Author(s):
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Broekhoven, D.L.M. van
; Verschoor, A.J.; Dalen, T. van; Grunhagen, D.J.; Bakker, M.A. den; Gelderblom, H.; Bovee, J.; Haas, R.L.M.;
Bonenkamp, H.
; Coevorden, F. van; Oever, D. Ten;
Graaf, W.T.A. van der
;
Flucke, U.E.
; Pras, E.; Reyners, A.K.; Westermann, A.M.; Oldenburger, F.; Verhoef, C.; Steeghs, N.
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Subject:
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Radboudumc 14: Tumours of the digestive tract RIHS: Radboud Institute for Health Sciences Radboudumc 9: Rare cancers RIHS: Radboud Institute for Health Sciences Radboudumc 9: Rare cancers RIMLS: Radboud Institute for Molecular Life Sciences |
Organization:
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Surgery Medical Oncology Pathology |
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.
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