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Pediatric

Appendix carcinoid

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Kinder- und JugendmedizinAppendix carcinoid

The incidental finding every 300th Appendectomy

M. Wilhelm (1), U. Bierbach (1), R. Kluge (2), W. Kiess (3), H. Christiansen (1) (1) Department of Children Hematology, Oncology and Hämostaseologie, University Clinic and Policlinic for Children and Young People Leipzig (2) Department of Nuclear Medicine of Leipzig, (3) University Clinic and Policlinic for Children and Young People Leipzig

Abstract

The appendix carcinoid (AC) is the most common neuroendocrine tumors and is found as an incidental finding in 0.3% of all appendectomies. The prognosis is very good, the 5-year survival rate is 99%. The AC may occur in the context of MEN 1 (Wermer Syndrome). At a size of the AC less than 2 cm very rarely occur metastases. The therapeutic approach is usually curative appendectomy for a tumor in the apex and a tumor size smaller than 2 cm. When a tumor larger than 2 cm or a tumor of the appendix base is a hemicolectomy with lymph node resection is required. The best diagnostic and therapeutic markers in serum is chromogranin A (CgA) and the 24-hour urine 5-HIAA (5-HIAA). Patients should be interdisciplinary, multicenter study GPOH MET 97 (GPOH: Society for Pediatric Oncology and Hematology, MET: be included malignant endocrine tumors in childhood and adolescence) and provided with support in accordance with current guidelines. This is particularly the DOTATOC-PET application.

Tags

neuroendocrine tumors, carcinoid appendix, MEN 1, Appendectomy, carcinoid, chromogranin A (CgA), 5-hydroxyindoleacetic acid (5 HIAA), DOTATOC-PET

From the magazine:

Kinder- und Jugendmedizin
Issue:
2009 (Vol. 9): number 7 2009
ISSN: 1617-0288
 

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