2018
DOI: 10.21873/anticanres.12264
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Neuroendocrine Neoplasms of the Appendix: A Review of the Literature

Abstract: Appendiceal neuroendocrine neoplasms (ANENs) comprise rare tumors of the appendix, mainly affecting young populations and characterized by a rather favorable prognosis. The aim of this review was to summarize the current knowledge on these neoplasms, focusing on the management and follow-up of such patients, which still remain under debate. ANENs account for 0.16-2.3% of appendectomies and are usually diagnosed incidentally. The histopathological diagnosis includes the immunohistochemical profile of the tumor … Show more

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Cited by 57 publications
(66 citation statements)
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“…In these tumors, right hemicolectomy should be performed. [11] Because of the long-term risk of recurrence, right hemicolectomy should also be considered in carcinoids 1-2 cm in size, if the following factors are present: involvement of the mesoappendix, demonstrable angioinvasion, apparent high proliferative index and Ki67 level, tumor located at the base of the appendix with positive margins, and in younger patients with positive lymph nodes. If tumor size <1 cm, appendectomy is enough for a cure.…”
Section: Discussionmentioning
confidence: 99%
“…In these tumors, right hemicolectomy should be performed. [11] Because of the long-term risk of recurrence, right hemicolectomy should also be considered in carcinoids 1-2 cm in size, if the following factors are present: involvement of the mesoappendix, demonstrable angioinvasion, apparent high proliferative index and Ki67 level, tumor located at the base of the appendix with positive margins, and in younger patients with positive lymph nodes. If tumor size <1 cm, appendectomy is enough for a cure.…”
Section: Discussionmentioning
confidence: 99%
“…Among carcinomas of the gastrointestinal tract, appendiceal primaries are rare [43,44]. Moreover, appendiceal signet ring cell carcinoma is an extremely rare malignancy, that accounts for 4% of all appendiceal tumors [45].…”
Section: Discussionmentioning
confidence: 99%
“…In most patients with appendix neuroendocrine neoplasm with a tumor diameter <1 cm, isolated appendectomy is sufficient for treatment, as they rarely metastasize (Gu;Wang;Xu, 2015). Tumors smaller than or equal to 2 cm that infiltrate the submucosa, muscle, subserosa and mesoappendix (at least 3mm deep) are considered to be stage IIa by the TNM classification of ENETS -European Neuroendocrine Tumors Society (Moris et al, 2018) and should be treated with right hemicolectomy, not just appendectomy.…”
Section: Discussionmentioning
confidence: 99%
“…Regarding the follow-up of these patients, the literature shows that regular followup is required for tumors between 1 and 2 cm with high risk factors for lymphatic spread of the disease, as well as in cases of mesoappendix invasion of more than 3 mm, localization of the injury at the base of the appendix, vascular infiltration or intermediate differentiation (Moris et al, 2018).…”
Section: Discussionmentioning
confidence: 99%