Endocrine Abstracts

Introduction: Primary hepatic neuroendocrine tumours (PHNTs) are a very rare medical entity. Usually, the liver represents the preferential site for metastasis. Large sized, singular nodules are the usual presentation of PHNTs. We report a patient with PHNTs at 4 years of tumour free follow-up.

Case presentation: A 45-year-old Caucasian female who presented with nausea, vomiting, diarrhoea, accompanied by diffuse abdominal pain, was found to have on contrast enhanced computer tomography an encapsulated liver mass measuring 7/8.5/9 cm, which contained multiple daughter cysts separated by a matrix. In this context, the initial diagnosis was of an Echinococcus cyst. The patient underwent an uneventful left atypical hepatic resection. The resected specimen displayed a centrally brown–greyish solid cystic tumour measuring 8 cm, outlining a heavily vascularized cell proliferation with trabecular and band disposition and a homogenous growth pattern. The result of the immunohistochemical stain was diffusely positive in the tumour cells for chromogranin, synaptophysin and neuron specific enolase with 2–4% nuclear reactivity for Ki-67 index. Postoperatory, the specific neuroendocrine markers (serum Chromogranin A and 24-h-urinary 5-hydroxyindolacetic acid) were within the normal range. The patient underwent In¹¹¹ DTPA-octreotid scan, that showed no regions that were somatostatin receptor positive, and the PET scan with 5HTP could not find any suspicious lesions. The patient is now at 4 years of follow-up with no local recurrence or distant metastasis.

Conclusions: We report a case of primary PHNT in an adult female presenting with abdominal pain and diarrhoea, at 4 years of follow up with no signs of recurrence. We think that PHNT is an over-diagnosed medical entity, as most cases in English literature are incompletely documented, which makes it hard to establish the exact percentage of this type of tumour. In order to exclude an occult primary neuroendocrine tumour, thorough long-term investigations are required.