Surgical management and surveillance of pediatric appendiceal carcinoid tumor

Abstract

Purpose: Appendiceal carcinoid tumors are rare neuroendocrine neoplasms. The aim of this study was to determine if postoperative oncologic follow-up was necessary for this tumor.

Methods: A retrospective review was performed of patients with appendiceal carcinoid 2000-2015.

Results: 8382 patients underwent appendectomy 2000-2015. 30 (0.3%) had appendiceal carcinoid. 70% were female (n=21) with an average age of 13.5±2.8 years (range 8-18). Most presented with abdominal pain (n=29, 97%). 20% (n=6) had appendiceal perforation. Mean tumor size was 5.4±4mm (range microscopic - 15mm) with most at the appendiceal tip (n=18, 60%). No node infiltration was found, although 10% (n=3) had perineural and 3% (n=1) had lymphovascular invasion. Five were transmural (17%). Most patients were referred to oncology (n=19, 63%) for staging and surveillance including ultrasonography (n=11, 65%), MRI (n=7, 41%), and CT (n=6, 35%). The majority (79%, n=15) underwent serial 5-HIAA testing. All surveillance was found to be normal, and no patients required further treatment. Mean follow-up was 36±34 months, with 58% (n=11) continuing surveillance. Medical charges ranged $8500-$44,000. No recurrences have been identified.

Conclusion: Appendectomy is an adequate treatment for pediatric appendiceal carcinoid <16 mm despite presence of histological risk factors. More aggressive surgery and extensive oncologic follow up are of limited value.

Level of evidence: III.

Type of study: Retrospective comparative study.

Keywords: Appendiceal carcinoid; Carcinoid; Neuroendocrine tumor; Oncologic follow-up.