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The Desmoid Tumor Research Foundation

May 2023

Desmoid Tumors in Familial Adenomatous Polyposis Patients: Favorable Outcomes with Multidisciplinary Management.

Author Information

Al-Sukhni, Eisar; Shapiro, Joel; Suraweera, Harini; Semotiuk, Kara; Swallow, Carol J.; Brar, Savtaj; Razak, Albiruni; Gupta, Abha A.; Gladdy, Rebecca A.

1Division of Surgical Oncology, Mount Sinai Hospital, Princess Margaret Cancer Centre, Toronto, Canada. 2Department of Surgery, University of Toronto, Toronto, ON, Canada. 3Zane Cohen Centre for Digestive Diseases, Familial Gastrointestinal Cancer Registry, Mount Sinai Hospital, Toronto, ON, Canada. 4Division of Medical Oncology and Hematology, Princess Margaret Cancer Centre, University Health Network, Toronto, ON, Canada. 5Department of Medicine, University of Toronto, Toronto, ON, Canada. 6Department of Pediatrics, Hospital for Sick Children, University of Toronto, Toronto, ON, Canada. 7Division of Surgical Oncology, Mount Sinai Hospital, Princess Margaret Cancer Centre, Toronto, Canada. rebecca.gladdy@sinaihealth.ca. 8Department of Surgery, University of Toronto, Toronto, ON, Canada. rebecca.gladdy@sinaihealth.ca. #Contributed equally.

Publication

Ann Surg Oncol

Summary

OBJECTIVE: In this study, we aimed to describe the clinical features, management, and outcomes of desmoid tumors (DTs) in familial adenomatous polyposis (FAP) patients at a high-volume sarcoma center. METHODS: Consecutive patients with FAP and DTs were identified from our institutional databases (1985-2021). Patient demographics, treatment, and outcomes were described. Categorical data were compared using Fisher's exact test, and Kaplan-Meier curves were used to estimate progression-free survival (PFS). RESULTS: Forty-five patients with 67 DTs were identified: 39 mesenteric or retroperitoneal (58.2%), 17 abdominal wall (25.4%), 4 extremity (6%), 4 breast (6%) and 3 back (4.4%). Severe DT symptoms were present in 12 patients (26.7%). Initial treatments per tumor were observation in 30 (44.8%) DTs, chemotherapy in 15 (22.4%) DTs, surgery in 10 (14.9%) DTs, and other systemic therapies in 10 (14.9%) DTs. The majority of DTs remained stable with observation or a single intervention (77.8%). Median PFS was 23.4 years (95% confidence interval 7.6-39.2). In the 12 severely symptomatic patients, four patients required more than two interventions for DT control. At a median follow-up of 6.0 years (range 0.7-35.8 years), 33 (73.3%) patients were alive with disease, 7 (15.6%) were alive without disease, and 5 (11.1%) died of other causes. No patients died of DT-related complications. CONCLUSIONS: The majority of DTs in FAP patients remained stable with observation or a single intervention. There were no DT-related deaths; however, 12 of 45 patients (26.7%) experienced significant tumor morbidity and required more interventions for disease control. Further studies on quality of life are required.

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