Abstract
BackgroundDesmoid-type fibromatosis is a benign mesenchymal neoplastic process. It exhibits an uncertain growth pattern and high recurrence rate. Previously radical surgical resection was the mainstay of treatment, but recently more surgeons are opting for conservative management with observation (“wait and see” policy). The authors intend to evaluate different therapeutic modalities and oncological outcomes for abdominal wall desmoid tumors.MethodsWe performed a retrospective study of patients who underwent surgical, hormonal or chemotherapy treatment for abdominal wall desmoid tumors between 1982 to 2014 at two institutions affiliated with the University of São Paulo, Brazil.ResultsIn the study period, 32 patients were included. Twenty-seven patients had surgery upfront. Of those, 89% were women with a median age of 33 years. Mean tumor size was 10 cm. Pathology confirmed free margins in 92% of resections. Tumor recurrence rate was 11%, with median relapse-free survival being 24 months. Multivariate analysis showed that positive final margins (p < 0.001) and positive frozen section (p = 0.001) were independent predictors of recurrence. For the 5 patients who underwent pharmacological therapy, median age was 33 years and median tumor diameter before treatment was 13 cm. Four patients exhibited partial response by Response Evaluation Criteria in Solid Tumors (RECIST). The single patient who did not respond to RECIST underwent radiotherapy.ConclusionDesmoid tumor treatment has been evolving over the past decade towards a more conservative approach. Pharmacological treatment may result in tumor size regression. When surgical excision is indicated, positive margins represent an important prognostic factor for local tumor recurrence.
Highlights
Desmoid-type fibromatosis is a benign mesenchymal neoplastic process
30% of patients have tumors related to Familial Adenomatous Polyposis (FAP), including those with a mutation on the APC gene [5]
Patients who underwent treatment for histologically confirmed abdominal wall desmoid tumor between 1982 and 2015 at Hospital das Clínicas (HC - FMUSP) and at Sao Paulo State Cancer Institute (ICESP) were retrospectively identified and analyzed. They were divided into two groups, those who were managed with surgery upfront, the surgical group (SG), and those who underwent a conservative non-surgical approach, the non-surgical group (NSG)
Summary
Desmoid-type fibromatosis is a benign mesenchymal neoplastic process. It exhibits an uncertain growth pattern and high recurrence rate. Desmoid-type fibromatosis is a benign mesenchymal neoplasia with monoclonal proliferation [1]. Despite the increased recurrence rate after resection, it exhibits no metastatic potential [2]. Mac Farlane described the disease in 1832 [3]. Desmoid-type fibromatosis is rare, comprising about 3% of soft-tissue tumors. 30% of patients have tumors related to Familial Adenomatous Polyposis (FAP), including those with a mutation on the APC gene [5]. Trauma and previous surgery may be related to he onset of the disease in up to 25% of the cases [2, 9,10,11]
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