RESUMEN
Pancreatic acinar cell carcinoma (PACC) is a rare pancreatic tumor, with an estimated frequency of less than 1% of pancreatic malignancies. There are no prospective studies to guide diagnostic or therapeutic algorithms. We report the case of a 36 year-old woman, diagnosed of a pancreatic tumor with liver and peritoneal metastases that was initially managed as a neuroendocrine tumor with temozolomide and capecitabine. After two cycles a severely painful arthritis developed in her left ankle with panniculitis and extensive fat necrosis, and CT scan demonstrated progressive disease. Pathology of the primary was reassessed establishing the diagnosis of PACC. The patient started treatment with FOLFIRINOX regimen, achieving clinical benefit and disease stabilization. We also briefly reviewed the literature on this rare subtype of pancreatic tumor.