Introduction: Desmoid-type Fibromatoses (DTF) tumours are rare, benign fibrous tumours with aggressive invasive behaviour that account for approximately 0.03% of all neoplasms. We report the success in curing a rare, invasive, and huge pancreatic intraabdominal DTF.

Presentation of case: A 42 years old male was medically free apart from recurrent left upper abdominal pain, anorexia, and nausea for more than ten years and no significant past surgeries, trauma, or family history of cancer. The patient has a non-tender large abdominal mass at the left hypochondria area extending down to the pelvis below the umbilicus with a rigid and smooth surface. The computed tomography scan showed a huge heterogeneous mass appears to be of pancreatic origin, measuring about 23 cm by 15 cm by 11 cm. The patient underwent radical antegrade modular pancreato-splenectomy, segmental transverse colectomy, adrenalectomy, and subsequent colo-colic anastomosis. The accurate gross size of the tumour specimen was 26 × 17 × 9 cm, and the weight was found to be 3.6 kg. Immunohistochemistry confirmed the diagnosis of pancreas DTF. The follow up to 5 years confirmed no recurrence reported clinically or by imaging.

Discussion: The Pancreas origin of DTF is a rarely reported subset with an incidence of around 5% of all DTF. Establishing the diagnosis is fundamentally based on the characteristic pathological and immunohistochemical studies, for the only available cure modality by complete radical resection to be promptly offered.

Conclusion: Our case is rare and uniquely the largest pancreatic DTF reported in the literature with curative resection despite being locally invasive.

Keywords: Desmoid tumour; Desmoid type fibromatosis; Fibromatosis; Pancreatic desmoid tumour; Radical pancreas resection; S100 protein; β-Catenin.

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