Sporadic extra abdominal wall desmoid-type fibromatosis: Surgical resection can be safely limited to a minority of patients

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Abstract

Background

To analyse the natural history of extra-abdominal wall desmoid-type fibromatosis (DF) and compare outcome in patients who underwent initial surgery with those who did not.

Patients and methods

All consecutive patients affected by primary sporadic extra-abdominal wall DF observed between January 1992 and December 2012 were included. Patients were divided into surgical (SG) or non-surgical groups (NSG) according to initial treatment. Relapse free survival was calculated for SG, and crude cumulative incidence (CCI) of switching to surgery or other treatments for NSG.

Results

216 patients were identified, 94 in SG (43%), 122 in NSG (57%). A shift towards a more systematic use of a conservative approach (78% of all comers) was observed in the latter years (2006–2012), although a small proportion of patients (28%) had been offered the conservative strategy even in the early period (1992–2005). Median follow-up (FU) was 49 mo. (interquartile (IQ), 20–89 mo.), 76 months for SG and 39 months for NSG. 5-year relapse-free survival (RFS) for SG was 80% (95% confidence interval (CI), 72–89%). For the NSG, 5-year CCI of switching to surgery was 5% (95% CI: 1.7%, 14%), and 51% to other treatments (95% CI: 41%, 65%). 27 (20%) NSG patients underwent spontaneous regression.

Conclusion

A non-surgical approach to extra-abdominal wall DF allowed surgery to be avoided in the majority of patients. This approach can be safely proposed and surgery offered as an option in selected cases.

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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