Abstract
Pancreatic cancer accounts for less than 5% of all cancers. And the rarest type of them all is the pancreatic acinar cell carcinoma accounting for less than 0.05% of all cancers. Acinar cell carcinomas are highly aggressive neoplasms, with a median DFS (Disease Free State) for patients with localized disease and metastatic disease of 47 and 14 months, respectively, and an overall 5-year survival rate ranging from 36.2% to 72.8% in surgically resected individuals.
A 21-year old man presented with non-specific symptoms like progressive abdominal pain, weight loss, nausea and vomiting, melena, weakness, anorexia. Patient was a smoker and consumed moderate amounts of alcohol. There was no family history of gastrointestinal disease. Examinations including enhanced abdominal and pelvic CT scans, chest X-ray, abdominal ultrasound, tumour markers, liver and renal function and coagulation function were performed.
With the imaging, the differential diagnosis could be well-differentiated pancreatic endocrine neoplasms, solid pseudopapillary neoplasms, mixed acinar neoplasms and pancreatoblastomas.
He was referred to surgical oncology for tumour resection. The following day, a modified laparoscopic Whipple (pylorus preserving pancreaticoduodenectomy) was performed. The reason being, aggressive surgical resection with negative margins is associated with long-term survival. Negative margins were obtained in the pancreatectomy specimen. Post operative histological analysis revealed abundant material with cells depicting varying degrees of acinar differentiation and lack ductal and endocrine cells. The aspirates contained mostly cohesive fragments forming acini, cellular cords or solid nests of neoplastic epithelium. The neoplastic cells did not show the compact, orderly lobular “bunch of grapes” arrangement of normal epithelium.
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