Optimal management of desmoid fibromatosis

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.

We investigated the prognostic role of cyclin A and Ki67 in desmoid tumors by immunohistochemistry. The study included 76 patients with desmoid tumor operated at Helsinki University Hospital between 1987 and 2011. A tissue micro array (TMA) was constructed and the TMA sections were immunostained with cyclin A and Ki67 antibodies. Cyclin A expression correlated significantly with Ki67. Cyclin A expression was associated with recurrence-free survival (HR 1.9, 95% CI = 1.1-3.2, P = .02), as were positive margin (HR 6.0, 95% CI = 1.6-22.5, P = .008) and extremity location (HR 5.3, 95% CI = 1.7-16.8, P = 0.005). Ki67 immunopositivity varied from 0.33% to 13.8%, with a mean of 4.6%, but had no significant prognostic impact (HR 1.1, P = .2). Our study indicates that cyclin A may be a new prognostic biomarker in surgically treated desmoid tumors.

A 39-year-old female patient was referred to our tertiary oncologic center for additional investigations relating to a suspect pancreatic tail lesion. An abdominal computed tomography scan and magnetic resonance imaging scan showed a solid lesion demonstrating progressive enhancement. Complete resection was obtained and the final diagnosis was that of a desmoid tumor of the pancreas, an exceptionally rare tumor demonstrating overlap with other solid and cystic lesions of the pancreas [1]. Therefore, it is important to recognize the essential role of pathology, particularly immunohistochemistry, in identifying this tumor. The high rate of postsurgical recurrence should prompt repeated follow-ups considering the potential aggressive nature of desmoid tumors.

The aim of the paper is to present a case of a 33-year-old woman with diagnosed familial polyposis in the colon, who has been identified with mesenteric desmoid.