Time Trends in the Incidence and Treatment of Extra-Abdominal and Abdominal Aggressive Fibromatosis: A Population-Based Study

Abstract

BackgroundAggressive 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.MethodsIn 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 χ2and regression analysis.ResultsDuring 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.

Identification and assessment of health-related quality of life issues in patients with sporadic desmoid-type fibromatosis: a literature review and focus group study

“…However, due to its local aggressive behaviour and its known tendency of local recurrence after initial surgical resection, it is categorised as a borderline tumour [1]. Desmoid-type fibromatosis is rare, with a reported incidence of 5.4 new cases per million persons per year in the Dutch population [2]. Symptoms vary, depending on tumour location and size, and can be very severe.…”

Fibromatosis of the Male Breast: A Case Report

“…While this entity has been described extensively in the trunk and extremities, there are few case series reported in the literature (PubMed and Medline) of fibromatosis primarily the male breast. Genetic alterations in male breast fibromatosis have not been characterized previously.Various etiologies have been evoked including endocrine and genetic factors as well as surgical trauma (5,6). It is a locally aggressive and infiltrative tumor with no potential for distant metastasis.…”

Mesenteric fibromatosis after resection for gastrointestinal stromal tumor of stomach

“…Morbidity due to mesenteric fibromatosis increased from 2.10 to 5.36 per million population annually from 1993 to 2013. [ 13 ] Mesenteric fibromatosis is a group of fibroblastic or myofibroblastic tissue that develops ordinarily because of surgery or trauma experiences, but which can appear spontaneously. About 20% of patients diagnosed as mesenteric fibromatosis are associated with familial adenomatous polyposis, and a few cases have uncertain reasons that may include radiation exposure.…”