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

Njere, Ike; Smith, Lisa L.; Thurairasa, D; Malik, Rubina; Jeffrey, I; Okoye, Bruce; Sinha, Chandni

doi:10.1002/pbc.27069

Cited by 34 publications

(52 citation statements)

References 38 publications

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“…Another issue is the high proportion of patients lost to FU or who opted out, as it happened in six patients of our series (23% of the total population). This was often reported in the past, 14,16,27 and in our experience it seems a consequence of parents’ decision to look for a second or third opinion in other centers outside the pediatric network.…”

Section: Discussionsupporting

confidence: 50%

“…Bronchi and pancreas seem to represent the most frequent sites in extra‐appendicular NET, 3,14 but it is undoubted that the true incidence remains currently unveiled. A report from the Surveillance, Epidemiology, and End Results (SEER) registry demonstrated that in children the bronchial NETs occurred more frequently than the appendicular tumors, 3 but the number of appendicular NET might be underestimated due to their benign course 1,16–18 . Moreover, series from single institution, though covering a long frame of time, seem to alternately over‐ or underestimate the site of occurrence 15,19,20 .…”

Section: Discussionmentioning

confidence: 99%

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Extra‐appendicular neuroendocrine tumors: A report from the TREP project (2000‐2020)

Virgone

Ferrari

Chiaravalli

et al. 2021

Pediatric Blood & Cancer

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Background Extra‐appendicular neuroendocrine tumors (NETs) are very rare tumors. While diagnostic and therapeutic guidelines are well established for adults, data on children and adolescents are lacking. Patients and Methods Patients with a diagnosis of extra‐appendicular NET registered on the Tumori Rari in Età Pediatrica ‐ Rare Tumors in Pediatric Age (TREP) from 2000 to 2020 were analyzed. Clinical characteristics including patients’ presentation, tumor features, treatment, and outcome were reviewed. Results Twenty‐seven patients with extra‐appendicular NET registered on TREP with a median age of 173 months. The primary site was the pancreas (12) or bronchi (10) in the majority of cases. Other primary sites included the thymus, Meckel's diverticulum, and liver. Thirteen (48%) of tumors extended beyond the organ of origin: four invaded neighboring organs and/or regional nodes and nine involved distant metastases. The 3‐year event‐free survival (EFS) for those with localized disease was superior to those with metastatic disease (66.6% 95% CI 5‐95% vs 33% 95% CI 5‐68%, respectively; P = .005). A complete resection was feasible in 17 patients. The 3‐year EFS in these patients was superior to those with no or incomplete resection (R0 vs R1/R2, respectively; P = .007). Overall, 16 children had no evidence of disease at follow‐up, and one is alive with disease; five died, and five were lost to follow‐up. Conclusions Data from our experience demonstrated a wide heterogeneity of presentation and outcome of these tumors. Localized disease and complete surgical resection were the main prognostic factors of good outcome. Other therapies may have a role in prolonging survival in metastatic disease.

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Section: Discussionsupporting

confidence: 50%

Section: Discussionmentioning

confidence: 99%

Extra‐appendicular neuroendocrine tumors: A report from the TREP project (2000‐2020)

Virgone

Ferrari

Chiaravalli

et al. 2021

Pediatric Blood & Cancer

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“…For tumors of this size, previous studies have indicated that appendectomy is sufficient. The guidelines set by The North American Neuroendocrine Society (NANETS) reveal that right hemicolectomy is recommended for tumors originating at the base of the appendix, tumors >2 cm in size, if there is evidence of lymphovascular or meso-appendiceal invasion, in patients with lymph node metastases, or for intermediate or high-grade tumors [22,23]. In regards to those tumors between 1 cm and 2 cm, there is no clear consensus.…”

Section: Discussionmentioning

confidence: 99%

Incidental neuro-endocrine tumor of the appendix: Case report and literature review

Elkbuli

Sanchez

McKenney

et al. 2019

Annals of Medicine and Surgery

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Introduction Neuroendocrine tumors (NETs) are neoplasms that arise from neuroendocrine cells that have properties of both neuronal and endocrine cells. NETs are most common in the small intestine, rectum, and the appendix and frequently termed carcinoid. Presentation of case A 30-year-old male presented with abdominal pain and tenderness in the RLQ. Computerized tomography revealed findings consistent with acute appendicitis. The patient underwent an uneventful laparoscopic appendectomy for an acutely inflamed appendicitis. Histopathological examination, showed a 0.5 cm well-differentiated neuroendocrine tumor of the distal appendix, with clear margins. The mitotic rate was <2 mitoses/10 high power field. Following guidelines, no further procedures were performed and follow-up one week later was uneventful. Discussion Appendectomy for the treatment of appendiceal NETs smaller than 1 cm has been recommended as the treatment of these neoplasms by the guidelines set by The North American Neuroendocrine Society (NANETS). NANETS recommends right hemicolectomy for tumors originating at the base of the appendix, for tumors >2 cm in size, if there is evidence of lymphovascular or meso-appendiceal invasion, with mesenteric lymph node metastases, or for intermediate or high-grade tumors. Conclusion We present the case of a 30-year old male that presented with an appendiceal, well-differentiated NET that manifested as appendicitis and laparoscopic appendectomy was performed. The appendix was resected with clear margins. Given appropriate markers appendectomy can be curative.

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“…A recent systematic review of pediatric carcinoid tumors found that in 91% cases, carcinoid was only identified following histology, as we report in our case. 5 The majority of studies in this review report cases of appendectomies undertaken for symptomatology, acute appendicitis or chronic abdominal pain with a carcinoid incidence of 0.3% of appendectomies. 5 Incidental appendectomies, performed in an adult population of patients with normal appendices, demonstrated a much higher carcinoid incidence of 1.6%.…”

Section: Discussionmentioning

confidence: 99%

The Excised Appendix Tip—To Send or not to Send, That is the Question

Tullie

Vadgama

Anbarasan

et al. 2018

European J Pediatr Surg Rep

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A 9-year-old boy, with previous anorectal malformation and neuropathic bladder and bowel, underwent ileocystoplasty, Monti–Mitrofanoff and appendix antegrade colonic enema procedure. The tip of the macroscopically normal appendix was sent for routine histopathology. Microscopy demonstrated a 5-mm well-differentiated neuroendocrine tumor extending into muscularis propria. K i -67 index was <2%. Due to margin involvement, the appendix conduit and surrounding skin were re-excised and a tube cecostomy was created through a separate incision. Microscopy revealed no residual neuroendocrine tumor, and no further treatment was required.

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

Abstract: Appendicectomy alone is an adequate treatment for an appendicular carcinoid in children irrespective of size, position, lymph node, or mesenteric involvement. Post-appendicectomy investigations were found to be not helpful in this study.

Cited by 34 publications

References 38 publications

Extra‐appendicular neuroendocrine tumors: A report from the TREP project (2000‐2020)

Extra‐appendicular neuroendocrine tumors: A report from the TREP project (2000‐2020)

Incidental neuro-endocrine tumor of the appendix: Case report and literature review

The Excised Appendix Tip—To Send or not to Send, That is the Question

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