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Clinical and diagnostic problems of desmoid-type fibromatosis of the mesentery: case report and review of the literature.

BACKGROUND
Desmoid-type fibromatosis (DTF) is a "locally aggressive, but not metastasizing" proliferation of soft tissues that is difficult to diagnose pre-operatively. Surgical en bloc resection of abdominal DTF can be difficult or an overtreatment. The diagnosis is therefore crucial for the decision making to minimize unnecessary morbidity.


CASE REPORT
A 33-year old female patient with an incidental intra-operative diagnosis of a 20 cm mesenteric mass involving the duodenum, jejunum, ileum and right colon underwent extended resection for a DTF. A review of the literature, limited only to mesenteric localization of DTF, was performed for the diagnosis, treatment and outcome.


RESULTS
Twentyfive cases of DTF have been analyzed. Pre-operative diagnosis was suspected in 20% but 95,2% of all the patients underwent resection without pre-operative diagnosis. In two cases, intra-operative, frozen section diagnosis was unsuccessfully attempted. Complications were reported in 12%, recurrence in 4%, and mortality in 0% of the patients.


CONCLUSION
Pre-operative diagnosis of mesenteric DTF is challenging in most cases, but it can avoid unnecessary extended surgery. Efforts should be made to achieve a correct pre-operative diagnosis based on more reliable imaging and pathological features, to help in decision making.