Neuroendocrine Tumor of the Appendix in Children

Abstract

Neuroendocrine tumor (NET) of the appendix is the most common gastrointestinal epithelial tumor in children. The utility of serum markers or the indication for hemicolectomy has not been established in children. In 45 children diagnosed with appendiceal NET, 89% NETs were incidentally found following appendectomy performed for suspected acute appendicitis. The median age was 12 years, and 56% patients were female. Postoperative somatostatin scan (n=5), serum chromogranin A (n=4), and urine 5-HIAA (n=9) were all within normal limits. Pathology slides of 35 patients showed mesoappendiceal invasion in 29% patients, and vascular invasion in 6% patients. Seven patients (16%) underwent hemicolectomy for invasion of mesoappendix (n=5), tumor near the resection margin (n=1), and tumor size 1.5 cm with vascular invasion (n=1). Only 2 hemicolectomy specimens showed disease: one in the appendiceal stump and the other as a micrometastasis in a mesenteric lymph node. There were no recurrences and all patients were alive and without evidence of disease at last follow-up. Pediatric appendiceal NET tends to have a benign clinical course with excellent prognosis. In the absence of carcinoid syndrome, postoperative scans and serum biomarkers do not seem to be useful. With completely resected tumors, the indication for hemicolectomy is unclear.

Systematic review and meta‐analysis of appendiceal carcinoid tumors in children

“…No survival advantage was therefore conferred by another procedure. This confirms the observations of several authors …”

“…reported an incidence of 1.14 per million per year in children . Most are incidental findings following removal of an appendix specimen for acute appendicitis . The dilemma has been what care to provide following incidental histological identification of an appendiceal carcinoid.…”

“…12 Most are incidental findings following removal of an appendix specimen for acute appendicitis. 9,13 The dilemma has been what care to provide following It also recommends RHC in patients with nodal involvement.…”

“…This confirms the observations of several authors. 2,6,8,9,13,16,17 Lambert et al in the French study of 2016, identified 29 pediatric patients with criteria for a secondary procedure. 16 Ten of these had an RHC.…”

Nowotwory neuroendokrynne jelita cienkiego i wyrostka robaczkowego — zasady postępowania (rekomendowane przez Polską Sieć Guzów Neuroendokrynnych)

“…In post-mortem examinations the reported prevalence increases to 1.22% [3,12]. Tumours originating from the lower part of the jejunum and the ileum constitute 23-38% of all gastro-entero-pancreatic endocrine neoplasms, and they are more common than endocrine neoplasms of the appendix, also described in paediatric patients [3,13,14]. Hormonally active neuroendocrine neoplasms of the small intestine are often multifocal, and in 15% of cases are associated with other neoplasms such as gastrointestinal adenocarcinoma or breast cancer [3].…”