Primary hepatic neuroendocrine tumor (PHNET) is an extremely rare liver tumor. Patients often have no clinical symptoms or have only non-specific symptoms, such as abdominal pain and abdominal mass. The clinical manifestations, disease development, treatment methods, and treatment outcomes of PHNET vary greatly among cases. Here we report a case of PHNET with a confirmed 26-year survival before surgery. The patient was a 56-year-old female. A large right hepatic mass was detected when the patient was 30 years old. The tumor could not be removed during exploratory laparotomy, and constriction of the right hepatic artery and biopsy were conducted. Pathological results indicated a diagnosis of benign tumor, but a confirmed diagnosis was not reached. Twenty-six years after the patient had been living with the tumor, she sought treatment again because of tumor progression. After systematic evaluation of the resectability, the tumor was resected. Based on the examination results of the gastrointestinal tract and lungs, intraoperative examination results, pathological findings, and long-term follow-up results, the diagnosis of PHNET was confirmed. This case represents the longest reported survival time for a PHNET patient before removal of the tumor.
Keywords: Follow up; Pathology; Primary hepatic neuroendocrine tumor; Radiology; Surgery; Surgical plan.