Sarcoma (Jan 2018)

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

DOI
https://doi.org/10.1155/2018/5982575
Journal volume & issue
Vol. 2018

Abstract

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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.