Abstract
BACKGROUND: Pancreatic cancer is associated with a dismal prognosis, with ductal adenocarcinoma being the most common form of primary neoplasm diagnosed. Clear cell carcinoma is usually associated with kidney, ovarian or bladder malignancy but rarely associated with pancreatic malignancy. According to the WHO classification, primary clear cell adenocarcinoma of the pancreas is a rare "miscellaneous" carcinoma, and to date few cases have been reported in the literature. CASE: A 63-year old female who presented to a metropolitan hospital in Australia with abdominal pain suggestive of pancreatitis. Abdominal computed tomography subsequently demonstrated a necrotic mass in the neck of the pancreas with hepatic metastasis. Subsequent histopathological and immunohistochemical analysis was consistent with pancreatic clear cell adenocarcinoma. The patient was discharged to home to commence on dose reduced nab-paclitaxel and gemcitabine. CONCLUSION: This case reports a rare variant of pancreatic malignancy and contributes further evidence to the body of literature highlighting clear cell adenocarcinoma as a histological subtype of pancreatic neoplasm, albeit rare in nature.