Long term recurrence in primary liver neuroendocrine tumor: Report of a single case and review of literature

Primary liver neuroendocrine tumors (PLNETs) are rare tumors of the liver. They share some common characteristics with neuroendocrine tumors (NETs) of the extrahepatic bile ducts, such as slow rise, hormonal, and histological features. Nevertheless, they possess some peculiarities and the major feature is the difference in the metastatic potential between PLNETs and NETs. PLNETs have less metastatic potential compared with NETs, which is the main factor based on which differential diagnosis between the two groups is achieved. There exists few reports disease's long-term outcome, especially about the recurrences management. We report the case of a 52-year-old woman admitted to hospital for jaundice and presence of liver mass. She underwent extended right hepatectomy and subsequently, PLNET was revealed. After 9 years, a new mass was discovered in the remnant liver, far from the resection line, and was surgically removed. Histological examination confirmed a PLNET recurrence. The patient is alive and doing well after a year of surgery. We conducted a review of the literature on recurrent PLNETS. Five papers followed our inclusion criteria and included 10 patients. Clinical presentation was mostly nonspecific in included cases and no carcinoid syndrome was reported. Median overall survival and median disease-free survival periods were 22 and 5 months, respectively. The primary disease was treated with surgical resection in all the included cases and recurrent diseases were mostly treated with non-surgical techniques (mainly transarterial chemoembolization). In conclusion, more studies should be conducted in order to have significant data about this uncommon neoplasm. Finally, considering the lack of data on long-term outcome, a long and accurate follow-up should be considered.