Revisión actual de la etiología, diagnóstico, y tratamiento del feocromocitoma y paraganglioma en pediatría.

Revisión actual de la etiología, diagnóstico, y tratamiento del feocromocitoma y paraganglioma en pediatría. A current review of the etiology, diagnosis, and treatment of pediatric pheochromocytoma and paraganglioma. Waguespack SG, Rich T, Grubbs E, Ying AK, Perrier ND, Ayala-Ramirez M, Jimenez C. Department of Endocrine Neoplasia and Hormonal Disorders, The Children's Cancer Hospital, University of Texas M. D. Anderson Cancer Center, PO Box 301402, Unit 1461, Houston, Texas 77230-1402, USA.swagues@mdanderson.org J Clin Endocrinol Metab. 2010 May;95(5):2023-37. Epub 2010 Mar 9. Abstract CONTEXT: Pheochromocytomas and paragangliomas (PHEO/PGL) are neuroendocrine tumors that arise from sympathetic and parasympathetic paraganglia. Diagnosed rarely during childhood, PHEO/PGL are nonetheless important clinical entities, particularly given our evolving understanding of their pathophysiology. EVIDENCE ACQUISITION: We identified articles through the U.S. National Library of Medicine by using the search terms pheochromocytoma and paraganglioma. Results were narrowed to manuscripts that included children and studies related to the genetics of PHEO/PGL. Web-based resources for genetic disorders were also used. For all articles, we performed subsequent reference searches and verification of source data. EVIDENCE SYNTHESIS: Up to 20% of PHEO/PGL are diagnosed in children. Most are functional tumors, and clinical presentation includes symptoms related to catecholamine hypersecretion and/or tumor mass effect. Increasingly, PHEO/PGL are identified during presymptomatic screening in children with genetic syndromes associated with PHEO/PGL (multiple endocrine neoplasia type 2, von Hippel-Lindau disease, and the paraganglioma syndromes). Plasma and/or urine metanephrines are the best diagnostic test for a functional tumor, and the management of pediatric patients is similar to adults. Genetic counseling should be undertaken in all cases. Although most pediatric PHEO/PGL are benign, these tumors can occasionally metastasize, a condition for which no curative treatment exists. CONCLUSIONS: Although PHEO/PGL are rarely diagnosed during childhood, the pediatric provider should be able to recognize and screen for such tumors, particularly in the context of a known genetic predisposition. Optimal care of these children includes a multidisciplinary team approach at centers experienced in the evaluation and treatment of these uncommon yet fascinating endocrine neoplasms. http://jcem.endojournals.org/content/95/5/2023.full.pdf+html   Feocromocitoma: abordaje actual y direcciones futuras  Pheochromocytoma: current approaches and future directions. Adler JT, Meyer-Rochow GY, Chen H, Benn DE, Robinson BG, Sippel RS, Sidhu SB. Section of Endocrine Surgery, Department of Surgery, University of Wisconsin, Madison, Wisconsin, USA. Oncologist. 2008 Jul;13(7):779-93. Epub 2008 Jul 10. Abstract Pheochromocytomas are rare catecholamine-secreting tumors that arise from chromaffin tissue within the adrenal medulla and extra-adrenal sites. Because of the excess secretion of hormones, these tumors often cause debilitating symptoms and a poor quality of life. While medical management plays a significant role in the treatment of pheochromocytoma patients, surgical excision remains the only cure. Improved medical management and surgical techniques and an increased understanding of hereditary disease have improved the outcome of pheochromocytoma patients with benign disease; however, the outcome of patients with malignant disease remains poor. In this review, we discuss the presentation, diagnosis, management, and future directions in the management of this disease. http://theoncologist.alphamedpress.org/content/13/7/779.full.pdf+html   Manejo anestésico de feocromocitoma: reporte de un caso Dra. Celia Gómez Ledesma, Dra. Gabriela Silva Ocampo Rev. Mex. Anest. Vol. 26 - No. 3 Julio-Septiembre 2003 El manejo anestésico del paciente con feocromocitoma es un reto para el Anestesiólogo y su equipo quirúrgico. Nuestro objetivo es evitar la mortalidad trans y postoperatoria. Este padecimiento implica amplios conocimientos acerca de esta patología que afortunadamente es poco frecuente. Consideramos que es de vital importancia la comunicación entre el Cirujano, Cardiólogo, Enfermera, Laboratorio y Anestesiólogo, así como la visita preoperatoria es menester infundirle confianza al paciente dado el difícil manejo a que va a ser sometido por todo el equipo quirúrgico. Incluye tratamiento a base de alfa y betabloqueadores 2 a 3 semanas antes de su ingreso a quirófano, el cual debe ser en óptimas condiciones. Podemos concluir que la anestesia mixta con bloqueo epidural más anestesia general es una buena alternativa de manejo. El bloqueo epidural proporciona disminución de la descarga adrenérgica y disminución en el consumo de narcóticos, halogenados y relajantes musculares, además de proporcionar analgesia postoperatoria. Siempre debemos estar atentos y preparados con medicamentos para cualquier contingencia. Palabras clave: Feocromocitoma, bloqueo epidural, anestesia mixta. http://www.medigraphic.com/pdfs/rma/cma-2003/cma033g.pdf    Manejo anestésico del paciente con feocromocitoma Raquel Durá, Elvira Calabuig, Servicio de Anestesia, Reanimación y Terapéutica del Dolor. Consorcio Hospital General Universitario de Valencia. Introducción Los feocromocitomas son tumores poco frecuentes, derivados del sistema APUD, y habitualmente benignos que sintetizan, almacenan y liberan catecolaminas.. Ocurre solamente en el 0,5 - 0,1% de la población hipertensa, pero constituye una causa tratable de HTA. http://chguv.san.gva.es/Inicio/ServiciosSalud/ServiciosHospitalarios/AnestRea/Documents/ProtFeocromocitoma.pdf

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