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

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*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

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Abstract

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.

Original languageEnglish
Article number5982575
JournalSarcoma
Volume2018
DOIs
Publication statusPublished - 21 Jun 2018

Research programs

  • EMC OR-01

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