Abstract
Clear cell pancreatic carcinoma, as a primary lesion, represents a rare malignant entity with very few references. As for the incidence, the clinical characteristics and the prognosis are still to be defined. Here, we present the case of a 73-year-old female who presented with epigastric pain radiating to the back, anorexia, and dyspepsia. Abdominal computed tomography (CT) showed a well-circumscribed, low-attenuating tumor with peripheral enhancement, arising from the upper border of the pancreatic head. There were no metastases or other primary tumors. Radical resection of the tumor was undertaken. Histopathology showed round-to-oval neoplastic cells with a well-defined cell membrane, prominent cell borders, abundant clear cytoplasm, and centrally located nuclei. The periodic acid-Schiff reaction was positive and a diagnosis of primary clear cell pancreatic carcinoma was made. No adjuvant treatment was given. She remained under regular follow-ups with abdominal and thoracic CT scans for seven years without evidence of recurrence of other primary tumors. She was deceased 87 months after tumor resection because of conditions unrelated to the disease. This is the first case to the best of our knowledge, of long-term survival after radical resection of a primary pancreatic clear cell carcinoma, suggesting surgery as a treatment option for this rare tumor and review of the relevant literature.