Primary hepatic neuroendocrine neoplasms of children, a systematic review.

Abstract

The term "carcinoid tumour" is no longer used and has given up its place to neuroendocrine neoplasms (NENs). Primary hepatic neuroendocrine neoplasms (PHNENs) are as rare as compromising 0.4% of all neuroendocrine neoplasms (NENs). Searching the central medical databases of PubMed/Medline, Web of Science, Scopus, Google Scholar, and the references of the articles, we have collected 10 cases of pediatric PHNENs of children. The inclusion criteria were full-text available literature in English and those under and equal to 19 years old. Diseases found during autopsy, carcinoma, and isolated gall bladder involvement also made the exclusion criteria. The mean age of the patients was 13.86 years, ranging from 8 to 19 years. Six female patients (55%) were in front of 5 males (45%). The right lobe was the most frequent site of involvement and surgery in four cases. Abdominal pain comprised the main symptom, and CT scans of most of them were common which helped in diagnosis. While a hepatic tumour is considered NEN, a detailed systemic evaluation of a primary tumour is mandatory to exclude metastatic HNEN. Secondary hepatic NENs are more common than primary ones. Surgical resection has had the most long-term success.