S. C. Fallon1, M. J. Hicks2, J. L. Beer1, S. A. Vasudevan1, J. G. Nuchtern1, D. L. Cass1 1Texas Children’s Hospital,Division Of Pediatric Surgery, Michael E. DeBakey Department Of Surgery, Baylor College Of Medicine,Houston, TX, USA 2Texas Children’s Hospital,Department Of Pathology,Houston, TX, USA
Introduction:
Appendiceal carcinoid tumors are rare lesions detected incidentally following appendectomy in children. There are limited data about the natural history of these tumors, and guidelines regarding family counseling and need for additional surgery or follow-up imaging are not established in the pediatric age group. The purpose of this study was to review our institutional experience with appendiceal carcinoid tumors to provide data that might improve management.
Methods:
After IRB approval, the charts of all patients treated at our institution for an appendiceal carcinoid tumor between 2002 and 2014 were reviewed. Data collected included patient demographics, pathologic details, postoperative management, and follow-up information. Descriptive analyses were performed.
Results:
Twenty-eight patients were identified, which represents an incidence of 0.2% of children undergoing appendectomy during that time interval. The mean age at surgery was 13.8+2.1 years; 54% were females. Two patients had symptoms suspicious for carcinoid syndrome at presentation, though none had evidence of metastatic disease. The mean tumor size was 0.8 (+0.4) cm. Five patients (18%) underwent subsequent ileocecectomy or right hemicolectomy due to pathologic findings of invasion of the mesoappendix (n=4) or lymphovascular invasion and subserosal extension (n=1). One child was found to have positive lymph nodes on pathologic examination. No recurrences have been detected at mean follow-up of 1.2 yrs.
Conclusion:
Appendiceal carcinoid tumors are discovered incidentally in about 0.2% of children undergoing appendectomy. Based on findings from the largest series to date, we can conclude that these tumors are generally small, and demonstrate lymphovascular invasion or mesenteric extension in fewer than 20% of cases. Prospective, multi-center studies are necessary to better define the indication for ileocecectomy and follow-up imaging protocols.