The Desmoid Tumor Research Foundation

September 2017

Outcome of Primary Desmoid Tumors at All Anatomic Locations Initially Managed with Active Surveillance

Author Information

van Houdt WJ(1)(2), Husson O(3)(4)(5), Patel A(1), Jones RL(3)(4), Smith MJF(1), Miah AB(6), Messiou C(4)(8), Moskovic E(7), Al-Muderis O(3), Benson C(3), Zaidi S(6), Dunlop A(1)(3)(6), Strauss DC(1), Hayes AJ(1)(4), van der Graaf WTA(3)(8).

(1)Dept of Surgery, Sarcoma Unit, Royal Marsden Hospital, London, UK. (2)Dept of Surgery, Netherlands Cancer Institute, Amsterdam, The Netherlands. (3)Dept of Medical Oncology, Sarcoma Unit, Royal Marsden Hospital, London, UK. (4)The Institute of Cancer Research, London, UK. (5)Dept of Psychosocial Research and Epidemiology, Netherlands Cancer Institute, Amsterdam, The Netherlands. (6)Dept of Radiotherapy, Sarcoma Unit, Royal Marsden Hospital, London, UK. (7)Dept of Radiology, Sarcoma Unit, Royal Marsden Hospital, London, UK. (8)Dept of Medical Oncology, Netherlands Cancer Institute, Amsterdam, The Netherlands.

Publication

Ann Surg Oncol

Summary

This study aimed to evaluate the radiologic and symptomatic course of the disease in patients initially managed with active surveillance. Patients with a primary desmoid tumor at any anatomic location diagnosed between 1998 and 2016 were identified in a prospectively maintained database from a single sarcoma reference center in the United Kingdom. The study identified 168 patients with a primary desmoid tumor initially managed with active surveillance. The tumors were located in the abdominal wall (n?=?61, 36%), an extremity (n?=?51, 30%), chest wall (n?=?30, 18%), intra-abdominal site (n?=?15, 9%), or elsewhere (n?=?11, 6%). Of all the patients, 36% experienced radiologic progressive disease, 36% had stable disease, and 27% regressed. The patients younger than 50 years were more likely to progress (p?=?0.046), whereas the patients with chest wall or upper-extremity tumors reported significantly more pain (p?=?0.01). Eventually, 46% of the patients proceeded to treatment. The median time to start of treatment after initial surveillance was 31 months, whereas the median follow-up time for the patients not receiving any treatment was 40.5 months. The indications for initiation of treatment were pain (32%), progression (31%), or both (13%). Patients with desmoid tumors can be managed with initial active surveillance, although almost half of patients may eventually need treatment. Pain, tumor progression, or both are the most common indications for the initiation of treatment.

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