Management and outcome of children with neuroendocrine tumors of the appendix in Spain: Is there room for improvement?
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P. Pérez-Albert [1] ; T. de Rojas [1] ; M. Á. Lendínez [2] ; L. Illade [1] ; M. García-Abos [3] ; J. Alonso-Cadenas [1] ; M. Andión [1] ; L. Madero [1]
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[1] Hospital Infantil Universitario Niño Jesus de Madrid
Hospital Infantil Universitario Niño Jesus de Madrid
Madrid, España
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[2] Hospital Regional Universitario de Málaga
Hospital Regional Universitario de Málaga
Málaga, España
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[3] Hospital Universitario de Donostia
Hospital Universitario de Donostia
San Sebastián, España
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- Localización: Clinical & translational oncology, ISSN 1699-048X, Vol. 19, Nº. 9 (September 2017), 2017, págs. 1168-1172
- Idioma: inglés
- Texto completo no disponible (Saber más ...)
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Resumen
Purpose Neuroendocrine tumors (NETs) are, after lymphomas, the most frequent gastrointestinal tumors in children, mainly located in the appendix. Best management remains unclear, given the absence of pediatric guidelines. We present the first Spanish series of pediatric patients with NETs.
Patients and methods Retrospective study of all pediatric patients (<18 years) with NET treated in four oncology reference institutions in Spain between 1994 and 2015.
Results Seventeen patients were included. All patients presented with acute abdomen. TNM stage was T1a (82%) and T1b (12%). Extension study was heterogenous, with only 4 patients undergoing an OctreoScan. Four patients met criteria for second surgery (affected surgical margins or mesoappendix invasion), but it was only performed in two. Despite the diverse management, none of the patients relapsed during follow-up.
Conclusions The disparity in diagnostic tests, second surgery criteria and follow-up shown in this study highlights the need for specific pediatric guidelines.
