Outcome of Nonsurgical Management of Extra-Abdominal, Trunk, and Abdominal Wall Desmoid-Type Fibromatosis: A Population-Based Study in the Netherlands

• Main Authors: Danique L. M. van Broekhoven, Arie J. Verschoor, Thijs van Dalen, Dirk J. Grünhagen, Michael A. den Bakker, Hans Gelderblom, Judith V. M. G. Bovee, Rick L. M. Haas, Han J. Bonenkamp, Frits van Coevorden, Diederik ten Oever, Winette T. A. van der Graaf, Uta E. Flucke, Elisabeth Pras, Anna K. L. Reyners, Anneke M. Westermann, Foppe Oldenburger, Cornelis Verhoef, Neeltje Steeghs
• Format: Article
• Language: English
• Published: Hindawi Limited 2018-01-01
• Series: Sarcoma
• Online Access: http://dx.doi.org/10.1155/2018/5982575
• ISSN: 1357-714X; 1369-1643

Introduction. Nonsurgical management of patients with desmoid-type fibromatosis (DF) is increasing. This study tries to provide insight on type, usage, and outcome of first-line nonsurgical management strategies. Patients and Methods. From the Dutch Pathology Registry (PALGA), patients with extra-abdominal or trunk/abdominal wall DF, diagnosed between 1993 and 2013, were identified. First-line treatment was analyzed. Best response (BR) using RECIST criteria from start of treatment/surveillance until change of treatment or last follow-up was analyzed. Results. Ninety-one of the 1141 identified patients had first-line nonsurgical management. The percentage of patients treated nonsurgically increased from 0.6% in 1993–1998 to 12.8% in 2009–2013. Thirty-seven patients had surveillance (41%), 35 radiotherapy (38%), and 19 systemic treatment (21%). BR for surveillance was complete response (CR) in 2/37, partial response (PR) in 4/37, stable disease (SD) in 21/37, progressive disease (PD) in 5/37, and unknown in 5/37 patients. BR for radiotherapy was CR in 4/35, PR in 11/35, SD in 16/35, and unknown in 4/35. BR for systemic treatment was CR in 1/19, PR in 1/19, SD in 10/19, PD in 2/19, and unknown in 5/19. Totally, 91% of patients did not progress. Discussion. Given the low percentage (9%) of PD of nonsurgical management, these data can be used in shared decision making with the patient regarding optimal treatment.