Well-Differentiated Neuroendocrine Tumors of the Appendix in Children and Adolescents: A Clinicopathologic Study

Pediatr Dev Pathol. 2023 May-Jun;26(3):250-258. doi: 10.1177/10935266221146001. Epub 2023 Feb 21.


Background and aims: Pediatric neuroendocrine tumors (NET) of the GI tract are rare and appendiceal NET are typically incidental. Few studies have been done in the pediatric population and practice guidelines are mainly based on adult data. There are currently no diagnostic studies specific for NET. Our study aimed to identify clinical, radiological, and pathological findings in pediatric appendiceal NET, test criteria for follow up surgical treatment, review potential prognostic pathological findings, and possible pre-operative diagnostic radiological studies.

Materials and methods: A retrospective data search was conducted for well-differentiated NET of the appendix in patients ≤21 years between 1/1/2003 and 7/1/2022. Available clinical, radiologic, pathological, and follow-up information was recorded.

Results: Thirty-seven patients with appendiceal NET were identified. No masses were reported in the patients who underwent presurgical imaging. Appendectomy samples showed NET (0.2->4 cm), most located in the tip. Most cases were WHO G1 (34/37), with negative margins (n = 25). Sixteen cases extended to the subserosa/mesoappendix (pT3). Lymphovascular (6), perineural (2), and both lymphovascular and perineural invasion were also noted (2). The specified tumor stages were pT1 (10/37), pT3 (16/37), and pT4 (4/37). Patients who underwent laboratory testing for chromogranin A (20) and urine 5HIAA (11) had normal limits. Subsequent surgical resection was recommended in 13 cases and performed in 11. To date, all patients have no recurrent or additional metastatic disease.

Conclusions: Our study showed that all pediatric well-differentiated appendiceal NET were incidentally found as part of acute appendicitis management. Most NET were localized with low-grade histology. Our small cohort support the previously suggested management guidelines with follow up resection in certain cases. Our radiologic review didn't identify a best modality for NET. Comparing cases with and without metastatic disease, no tumors under 1 cm had metastasis, but serosal and perineural invasion along with G2 status were associated with metastasis in our limited study.

Keywords: appendix; neuroendocrine tumor; pediatric; surgical follow up.

MeSH terms

  • Adolescent
  • Adult
  • Appendiceal Neoplasms* / diagnosis
  • Appendiceal Neoplasms* / epidemiology
  • Appendiceal Neoplasms* / surgery
  • Appendix* / pathology
  • Child
  • Humans
  • Neuroendocrine Tumors* / diagnosis
  • Neuroendocrine Tumors* / pathology
  • Neuroendocrine Tumors* / surgery
  • Retrospective Studies

Supplementary concepts

  • Gastro-enteropancreatic neuroendocrine tumor