resectability are the important factors when defining a treatment plan for mesenteric desmoid tumours. A multidisciplinary approach of surgery combined with chemotherapy is an effective and function-sparing strategy for managing this disease.

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.

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