Sporadic extra abdominal wall desmoid-type fibromatosis: Surgical resection can be safely limited to a minority of patients
Introduction
Surgery was for many years the mainstay of therapy for desmoid-type fibromatosis (DF) [1]. In the past an observational approach was reserved for patients with unresectable/recurring disease [2], [3]. More recently this strategy has been applied also to primary DF [4], [5], with some concerns about tumour location [6], [7], [8]. Abdominal wall DFs are characterised by the most indolent course. We recently showed a 30% spontaneous regression rate in this subgroup [9]. Arguments have been made about using same approach to extra-abdominal wall DF [10]. On this basis we undertook the present analysis in a retrospective series of patients observed and treated at two major reference centers over the past 20-years.
Section snippets
Patients and methods
All consecutive patients with primary sporadic extra-abdominal wall DF treated at Fondazione IRCCS Istituto Nazionale dei Tumori, Milan, Italy and Institute Gustave Roussy, Paris, France between January 1992 and December 2012 were included in this study. Patients with familial adenomatous polyposis-related DF and recurrent disease at presentation were excluded. Patients were divided into two groups according to whether they had received surgery as the initial treatment (surgical group, SG) or
Results
Patient and tumour characteristics are listed in Table 1. There were 135 females/81 males (ratio 1.7/1). Median age of patients at the time of the initial diagnosis was 41 years (interquartile, IQ range, 31–56). The median size of the primary tumour was 7 cm (IQ range, 5–11).
Discussion
In this series of 216 patients affected by primary extra-abdominal wall DF treated over 20-year time span, 5-year-RFS was 80% for patients undergoing surgical resection as the primary approach and 5 year surgery free survival was 95% for patients initially treated with conservative approach (W&S or medical treatments). Sex, size and anatomical site did not predict RFS in the SG group or surgery free survival in the NSG group although a trend towards a better prognosis was observed for
Conflict of interest statement
None declared.
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