Long-term survival after a repetitive surgical approach in a patient with acinar cell carcinoma of the pancreas and recurrent liver metastases: report of a case.

Acinar cell carcinoma is a relatively rare malignant neoplasm, which represents 1%-2% of all pancreatic exocrine tumors. Its prognosis is thought to be poor, especially when it metastasizes to the liver. This report concerns a case of a long-term survivor of metastatic acinar cell carcinoma who was successfully treated with repetitive surgery. A 62-year-old man underwent a distal pancreatectomy for a pancreatic tumor, which was histologically diagnosed as an acinar cell carcinoma. The tumor recurred in the liver three times within 41 months. At the first recurrence, four hepatic lesions appeared 7 months after the initial pancreatectomy and were managed with an extended left hepatic lobectomy and partial liver resection. Thereafter, a solitary nodule in Segment 6 was identified 21 months after the second surgery and was treated with a partial liver resection. A solitary lesion in Segment 8/5 appeared 11 months after the third surgery and was also managed by a partial liver resection. The patient has remained disease-free for 22 months since the last surgery and has survived 65 months since the initial diagnosis. Although no consensus has been reached on surgery for metastatic acinar cell carcinoma, the current case has important implications for establishing an appropriate treatment strategy.