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The Desmoid Tumor Research Foundation

June 2018

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

Author Information

van Broekhoven, D (1), Verschoor A(2), van Dalen T(3,4), Grunhagen D(1), den Bakker M(5), Gelderblom H(2), Bovee J(6), Haas R(7), Bonenkamp H(8), van Coevorden F(9), ten Oever D(10), van der Graaf W (11,12), Flucke U(13), Pras E(14), Reyners A(15), Westermann A(16), Oldenburger F(17), Verhoef C(1) and Steeghs N(10).

(1)Dept of Surgery, Erasmus MC Cancer Institute, P.O. Box 5201, 3008 AE Rotterdam,NL. (2)Dept of Medical Oncology, Leiden University Medical Center, P.O. Box 9600, 2300 RC Leiden, NL. (3)Dept of Surgery, University Medical Center Utrecht, P.O. Box 85500, 3508 GA Utrecht, NL. (4)Dept of Surgery, Diakonessenhuis, Postbus 80250, 3508 TG Utrecht, NL. (5)Dept of Pathology, Erasmus MC Cancer Institute, P.O. Box 5201, 3008 AE Rotterdam, NL. (6)Dept of Pathology, Leiden University Medical Center, P.O. Box 9600, 2300 RC Leiden, NL. (7)Dept of Radiotherapy, Netherlands Cancer Institute-Antoni van Leeuwenhoek, P.O. Box 90203, 1006 BE Amsterdam,NL.(8)Dept of Surgery, Radboud University Medical Center, P.O. Box 9101, 6500 HB Nijmegen, NL. (9)Dept of Surgery, Netherlands Cancer Institute-Antoni van Leeuwenhoek, P.O. Box 90203,1006 BE Amsterdam, NL. (10)Dept of Medical Oncology, Netherlands Cancer Institute-Antoni van Leeuwenhoek, P.O. Box 90203,1006 BE Amsterdam, NL. (11)Dept of Medical Oncology, Radboud University Medical Center, P.O. Box 9101, 6500 HB Nijmegen, NL. (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)Dept of Pathology, Radboud University Medical Center, P.O. Box 9101, 6500 HB Nijmegen, NL. (14)Dept of Radiotherapy, University Medical Center Groningen, University of Groningen, P.O. Box 30001,9700 RB Groningen NL. (15)Dept of Medical Oncology, University Medical Center Groningen, University of Groningen, P.O. Box 30001, 9700 RB Groningen, NL. (16)Dept of Medical Oncology, Academic Medical Center, P.O. Box 22660, 1100 DD Amsterdam, NL. (17)Dept of Radiotherapy, Academic Medical Center, P.O. Box 22660, 1100 DD Amsterdam, NL.

Publication

Hindawi Sarcoma

Summary

This study tries to provide insight on type, usage, and outcome of first-line nonsurgical management strategies. 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. Ninety-one of the 1141 identified patients had first-line nonsurgical management. 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. 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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