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.