A Single Experience of a Well-Differentiated Pediatric Neuroendocrine Tumor in the Appendix
Stefan Bittmann *
Department of Pediatrics, Hindenburgring 4, D-48599 Gronau, Germany and Shangluo Vocational and Technical College, Shangluo, 726000, Shaanxi, China.
*Author to whom correspondence should be addressed.
Abstract
Pediatric neuroendocrine tumours (NETs) of the gastrointestinal tract are uncommon, with appendiceal NETs usually being found incidentally. They are most often found in the lungs and the gastrointestinal tract. Neuroendocrine tumours 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. Limited research has been conducted in pediatric patients, and guidelines are primarily derived from adult data. Diagnostic tests specific to NETs are currently lacking. Treatment options include Somatostatin analogues, surgical interventions like hemicolectomy and chemotherapy. This study reports a case of a 16-year-old boy in which, incidentally, a highly differentiated neuroendocrine tumour (NET, G1) was found during laparoscopic appendectomy. This report describes a 16-year-old boy with an incidental, highly differentiated appendiceal NET (G1) measuring 0.25 cm, discovered during a laparoscopic appendectomy for florid ulcerative-phlegmonous appendicitis. The patient presented with atypical signs of appendicitis, including mild pain in the McBurney region. Laparoscopic appendectomy was performed to address the symptomatic presentation. Histopathological and macroscopic examination confirmed acute appendicitis and revealed an incidental highly differentiated NET (G1) of the appendix. Laboratory parameters at admission showed leukocytosis of 17.4 Th/cu., and the CRP level was 119 mg/l. Tumour-free surgical margins and subserosa/mesoappendix were confirmed, with TNM classification reported as pT1, pNx, pMx, G1, local R0. Preoperative intravenous antibiotic therapy with Unacid was administered. Pediatric appendiceal NETs are generally discovered incidentally, are localised, and exhibit low-grade histology. Tumours under 1 cm rarely metastasize, while features such as serosal or perineural invasion and G2 status may increase metastatic risk. These findings support careful surgical management and follow-up in pediatric patients.
Keywords: Neuroendocrine tumours, neuroendocrine carcinomas, pediatric, appendectomy, neoplasma