Introduction:
Desmoid tumour (DT) also called desmoid-type fibromatosis and aggressive fibromatosis, consider to be benign neoplasm in nature but locally invasive giving it the aggressive behaviour and fatal extent in advanced stages but without potential predilection for distant metastasis. The origin of DT is through proliferation from well differentiated mesenchymal fibroblasts. Types of DT (desmoids tumour) fibromatosis either abdominal wall, Extra-abdominal fibromatosis: mostly in the chest wall muscle, shoulder, thigh and back or Intra-abdominal fibromatosis originating from the mesentery or locating in the retroperitoneal and pelvic region or associated specifically with Gardner’s syndrome. The consistency of DT mass varies between cystic, solid, or mixed solid and cystic form. The majority of DT present as extra-abdominal mass (in the muscle of the abdominal wall) and less commonly as intra-abdominally, occasionally can raise from any fibrous connective tissues in any location [1,2].
Several possible risk factors or associated conditions reported in the literature may attribute to the development of DT include the history of DT in the family, exposure to irradiation, trauma, oral contraceptives bills use or pregnancy, some genetic mutation, and in familial adenomatosis polyposis (FAP) in 10-20% of cases or Gardner syndrome, nevertheless, there is no definitive aetiology of DT [1-7]. Desmoid-type fibromatosis tumours diagnosis is primarily based on pathological and immunohistochemical studies features. Surgical resection of the tumour is the primary option.
The presented case is a rare DT occupying the whole distal part of the pancreas up to the neck of the pancreas, furthermore, it is considered to be the largest pancreatic mass to be ever reported in the literature and the first case of a pancreatic DT in Saudi Arabia, diagnosed in October 2016, at the.