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

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.

<p>Feasibility of the Reconstruction with Fascia Lata Patch on the Abdominal Wall Defect After Resection of the Abdominal Desmoid Tumor</p>

“…5 Gronchi et al reported that the recurrence rate is unaffected by whether the margin is positive or negative. 6 Other studies 7 have reported that tumor size, age, and occurrence location are predictors.…”

“…Recently, conservative approaches such as pharmacological or simple observation have been selected as treatment. 7 …”

“…Recently, conservative approaches such as pharmacological or simple observation have been selected as treatment. 7 After the resection of abdominal desmoid tumor, the problem is reconstruction of the abdominal wall defect. Regarding the method of reconstruction of the abdominal wall, besides performing primary closure or using artificial materials such as mesh, reconstruction via transplantation of flap or autologous fascia is selected.…”

Abdominal wall endometriosis versus desmoid tumor – a challenging differential diagnosis

“…The disease occurs more frequently in female patients during their reproduction period, especially during pregnancy or in women with previous C-section [23]. Couto Netto et al present a study of 32 patients diagnosed with DT; 90% of them were female, 62.5% with previous pregnancies and 19% with surgical of the abdominal wall [25]. Leon et al (2015) present the case of a 24-year-old woman, pregnant in the 14 th week of gestation, presenting a palpable abdominal mass at the clinical examination.…”

Clinicopathological characteristics and prognostic factors for the recurrence of abdominal desmoid tumors: a retrospective study of 113 patients from two Chinese hospitals

“…Previous studies have demonstrated that the long-term prognosis of patients with abdominal wall DTs is significantly better than that of patients with intra-abdominal DT. [ 4 , 5 ] Wilkinson et al [ 4 ] reported the prognosis of 50 patients with abdominal wall DTs who underwent surgical resection. Within a median follow-up period of 5 years, the local recurrence rate was only 8% (4/50).…”