Primary hepatic neuroendocrine tumor case with a preoperative course of 26 years: A case report and literature review

Abstract

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.

Primary hepatic neuroendocrine tumor with multiple liver metastases: A case report with literature review

“…Clinically, 5-hydroxyindoleacetic acid in 24-hour urine specimens and the serum level of chromogranin A are commonly used diagnostic markers. 1 Unfortunately, because of the rarity of PHNETs, these diagnostic markers are rarely included in routine preoperative biochemistry panels.…”

“…In a recent report, the age-adjusted annual incidence of NETs was 1.09 per 100,000 persons in 1973, but the incidence increased to 6.98 per 100,000 persons by 2012. 1,2 Liver metastases are common in neuroendocrine neoplasms (NENs), although the liver itself is rarely described as the site of the primary tumor. 1,3 Until 2019, 150 cases of NENs were reported in the English literature 1,5 since the first report of this disease by Edmondson 4 in 1958.…”

Primary hepatic neuroendocrine tumours—Case series of a rare malignancy

“…Haematoxylin and Eosin stains classically show trabecular, glandular or solid cell patterns but is not specific for PHNET [7]. IHC staining typically show positivity to CgA (66.7–95% immunoreactivity) [3,13], neuron-specific enolase (74.1–90% immunoreactivity) [13,14] and synaptophysin (48.9–91.7% immunoreactivity) [13] as well as CD56 and cytokeratin [15].…”

18F-FDG-PET/CT Findings of a Primary Hepatic Neuroendocrine Tumor: A Case Report and Literature Review

“…PHNET is a low-grade and slow-growing malignant tumor. Surgical resection of PHNETs is the main treatment choice for those patients eligible for surgery [15,16] and early surgical resection results in a higher long-term progression-free survival and overall survival compared with other types of liver cancer [17][18][19]. Peptide Receptor Radionuclide Therapy (PRRT) using 90 Y-and 177 Lu-labelled peptides has been used over the last 15 years as a treatment option for neuroendocrine tumors, achieving approximately a 30% response rate [20].…”