Appendiceal Carcinoids in Children–Prevalence, Treatment and Outcome in a Large Nationwide Pediatric Cohort (original) (raw)
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Pediatric Surgery International, 2019
Aim of the study Appendiceal carcinoid (neuroendocrine tumor or NET) is a rare neuroendocrine neoplasm often found incidentally following appendectomy for appendicitis. Surgery for appendicitis is currently under scrutiny and children are increasingly managed conservatively with antibiotics alone. Herein, we aimed to review our experience with the management of appendiceal carcinoids at our institution. Methods Following ethical approval, we reviewed the charts of all patients who underwent appendectomy for appendicitis at our institution between 2000 and 2018. The pathology registry was consulted to identify children diagnosed with appendiceal carcinoid. Outcome measures included incidence, demographics, and management. Main results During the study period, 32 children (23 female) had an appendiceal carcinoid confirmed at pathology. Of these, 13 were initially treated with appendectomy (total of 5,059 appendectomies: 0.3% incidence). The other 19 had an appendectomy elsewhere by an adult general surgeon and were referred to our institution for further management. Overall, the mean age at diagnosis was 13 ± 2.7 years and all patient had a preoperative diagnosis of appendicitis, none of suspected carcinoid. Most children (75%) had acute non-perforated appendicitis. The overall mean size of the lesion was 1 ± 0.9 cm, with a > 2 cm lesion in 3 patients. Following diagnosis, 12 children (38%) underwent an ileocolic resection, due to carcinoid size, invasiveness, and margin clearance. Conclusions In our cohort, the incidence of appendiceal carcinoid among children with appendicitis is very low. Most carcinoids are small, located at the tip, associated with non-perforated appendicitis, and present in girls. Most were treated with appendectomy alone, with more extensive surgery performed in one third of children.
Systematic review and meta-analysis of appendiceal carcinoid tumors in children
Pediatric blood & cancer, 2018
The study's aim was to review the literature regarding past and current practices in managing incidental appendiceal carcinoid tumors and need for more procedures. A search of MEDLINE, Embase, CINAHL, and Cochrane databases of systematic reviews was undertaken of the English language literature. The mesh terms used were "carcinoid" or "neuroendocrine," "tumour" or "tumor," "appendix," "appendicectomy," or "appendectomy," and "child," "pediatric," or "paediatric." Of the 369 articles found, 37 met the inclusion criteria. Our hospital records and pathology database identified 11 patients with confirmed histological diagnosis of appendicular carcinoids from January 1996 to December 2016. Those cases were also included in this study. A total of 958 cases were identified from the literature and our own experience. There were 566 females and 343 males giving us a ratio of 1.65:1. The fr...
[Appendiceal carcinoid tumors. Evaluation of long-term outcomes in a tertiary level]
Cirugía y cirujanos
Appendiceal tumors are found in about 1% of appendectomies performed and 0.5% of intestinal neoplasias. Appendiceal carcinoids are the predominant histology in this group and are usually casual after appendectomy for other reasons. The prognosis is excellent and survival is 95% at 5 years after surgery. Retrospective analysis of all patients with appendiceal carcinoid surgery in our hospital for 20 years (1990-2010) and survival at 5 years. We also discuss the need for additional treatment and testing for follow-up visits. 42 patients underwent surgery for appendiceal carcinoid tumor. 38 of them were operated on urgently, mostly for suspected acute appendicitis, without having reached the carcinoid tumor diagnosed preoperatively in any of them. The predominant symptomatology at admission was abdominal pain. Surgical treatment was appendectomy in 34 patients (12 laparoscopic), 7 patients required colon resections over intraoperatively by colonic involvement; only one patient required...
Surgical Management of Appendicular Carcinoids
World Journal of Pathology, 2013
The carcinoid of the appendix, or well differentiated neuroendocrine tumor, is often asymptomatic and the diagnosis is usually 'accidental' after a surgical procedure. Multidisciplinary approach is essential for accurate management included analysis primary tumor size, staging, treatment plan and follows up. Case report: The authors reported 2 cases of appendicular carcinoid on 320 consecutive appendicectomy in the last two years and described clinical features and therapeutic approach for this art of tumors. Appendicectomy in little tumors (< 1 cm) is a gold standard for the treatment and the radical resection is often healing, if the tumor size is mean (between 1 and 2 cm) the resection (appendicectomy vs hemicolectomy) is subject to the valuation of individual case, while the right hemicolectomy is considered according to tumor size (> 2 cm), incomplete tumor resection and/or histological features (medium and high grade, mixed histological). Conclusions: The size of the tumor is a standard that influences both surgical strategy, both prognosis.The carcinoid of the appendix presents favorable prognosis as regards carcinoid tumors of the gastro enteric tract.
Hidden Hazard in Appendix in Children: Carcinoid Tumors
Indian Journal of Child Health, 2019
This retrospective study enrolled the patients who were diagnosed with CT of the appendix as a result of an examination of the appendix specimen after the patients underwent an appendectomy. All the patients, who underwent appendectomy in the pediatric surgery clinic of our hospital between November 2015 and November 2018, were included in the study. Patients' demographic characteristics, clinical findings, pre-operative laboratory and imaging results (Fig. 1), location, diameter and size of the tumor, mesoappendix invasion status, mitotic index and Ki-67 elevation (Fig. 2), cromogranin marker (Fig. 3), hospital stay duration, surgical types and complications, and results of laboratory and imaging tests were evaluated retrospectively. For this retrospective study, the ethics committee decision no. 3021156 dated August 17, 2018 was obtained from the Ethical Committee Board of Derince Training and Research Hospital. RESULTS Of the 621 patients, who underwent an appendectomy in the pediatric surgery clinic during the study period, eight were diagnosed with carcinoid tumor of the appendix. The median age of the patients was 13 years (range, 11-16 years). Of the eight
Appendicular Neuroendocrine Tumours in Children: Unicentric Retrospective Study
Iranian journal of pediatric hematology and oncology, 2018
Background: Neuroendocrine Neoplasms (NEN) represent 60% of all appendicular tumours. This type of cancer is predominantly benign. In this study, appendicular NEN tumours in children were investigated. Materials and Methods: This retrospective study was conducted on 540 patients underwent emergency appendectomy for the treatment of clinically suspected appendicitis at the department of pediatric surgery in Hedi Chaker hospital in Sfax, Tunisia. This study was performed between January 2013 and December 2016. Data on appendicular NEN demographic, preoperative diagnoses, investigations, operative findings, pathological reports, and follow up were reviewed and analysed. Results: Out of 540 patients, there were 309 males (57.3%) and 231 females (42.7%). The Mean age of the patients was 9.23 ± 2.78 years. One hundred and thirty seven patients (25.4%) had laparoscopic appendectomy and 74.6% were operated by the traditional open approach. The diagnosis of appendicular NEN tumours was histo...
Appendiceal Neuroendocrine Tumours in Childhood
Journal of Pediatric Gastroenterology & Nutrition, 2014
Background: Neuroendocrine tumours (NETs) of the appendix are slowgrowing tumours and, although rare, they are the most common gastrointestinal epithelial tumours in childhood and adolescence. The treatment and the follow-up screenings have not been standardised. In addition to this, although tumour size is considered the main prognostic variable to define the aggressiveness of approach, a precise cutoff needs to be established. Methods: A total of 113 patients younger than 18 years with a diagnosis of appendiceal NETs were registered as of January 1, 2000, until May 30, 2013, within the Rare Tumors in Pediatric Age (TREP) project, an Italian multiinstitutional network dedicated to rare tumours in children and adolescents. The recommendations of the Rare Tumors in Pediatric Age study included imaging and laboratory investigations. The treatment after appendectomy was decided on the basis of histology, tumour size, and imaging; primary reexcision (PRE) was not recommended in completely excised tumours, regardless of tumour size and invasiveness. Results: A total of 113 of 113 patients had a diagnosis of well-differentiated NETs; in 108 of 113 the tumour was smaller than 2 cm and in 5, larger than 2 cm. Excision margins were free in 111 of 113 patients. In 3 of 113 a PRE was performed, and in 1 residual tumour was detected. All 113 of 113 patients are alive in complete remission (median follow-up of 41 months). Conclusions: Reported data and our experience showed that no relapse or death occurred in children and adolescents affected by appendiceal NETs. Appendectomy alone should be considered curative for most patients, and a more aggressive surgical approach is warranted in the cases with incompletely excised tumours.
Carcinoid Tumor of the appendix: an incidental finding in child
With reference to carcinoid tumor of the appendix in a child, we want to share our experience and enlighten the occurrence of this carcinoid tumor in a child as an incidental finding in routine appendectomy specimen. Carcinoid tumors are rare neuroendocrine tumours with most common frequency in the appendix (1). In children, they are less common with a reported incidence of 0.08% (2). To the best of our knowledge, only handful of cases of carcinoid tumor in children has been reported in Indian sub-continent. Although rare, carcinoid tumor is considered the most common type of appendiceal primary malignant lesion and usually diagnosed incidentally in appendectomy specimen and is found in 0.3-0.9% of patients undergoing appendectomy (3). Despite the fact that it is among the most frequently occurring carcinoids in surgical practice with rare pre-operative diagnosis, most of surgeons may encounter only one or two such lesions during their career (4). Hence it is important to know correct management of such a rare tumor (5). Personally it was the first time; we encountered incidental histopathological diagnosis of carcinoid in appendix in 12 years of Pathology practice.
Carcinoid Tumors of the Appendix in Children: Experience From a Tertiary Center in Northern Greece
Journal of Pediatric Gastroenterology & Nutrition, 2010
Background and Objective: Carcinoid tumors of the appendix are rare in childhood and usually have a benign clinical course. Their incidence in appendectomy specimens ranges from 0.1% to 0.9%. The aim of the study was to report the frequency, clinical presentation, tumor characteristics, and outcome of children with appendiceal carcinoid treated in a pediatric oncology department. Patients and Methods: All of the cases referred during a 19-year period (1990-2008) were studied retrospectively. Demographics, clinical presentation, tumor characteristics, and follow-up results were recorded. Results: Among 839 admissions, 19 patients (9 boys) with appendiceal carcinoid were identified during the study period. Their median age was 10.5 years (range 4.5-13.2 years). In all of the cases, diagnosis was established after appendectomy. The mean tumor diameter was 4.55 (AE3.45) mm (range 1-15 mm). Concomitant appendicitis was diagnosed in 12 patients. In 18 children tumor size was 10 mm and did not infiltrate surrounding tissues. In 1 patient the size was 15 mm and a microscopic rupture of the appendix with infiltration of the surrounding fat was present. All of the tumors were located at the tip of the appendix and were of the classic histological type. Staging and follow-up consisted of abdominal ultrasound, chest and abdominal computed tomography scans, 99 Tc bone scan, urine 5-hydroxylindoloacetic acid levels, and 111 In octreotide scan. No patient had metastases requiring further therapeutic interventions. No relapses or other neoplasms occurred during a median follow-up period of 45 months (range 6-118 months). Conclusions: Carcinoid tumors of the appendix in children are rare. Longterm follow-up revealed that a good prognosis is possible provided they are diagnosed and surgically removed at an early stage.