The Desmoid Tumor Research Foundation

June 2018

Sporadic Abdominal Wall Desmoid type Fibromatosis: treatment paradigm after thirty two years

Author Information

Couto Netto SD(1), Teixeira F(1), Menegozzo CAM1), Leco-Filho HM()2, AlbertiniA(1), Ferreira FO(3), Akaishi EH(3), Utiyama EM(1).

(1)Dept of Surgery, Division of General Surgery & Trauma, Hospital das Clinicas - University of Sao Paulo, Av. Dr. Enzas de Carvalho Aguiar, 255, S‹ao Paulo, 05403-010, Brazil. (2)Departament of Radiology & Diagnostic Imaging, Sao Paulo Cancer Institute(ICESP), University of Sao Paulo, Sao Paulo, Brazil. (3)Sao Paulo Cancer Institute (ICESP), University of Sao Paulo, Sao Paulo ,Brazil.

Publication

BMC Surg

Summary

We 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 S‹o Paulo, Brazil. In the study period, 32 patients were included. Twenty-seven patients had surgery upfront. Pathology confirmed free margins in 92% of resections. Tumor recurrence rate was 11%, with median relapse-free survival being 24 months. 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. Pharmacological treatment may result in tumor size regression. When surgical excision is indicated, positive margins represent an important prognostic factor for local tumor recurrence.

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