Invasive giant pancreatic desmoid-type fibromatosis with curative resection: A case report
Autor: | Hanan M. Alghamdi |
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Jazyk: | angličtina |
Rok vydání: | 2021 |
Předmět: |
medicine.medical_specialty
business.industry β-Catenin Umbilicus (mollusc) Fibromatosis Cancer Case Report Radical pancreas resection Anastomosis medicine.disease Desmoid tumour Abdominal mass S100 protein medicine.anatomical_structure medicine Transverse Colectomy Desmoid type fibromatosis Surgery Radiology medicine.symptom Pancreatic desmoid tumour Pancreas business Pelvis |
Zdroj: | International Journal of Surgery Case Reports |
ISSN: | 2210-2612 |
Popis: | 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. Highlights • Desmoid-type Fibromatoses (DTF) tumours are rare, benign fibrous tumours with aggressive invasive behaviour. • Account for approximately 0.03% of all neoplasms. • The Pancreas is a rarely reported subset with an incidence of around 5% of all DTF. • Accurate diagnosis is fundamentally by characteristic pathological and immunohistochemical studies. • Cure can be achieved only by complete radical resection • We report curative resection of a rare and the largest pancreatic locally invasive DTF (26x17x9 cm in size and weight 3.6Kg) |
Databáze: | OpenAIRE |
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