Carcinoid Tumours of the Appendix in Children - Epidemiology, Clinical Aspects and Procedures

Dear Sir,

We studied the interesting summary “Carcinoid tumours of the appendix in children - epidemiology, clinical aspects and procedure”, written by T. Doede, H. D. Foss and J. Waldschmidt ([3]). We can supplement this by our own experience with a 12-year-old girl who had appendectomy for perforated appendicitis. Histological evaluation showed a carcinoid of less than two centimeters in diameter, located in the middle of the appendix and infiltrating the mesoappendix. The girl did not show any symptoms of carcinoid syndrom, and the levels of serum serotonin and chromogranin A as well as 5-HIAA, VMA and HVA in 24 h urine collection were within normal values.

Textbooks of pediatric oncology mention, that carcinoids “rarely require more than simple surgical removal regardless of their size or degree of invasion” ([5]). Surgical literature points out that “right hemicolectomy is occasionally necessary” ([6]). Bethel et al ([1]) described a case comparable to our girl with meso-appendiceal extension and decided that right hemicolectomy was required.

Doede et al ([3]) discussed infiltration of the mesoappendix as “usually not an indication for further intervention”, even though they did not find a single case of infiltration within their collective. We decided on secondary resection and carried out right hemicolectomy. Histological evaluation showed no further tumorous tissue or lymph node metastasis. Serum or urine controls, ultrasonography as well as clinical evaluation remained without pathological findings for the first postoperative year. Data about infiltrative carcinoids in childhood are extremely rare. Therefore, nobody knows what happens in cases of infiltrative carcinoids if treatment in childhood was limited to appendectomy alone. For infiltrative carcinoids in adults, hemicolectomy is indicated if lymphatics, serosa or mesoappendix are invaded ([2]). I cannot see any reason why we should change to this procedure. Usually, appendectomy is enough, but usually carcinoids in childhood are not infiltrative. Unusual infiltrative cases need unusual treatment - particularly perhaps in a young patient who has a longer life and may therefore suffer from a slowly growing local recurrence or metastasis, as described, nearly 30 years after initial surgery ([4]).

References

• 1 Bethel C AI, Bhattacharyya N, Hutchinson C, Ruymann F, Cooney D R. Alimentary tract malignancies in children.  J Pediatr Surg. 1997;  32 1004-1009
• 2 Deans G T, Spence R A. Neoplastic lesions of the appendix.  Br J Surg. 1995;  82 299-306
• 3 Doede T, Foss H D, Waldschmidt J. Carcinoid tumors of the appendix in children - epidemiology, clinical aspects and procedure.  Eur J Pediatr Surg. 2000;  10 372-377
• 4 Moertel C L, Weiland L H, Telander R L. Carcinoid tumor of the appendix in the first two decades of life.  J Pediatr Surg. 1990;  25 1073-1075
• 5 Morgan S K. Carcinomas and other uncommon tumors. Pochedly C Neoplastic Disease in Childhood. Chur; Harwood Academic Publishers 1994 2: 1031-1045
• 6 Parkes S E, Muir K R, al Sheyyab M, Cameron A H, Pincott J R, Raafar F, Mann J R. Carcinoid tumours of the appendix in children 1957 - 1986: incidence, treatment and outcome.  Br J Surg. 1993;  80 502-504