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

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-ab...

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Published in:Sarcoma 2018, Vol.2018, p.5982575-8
Main Authors: van Broekhoven, Danique L. M., Verschoor, Arie J., van Dalen, Thijs, Grünhagen, Dirk J., den Bakker, Michael A., Gelderblom, Hans, Bovee, Judith V. M. G., Haas, Rick L. M., Bonenkamp, Han J., van Coevorden, Frits, ten Oever, Diederik, van der Graaf, Winette T. A., Flucke, Uta E., Pras, Elisabeth, Reyners, Anna K. L., Westermann, Anneke M., Oldenburger, Foppe, Verhoef, Cornelis, Steeghs, Neeltje
Format: Article
Language:English
Subjects:
Abdomen
Abdominal wall
Analysis
Biopsy
Cancer
Care and treatment
Decision making
Fibromas
Gender
Localization
Management
Medical records
Medical research
Medicine, Experimental
Mutation
Nonsteroidal anti-inflammatory drugs
Oncology
Pathology
Patients
Population studies
Population-based studies
Radiation therapy
Radiotherapy
Studies
Surgery
Surveillance
Tumors
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Summary: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.
ISSN:1357-714X
1369-1643
DOI:10.1155/2018/5982575