Conclusion:
DT in the pancreas is exceedingly rare form of already rare intraabdominal form desmoid type fibromatosis. by virtue of nonspecific clinical presentation or asymptomatic incident mass discovered DT diagnosis is considered challenging, secondly, the lack of specific clinical investigation. immunohistochemical staining is the main pathological diagnosis positive in β-catenin nuclear or negative as in S100 protein.
Radical-margin-free surgical resection remains the mainstay management approach while chemotherapy and/or radiotherapy are not effective and no standardized protocol of consensuses and considered the last resort for only high risk for or advance cases.
long-term follow up is warranted. Due to recurrence behaviour of the tumour.
According to midline English literature our case presents the largest pancreatic DT fibromatosis to be published.