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

BACKGROUND/AIMS: Primary hepatic neuroendocrine tumor represents an extremely rare clinical entity with only very few cases having been reported to date.

METHODS

The case histories of 2 patients with presumably primary hepatic neuroendocrine tumor were analyzed and a complete follow-up obtained. The literature was reviewed to provide comprehensive data collection.

RESULTS

Both patients underwent partial hepatic resection. Histomorphologic diagnosis revealed a neuroendocrine tumor in both cases. Extensive preoperative as well as intra- and postoperative search for the primary tumor did not identify 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 tomography and somatostatin receptor scintigraphy. Neither a nonhepatic primary neuroendocrine tumor site nor recurrent disease was found in the 2 patients. The literature review resulted in a complete survey of all previously reported cases of primary hepatic neuroendocrine tumors.

CONCLUSION

We conclude 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.