Pancreatic acinar cell carcinoma with bilateral ovarian metastases, panniculitis and polyarthritis treated with FOLFIRINOX chemotherapy regimen. A case report and review of the literature.

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.