Neuroendocrine tumors of the appendix in children and adolescents

Abstract

ROC analysis and subsequent calculations identified a tumor size of >15 mm as the optimal cut-off point for the prediction of metastatic spread into the lymph system, with a sensitivity of 77.8% and a specificity of 66.7%. Therefore, secondary right hemicolectomy in completely removed appendical NET is recommended only in tumors >15 mm in size. For incompletely removed tumors ≤15 mm a local follow-up resection with lymph node sampling is recommended.

ENETS Consensus Guidelines for Neuroendocrine Neoplasms of the Appendix (Excluding Goblet Cell Carcinomas)

“…Most appendiceal NEN in adults as well as in children are incidental findings in postappendicectomy specimen and therefore no characteristic tumor-specific symptomatology has been established [5,17]. However, symptoms that lead to appendicectomy such as right lower abdominal pain are indirectly associated with appendiceal NEN, although due to their most frequent localization at the tip of the appendix (approx.…”

“…Neuroendocrine neoplasms (NEN) of the appendix are a relatively frequent subgroup of NEN with an approximate incidence rate of 0.15-0.6/100,000/year and with a slight female preponderance in Western series [1,2,3,4,5,6,7,8,9,10,11,12]. Although NEN are frequently reported [13], most cases are of an early stage [12,13,14].…”

Potential Pediatric Applications of PET/MR

“…They may be located in the appendix and are mostly detected incidentally through a histopathologic examination (53). Depending on the degree of tumor differentiation, PET/CT with various radiopharmaceuticals, such as 18 F-FDG, 68 Ga-octreotide, 11 Chydroxytryptophan, and 18 F-DOPA, has been described as potentially improving the assessment of tumor localization and the response to therapy.…”

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].…”

“…The indications for right-sided hemicolectomy are the following tumour features: 1. Diameter of more than 2 cm, aNEN G1/G2 (NET classified as at least T3 (ENETS) or T2 (UICC/AJCC) [5,13,[78][79][80][81][82]; 2. Location at the base of the appendix (although there is no evidence for a poorer prognosis, and in such cases a higher incidence of complications should always be considered, compared to simple appendectomy) [79,[83][84][85][86]; 3.…”