Primary hepatic neuroendocrine tumor: Successful hepatectomy in two cases and review of the literature

Background/Aims: Primary hepatic neuroendocrine tumor represents an extreme ly rare clinical entity with only very few cases having been reported to da te. Methods: The case histories of 2 patients with presumably primary hepat ic neuroendocrine tumor were analyzed and a complete follow-up obtained. Th e literature was reviewed to provide comprehensive data collection. Results : Both patients underwent partial hepatic resection. Histomorphologic diagn osis revealed a neuroendocrine tumor in both cases. Extensive preoperative as well as intra- and postoperative search for the primary tumor did not id entify another site of neuroendocrine tumor tissue. Six and ten years after hepatic segmentectomy, the 2 patients are alive and show no clinical signs of malignancy. Their most recent thorough follow-up included computed tomo graphy and somatostatin receptor scintigraphy. Neither a nonhepatic primary neuroendocrine tumor site nor recurrent disease was found in the 2 patient s. The literature review resulted in a complete survey of all previously re ported cases of primary hepatic neuroendocrine tumors, Conclusion: We concl ude that the liver was the primary site of the neuroendocrine tumor in both patients. Radical surgery was successfully performed as the only treatment option with curative intention.