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Sarcoma. 2018 Jun 21;2018:5982575. doi: 10.1155/2018/5982575. eCollection 2018.

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

Sarcoma

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

Affiliations

  1. Department of Surgery, Erasmus MC Cancer Institute, P.O. Box 5201, 3008 AE Rotterdam, Netherlands.
  2. Department of Medical Oncology, Leiden University Medical Center, P.O. Box 9600, 2300 RC Leiden, Netherlands.
  3. Department of Surgery, University Medical Center Utrecht, P.O. Box 85500, 3508 GA Utrecht, Netherlands.
  4. Department of Surgery, Diakonessenhuis, Postbus 80250, 3508 TG Utrecht, Netherlands.
  5. Department of Pathology, Erasmus MC Cancer Institute, P.O. Box 5201, 3008 AE Rotterdam, Netherlands.
  6. Department of Pathology, Leiden University Medical Center, P.O. Box 9600, 2300 RC Leiden, Netherlands.
  7. Department of Radiotherapy, Netherlands Cancer Institute-Antoni van Leeuwenhoek, P.O. Box 90203, 1006 BE Amsterdam, Netherlands.
  8. Department of Surgery, Radboud University Medical Center, P.O. Box 9101, 6500 HB Nijmegen, Netherlands.
  9. Department of Surgery, Netherlands Cancer Institute-Antoni van Leeuwenhoek, P.O. Box 90203, 1006 BE Amsterdam, Netherlands.
  10. Department of Medical Oncology, Netherlands Cancer Institute-Antoni van Leeuwenhoek, P.O. Box 90203, 1006 BE Amsterdam, Netherlands.
  11. Deparment of Medical Oncology, Radboud University Medical Center, P.O. Box 9101, 6500 HB Nijmegen, Netherlands.
  12. Clinical and Translational Sarcoma Research, The Institute of Cancer Research, The Royal Marsden NHS Foundation Trust, 15 Cotswold Road, Sutton, London, Surrey SM2 5NG, UK.
  13. Department of Pathology, Radboud University Medical Center, P.O. Box 9101, 6500 HB Nijmegen, Netherlands.
  14. Department of Radiotherapy, University Medical Center Groningen, University of Groningen, P.O. Box 30001, 9700 RB Groningen, Netherlands.
  15. Department of Medical Oncology, University Medical Center Groningen, University of Groningen, P.O. Box 30001, 9700 RB Groningen, Netherlands.
  16. Department of Medical Oncology, Academic Medical Center, P.O. Box 22660, 1100 DD Amsterdam, Netherlands.
  17. Department of Radiotherapy, Academic Medical Center, P.O. Box 22660, 1100 DD Amsterdam, Netherlands.

PMID: 30034268 PMCID: PMC6032644 DOI: 10.1155/2018/5982575

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.

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