Winan J van Houdt1,2, Olga Husson3,4,5, Alisha Patel6, Robin L Jones3,4, Myles J F Smith6, Aisha B Miah7, Christina Messiou4,8, Eleonor Moskovic8, Omar Al-Muderis3, Charlotte Benson3, Shane Zaidi7, Alison Dunlop6,3,7, Dirk C Strauss6, Andrew J Hayes6,4, Winette T A van der Graaf3,9. 1. Department of Surgery, Sarcoma Unit, Royal Marsden Hospital, London, UK. w.v.houdt@nki.nl. 2. Department of Surgery, Netherlands Cancer Institute, Amsterdam, The Netherlands. w.v.houdt@nki.nl. 3. Department of Medical Oncology, Sarcoma Unit, Royal Marsden Hospital, London, UK. 4. The Institute of Cancer Research, London, UK. 5. Department of Psychosocial Research and Epidemiology, Netherlands Cancer Institute, Amsterdam, The Netherlands. 6. Department of Surgery, Sarcoma Unit, Royal Marsden Hospital, London, UK. 7. Department of Radiotherapy, Sarcoma Unit, Royal Marsden Hospital, London, UK. 8. Department of Radiology, Sarcoma Unit, Royal Marsden Hospital, London, UK. 9. Department of Medical Oncology, Netherlands Cancer Institute, Amsterdam, The Netherlands.
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.
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.
Authors: Andrew J Bishop; Jace P Landry; Christina L Roland; Ravin Ratan; Barry W Feig; Bryan S Moon; Maria A Zarzour; Wei-Lien Wang; Alexander J Lazar; Valerae O Lewis; Keila E Torres; B Ashleigh Guadagnolo Journal: Cancer Date: 2020-04-28 Impact factor: 6.860
Authors: Anne-Rose W Schut; Emma Lidington; Milea J M Timbergen; Eugenie Younger; Winette T A van der Graaf; Winan J van Houdt; Johannes J Bonenkamp; Robin L Jones; Dirk J Grünhagen; Stefan Sleijfer; Cornelis Verhoef; Spyridon Gennatas; Olga Husson Journal: Cancers (Basel) Date: 2022-06-16 Impact factor: 6.575
Authors: Anne-Rose W Schut; Emma Lidington; Milea J M Timbergen; Eugenie Younger; Winette T A van der Graaf; Winan J van Houdt; Johannes J Bonenkamp; Robin L Jones; Dirk J Grünhagen; Stefan Sleijfer; Cornelis Verhoef; Spyridon Gennatas; Olga Husson Journal: Cancers (Basel) Date: 2022-01-29 Impact factor: 6.639
Authors: Federica Bini; Marco Fiore; Salvatore Provenzano; Rossella Bertulli; Arianna Ottini; Chiara Colombo; Marco Vitellaro; Gabriella Greco; Carlo Morosi; Alessandro Gronchi; Paolo Giovanni Casali; Elena Palassini Journal: Cancer Rep (Hoboken) Date: 2021-06-24