Primary hepatic neuroendocrine tumours-Case series of a rare malignancy

Primary hepatic neuroendocrine tumours (PHNET) were first described by Edmondson et al. in 1958 and are rare, accounting for only 0.3% of all neuroendocrine tumours. Only several hundred cases have been reported.

We present two cases. The first is a 65-year-old asymptomatic male referred with a liver lesion on ultrasound performed to investigate a mildly elevated Alanine Aminotransferase (ALT). Hepatitis serology and tumour markers were normal. He had an unremarkable colonoscopy and gastroscopy. CT and MRI revealed a single liver lesion adjacent to the gallbladder suspicious for malignancy. He underwent a segment IVb/V liver resection. Histology was consistent with a 65 mm grade 2 PHNET. Subsequent Dotatate PET/CT scans have been normal at 5 years. The second is an asymptomatic 73-year-old male referred with fluctuating hepatic enzymes and a history of alcohol overuse. Imaging revealed a suspicious lesion in segment III of the liver. He underwent a left lateral liver resection. Histology revealed an 18 mm grade 1 PHNET. A subsequent Dotatate PET/CT was normal with no new disease at six months.

PHNET, albeit rare are in the differential diagnosis for primary hepatic malignancies. Tumour markers are usually normal and radiological imaging can mimic other hypervascular hepatic tumours. Surgery is the only curative treatment for localised disease to date.

PHNET needs to be considered in asymptomatic patients with hypervascular hepatic lesions. More research is required before other adjunct treatment options can be suggested.