Management of serious complications in intra‐abdominal desmoid‐type fibromatosis

Abstract

Background: Desmoid fibromatosis (DF) is a rare and locally infiltrative monoclonal fibroblastic proliferation arising from connective tissues, with lack of metastatic potential. About 10% of all DF cases are intra-abdominally sited. Complications in this site, due to the locally infiltrative nature of the disease, may be severe and potentially life threatening. However, data on incidence, management, and outcome of these complications are limited. Aim: Data of patients with sporadic or FAP-related intra-abdominal DF treated at Istituto Nazionale dei Tumori (INT) in Milano from 2005 to 2020 who developed a serious complication during the course of their disease were retrospectively collected and analyzed with a descriptive statistics. Methods and Results: Out of 72 intra-abdominal DF, 8 cases were identified (M/F: 5/3, median age: 35 years, FAP-related/sporadic: 2/6): 3 with bowel obstruction, 5 with bowel perforation. In 4 cases the serious complication was the first evidence of disease; in the other 4 cases it occurred at a time interval from diagnosis in the range of 4-44 months (during an active surveillance program in one case and duringchemotherapy in the other 3 cases). A surgical treatment was feasible and successful in 5 cases. In 3 surgically unmanageable patients, all progressing and experiencing acute complications while on chemotherapy, a non-surgical approach with intensive supportive treatment and with a prompt change of chemotherapy regimen was implemented, being successful in two, the other patient dying due to a concomitant progressive lymphoma thereafter. Conclusion:In this series of intra-abdominal DF, the incidence of serious complications was 11%. Most patients were successfully treated with surgery. When surgery was deemed to be unfeasible, a conservative management with intensive supportive care and a careful choice of chemotherapy was adopted, ensuring a favorable outcome in most.

Case report: Intra-abdominal aggressive fibromatosis: A rare cause of hyperemesis

“…Due to the rare distant metastasis rate, the clinical symptoms are often apparent only after severe local invasion. Intra-abdominal AF accounts for approximately 10% of all AF cases ( 3 ), and its manifestations are commonly associated with abdominal pain and gastrointestinal bleeding ( 4 ).…”

“…AF is closely associated with familial adenomatous polyposis (FAP). The risk of developing AF in patients with FAP is approximately 10%–15%, and AF associated with FAP is more aggressive ( 3 ). AF can occur after operations, such as laparoscopic and neck surgery, as well as after trauma ( 5 ).…”

Huge mesenteric desmoid-type fibromatosis with unusual presentation: A case report

“…Given the locally aggressiveness of the disease, this can lead to several life-threatening complications caused by adjacent tissues invasion including bowel obstructions, perforations, and ischaemia [ 12 ].…”

“…We reported a rare case of diffuse peritonitis due to sporadic MDF with aggressive proliferation and consecutive intestinal perforation. Only a few similar cases have been reported in the literature [ [12] , [13] , [14] , [15] ] [ [12] , [13] , [14] , [15] ] [ [12] , [13] , [14] , [15] ].…”

“…Among these patients, only 5 presented with bowel perforation. The DF was of mesenteric origin in all 5 cases [ 12 ].…”