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Annals of Surgical Oncology

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

Winan J. van Houdt MD, PhD, MSc, Olga Husson PhD, Alisha Patel, Robin L. Jones MD, Myles J. F. Smith MB, BCh, BAO, PhD, FRCSI, Aisha B. Miah MD, PhD, Christina Messiou MD, Eleonor Moskovic MD, Omar Al-Muderis, Charlotte Benson MD, Shane Zaidi MD PhD, Alison Dunlop, Dirk C. Strauss MD, Andrew J. Hayes MD, PhD, Winette T. A. van der Graaf MD, PhD
Sarcoma
Volume 26, Issue 13 / December , 2019

Abstract

Background

The behavior of desmoid tumors is unpredictable and varies from spontaneous remission to symptomatic and radiologic progression. This study aimed to evaluate the radiologic and symptomatic course of the disease in patients initially managed with active surveillance.

Methods

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. Inverse univariate Cox proportional hazard regression analyses were conducted to evaluate the course of the disease and indications for initiating treatment.

Results

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%).

Conclusions

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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