Time trends in the incidence and treatment of extra-abdominal and abdominal aggressive fibromatosis: a population-based study

Danique L M van Broekhoven, Dirk J Grünhagen, Michael A den Bakker, Thijs van Dalen, Cornelis Verhoef, Danique L M van Broekhoven, Dirk J Grünhagen, Michael A den Bakker, Thijs van Dalen, Cornelis Verhoef

Abstract

Background: Aggressive fibromatosis (AF) is a locally infiltrating soft-tissue tumor. In a population-based study in the Netherlands, we evaluated time trends for the incidence and treatment of AF.

Methods: In PALGA: Dutch Pathology Registry, all patients diagnosed between 1993 and 2013 as having extra-abdominal or abdominal wall aggressive fibromatosis were identified and available pathology data of the patients were evaluated. Epidemiological and treatment-related factors were analyzed with χ (2)and regression analysis.

Results: During the study period, 1134 patients were identified. The incidence increased from 2.10 to 5.36 per million people per year. Median age at the time of diagnosis increased annually by B 0.285 (P = 0.001). Female gender prevailed and increased over time [annual odds ratio (OR) 1.022; P = 0.058]. All anatomic localizations, but in particular truncal tumors, became more frequent. During the study period diagnostic histological biopsies were performed more often (annual OR 1.096; P < 0.001). The proportion of patients who underwent surgical treatment decreased (annual OR 0.928; P < 0.001). When resection was preceded by biopsy, 49.8 % of the patients had R0-resection versus 30.7 % in patients without biopsy (P < 0.001).

Conclusions: In this population-based study, an increasing incidence of extra-abdominal and abdominal-wall aggressive fibromatosis was observed. The workup of patients improved and a trend towards a nonsurgical treatment policy was observed.

Figures

Fig. 1
Fig. 1
Incidence of aggressive fibromatosis, per million people
Fig. 2
Fig. 2
Distribution among age and localization. a Distribution among age during study period. b Percentage of age distribution. c Distribution among localization during study period. d Distribution of localization per age group
Fig. 3
Fig. 3
Type of pathology records per patient
Fig. 4
Fig. 4
Absolute number of most common abdominal wall surgery, in relation to the absolute number of patients with abdominal wall AF (on secondary axis)
Fig. 5
Fig. 5
Abdominal surgery in the Netherlands

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