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

BACKGROUND: Desmoid-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. METHODS: 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 São Paulo, Brazil. RESULTS: In 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. CONCLUSION: Desmoid 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.

Abdominal wall reconstruction after desmoid type fibromatosis radical resection: Case series from a single institution and review of the literature.

BACKGROUND: Abdominal wall desmoid type fibromatosis management has been changing over recent years, from an aggressive approach towards a more conservative one. When radical resection is indicated, the surgical team faces the challenge of abdominal wall reconstruction, for which optimal technique is still debated. The present study reports the experience from a single center with abdominal closures after desmoid type fibromatosis resection. MATERIAL AND METHODS: Retrospective analysis of patients who underwent abdominal wall closure after sporadic abdominal desmoid type fibromatosis radical resection from 1982 to 2013. RESULTS: Twenty-seven patients were included, mean tumor diameter was 10 + 5.3 cm, and the main choice of abdominal wall reconstruction was midline closure with anterior rectus sheath relaxing incisions and polypropylene onlay mesh (74% of the cases). Only 7% of the cases required more complex procedures for skin closure. Mean follow-up was 5 years and 89% remained disease-free. No grade 4 or 5 complications were observed. CONCLUSION: High midline fascial closure rate can be achieved after resection of abdominal wall desmoid tumor using relaxing incisions and mesh, with low complication rate.