Highly Differentiated Neuroendocrine Tumor of the Appendix

Pediatric neuroendocrine tumors (NET) of the gastrointestinal tract are uncommon, with appendiceal NETs usually being found incidentally. Neuroendocrine tumors are a diverse group of neoplasms that share common features such as a similar histological appearance, special secretory granules, and the production of biogenic amines and polypeptide hormones. The term "neuro" refers to dense core granules similar to those found in serotonergic neurons, while "endocrine" refers to the synthesis and secretion of these monoamines. The neuroendocrine system includes endocrine glands like the pituitary, parathyroids, and neuroendocrine adrenals, as well as endocrine islet tissue in the pancreas and scattered cells in the exocrine parenchyma, known as the diffuse endocrine system. Limited research has been conducted in pediatric patients, and guidelines are primarily derived from adult data. Diagnostic tests specific to NETs are currently lacking. We report a case of a 16 years-old boy where incidentally a highly differentiated neuroendocrine tumor (NET, G1) was found during laparoscopic appendectomy.