{"id":145,"date":"2022-09-28T17:15:16","date_gmt":"2022-09-28T17:15:16","guid":{"rendered":"https:\/\/live-rawf.pantheonsite.io\/2022\/09\/28\/2022-9-28-2022-research-progress-on-craniopharyngioma\/"},"modified":"2025-12-23T02:58:47","modified_gmt":"2025-12-23T02:58:47","slug":"2022-9-28-2022-avances-en-la-investigacion-del-craneofaringioma","status":"publish","type":"post","link":"https:\/\/www.rawoodfoundation.org\/es\/2022\/09\/28\/2022-9-28-2022-research-progress-on-craniopharyngioma\/","title":{"rendered":"2022 Avances en la investigaci\u00f3n del craneofaringioma"},"content":{"rendered":"<div class=\"sqs-html-content\">\n<p class=\"\" style=\"white-space: pre-wrap;\"><em>por Nathalie Kayadjanian, Doctora y Asesora Cient\u00edfica de RAWF<\/em><\/p>\n<\/div>\n<div class=\"image-block-outer-wrapper          layout-caption-below          design-layout-inline          combination-animation-none          individual-animation-none          individual-text-animation-none\" data-test=\"image-block-inline-outer-wrapper\"><\/p>\n<figure class=\"sqs-block-image-figure              intrinsic\" style=\"max-width: 571px;\"><\/p>\n<div class=\"image-block-wrapper\" data-animation-role=\"image\">\n<div class=\"sqs-image-shape-container-element              has-aspect-ratio\" style=\"position: relative; padding-bottom: 80.03502655029297%; overflow: hidden; -webkit-mask-image: -webkit-radial-gradient(white, black);\"><\/p>\n<p><noscript><img decoding=\"async\" src=\"https:\/\/images.squarespace-cdn.com\/content\/v1\/587bc3d946c3c4be42a60e48\/afb72af1-4aaf-4b6a-ac1b-e062e470f14a\/803629-571.jpg\" alt=\"\"><\/noscript><img decoding=\"async\" class=\"thumb-image\" src=\"https:\/\/images.squarespace-cdn.com\/content\/v1\/587bc3d946c3c4be42a60e48\/afb72af1-4aaf-4b6a-ac1b-e062e470f14a\/803629-571.jpg\" alt=\"\" data-image=\"https:\/\/images.squarespace-cdn.com\/content\/v1\/587bc3d946c3c4be42a60e48\/afb72af1-4aaf-4b6a-ac1b-e062e470f14a\/803629-571.jpg\" data-image-dimensions=\"571x457\" data-image-focal-point=\"0.5,0.5\" data-load=\"false\" data-image-id=\"6334829b28124d3f86722a35\" data-type=\"image\" \/><\/p>\n<\/div>\n<\/div><figcaption class=\"image-caption-wrapper\">\n<div class=\"image-caption\">\n<p class=\"\" style=\"white-space: pre-wrap;\">Cr\u00e9dito de la imagen: Instituto Nacional del C\u00e1ncer<\/p>\n<\/div>\n<\/figcaption><\/figure>\n<\/div>\n<div class=\"sqs-html-content\">\n<p class=\"\" style=\"white-space: pre-wrap;\">Aunque la cirug\u00eda y la radioterapia representan las terapias de primera l\u00ednea recomendadas para el CP, son responsables, en parte, de las comorbilidades asociadas al CP, entre las que se incluyen alteraciones metab\u00f3licas primarias, como la obesidad, trastornos neuroendocrinos y alteraciones psicosociales. Los recientes avances en la comprensi\u00f3n de las alteraciones gen\u00f3micas y las v\u00edas moleculares implicadas en la tumorig\u00e9nesis de la CP han contribuido al avance de los f\u00e1rmacos de diagn\u00f3stico y ensayo dirigidos a estas v\u00edas. Un ejemplo de \u00e9xito es la significativa reducci\u00f3n tumoral medida en pacientes con un CP de inicio en la edad adulta tratados con inhibidores de BRAF y MEK de la que se ha informado recientemente en varios estudios cl\u00ednicos y en un ensayo cl\u00ednico de fase II (Fasano et al., 2022). La detecci\u00f3n de mutaciones de BRAF en suero (Brastianos et al.2016) puede contribuir a las evaluaciones diagn\u00f3sticas preoperatorias en un futuro pr\u00f3ximo. En conjunto, estos resultados est\u00e1n allanando el camino para el diagn\u00f3stico no quir\u00fargico y la estrategia de tratamiento molecular dirigido neoadyuvante para pacientes con CP.<\/p>\n<p class=\"\" style=\"white-space: pre-wrap;\"><a href=\"https:\/\/youtu.be\/9BvQR-Qqm1Q\">Deficiencia de hormona del crecimiento (GH)<\/a> afecta al patr\u00f3n de crecimiento en 75% de ni\u00f1os con PC. En adultos con PC, la deficiencia de GH se caracteriza por disminuci\u00f3n de la masa muscular, aumento de la grasa corporal, reducci\u00f3n de la energ\u00eda y el bienestar. La terapia de reemplazo de GH es la \u00fanica terapia eficaz. Adem\u00e1s de su efecto positivo sobre el crecimiento en ni\u00f1os, el reemplazo de GH tiene beneficios sobre la composici\u00f3n corporal, la salud \u00f3sea, los resultados metab\u00f3licos y la calidad de vida. Aunque la terapia con GH plante\u00f3 problemas de seguridad debido a sus conocidas propiedades pro-proliferativas, angiog\u00e9nicas y anti-apopt\u00f3ticas, numerosos estudios han demostrado que la terapia de reemplazo de GH no aumenta el riesgo de recurrencia tumoral en pacientes con CP. Este a\u00f1o se ha publicado una declaraci\u00f3n de consenso a favor de la terapia con GH en la CP (Boguszewski et al., 2022).<\/p>\n<p class=\"\" style=\"white-space: pre-wrap;\">Anteriormente se hab\u00edan descrito dos tipos de CP, el adamantinomatoso (ACP) y el papilar (PCP), bas\u00e1ndose en sus caracter\u00edsticas histol\u00f3gicas. Gracias a los avances moleculares y cl\u00ednicos, la OMS modific\u00f3 la taxonom\u00eda de los tumores cerebrales del SNC (5\u00aa edici\u00f3n), de modo que, el ACP y el PCP se reconocen ahora como tipos de tumores de CP distintos, dados sus diferentes perfiles clinicodemogr\u00e1ficos, radiol\u00f3gicos, histopatol\u00f3gicos y de alteraciones gen\u00e9ticas. Aunque puede afectar a adultos, el ACP es mayoritariamente un tumor pedi\u00e1trico y representa el tumor hipofisario m\u00e1s frecuente en ni\u00f1os. <a href=\"https:\/\/www.rawoodfoundation.org\/es\/craneofaringioma\/\" target=\"_blank\" rel=\"noopener\">Tratamiento del craneofaringioma infantil<\/a> se centra en equilibrar la extirpaci\u00f3n eficaz del tumor con la preservaci\u00f3n de la funci\u00f3n hormonal y la calidad de vida. En cambio, la NPC afecta predominantemente a adultos.<\/p>\n<p class=\"\" style=\"white-space: pre-wrap;\">La investigaci\u00f3n sobre la par\u00e1lisis cerebral carece de datos basados en pruebas sobre los puntos de vista de los pacientes y los cuidadores acerca de los s\u00edntomas de importancia y las prioridades de tratamiento. Recientemente se ha publicado una encuesta a pacientes y cuidadores para comprender mejor los enfoques que adoptan las familias respecto al control del peso y el impacto en la funci\u00f3n social de las personas con par\u00e1lisis cerebral y obesidad (Craven et al., 2022). Resulta interesante que los resultados destaquen la oportunidad de mejorar el funcionamiento social como un beneficio potencial adicional de los tratamientos mejorados para la obesidad hipotal\u00e1mica.<\/p>\n<p class=\"\" style=\"white-space: pre-wrap;\">Paralelamente a las mejoras en los resultados quir\u00fargicos, el est\u00e1ndar para el \u00e9xito de la cirug\u00eda se ha ampliado para centrarse en la calidad de vida (CdV) informada por el paciente. Una revisi\u00f3n reciente ha demostrado que la CdV de los pacientes con PC era inferior a la de la poblaci\u00f3n general (Castle-Kirszbaum et al., 2022). La afectaci\u00f3n hipotal\u00e1mica fue sistem\u00e1ticamente el factor predictivo m\u00e1s fuerte de la CdV. La endocrinopat\u00eda contribu\u00eda a la morbilidad, pero pod\u00eda mejorarse con terapia hormonal sustitutiva. La desregulaci\u00f3n social y emocional y la mala memoria son quejas frecuentes tras la cirug\u00eda. Este estudio arroja luz sobre comorbilidades poco estudiadas que afectan significativamente a la CdV de los pacientes con PC.<\/p>\n<p class=\"\" style=\"white-space: pre-wrap;\">Los trastornos del sue\u00f1o representan un problema importante en los pacientes con PC. Sin embargo, siguen estando infradiagnosticados y sin tratar en la pr\u00e1ctica cl\u00ednica y no se dispone de directrices para el diagn\u00f3stico y el tratamiento de los trastornos del sue\u00f1o en estos pacientes. Romigi y cols. (2022) han publicado un art\u00edculo reciente sobre el conocimiento actual de la patogenia y la prevalencia de los trastornos del sue\u00f1o en pacientes con PC y proponen algoritmos pr\u00e1cticos para su evaluaci\u00f3n y tratamiento en la pr\u00e1ctica cl\u00ednica.<\/p>\n<p class=\"\" style=\"white-space: pre-wrap;\"><strong>Enlaces relacionados:<\/strong><br \/>\n<a href=\"https:\/\/www.rawoodfoundation.org\/es\/ensayos-clinicos\/\">Ensayos cl\u00ednicos actuales sobre craneofaringioma<\/a><br \/>\n<a href=\"https:\/\/www.frontiersin.org\/articles\/10.3389\/fendo.2022.876770\/full\" target=\"_blank\" rel=\"noopener\">Estudio de los resultados relevantes para el paciente en el craneofaringioma pedi\u00e1trico: Enfoque en la obesidad hipotal\u00e1mica<\/a><br \/>\n<a href=\"\/es\/recien-diagnosticado\/\">Recursos para reci\u00e9n diagnosticados<\/a><\/p>\n<p class=\"\" style=\"white-space: pre-wrap;\"><strong>Referencias<\/strong><\/p>\n<p class=\"\" style=\"white-space: pre-wrap;\">Boguszewski MCS et al., (2022). Safety of growth hormone replacement in survivors of cancer and intracranial and pituitary tumours: a consensus statement. Eur J Endocrinol. 2022 Abr 21;186(6):P35-P52.<\/p>\n<p class=\"\" style=\"white-space: pre-wrap;\">Castle-Kirszbaum M, et al., (2022). Quality of Life in Craniopharyngioma: A Systematic Review. World Neurosurg. Aug;164:424-435.e2. doi: 10.1016\/j.wneu.2022.05.038.<\/p>\n<p class=\"\" style=\"white-space: pre-wrap;\">Craven M, et al (2022) A Survey of Patient-Relevant Outcomes in Pediatric Craniopharyngioma: Focus on Hypothalamic Obesity. Front.  Endocrinol. 13:876770.<\/p>\n<p class=\"\" style=\"white-space: pre-wrap;\">Fasano M et al., (2022) Dramatic Therapeutic Response to Dabrafenib Plus Trametinib in BRAFV600E Mutated Papillary Craniopharyngiomas: A Case Report and Literature Review. Front. Med. 8:652005.<\/p>\n<p class=\"\" style=\"white-space: pre-wrap;\">Romigi A. et al., (2022), Trastornos del sue\u00f1o en pacientes con craneofaringioma: Una Actualizaci\u00f3n Fisiopatol\u00f3gica y Pr\u00e1ctica. Front Neurol. 2022 Feb 9;12:817257. doi: 10.3389\/fneur.2021.817257.<\/p>\n<\/div>","protected":false},"excerpt":{"rendered":"<p>por Nathalie Kayadjanian, doctora y asesora cient\u00edfica de RAWF Cr\u00e9dito de la imagen: Instituto Nacional del C\u00e1ncer Mientras que la cirug\u00eda y ... <a title=\"2022 Avances en la investigaci\u00f3n del craneofaringioma\" class=\"read-more\" href=\"https:\/\/www.rawoodfoundation.org\/es\/2022\/09\/28\/2022-9-28-2022-research-progress-on-craniopharyngioma\/\" aria-label=\"Leer m\u00e1s sobre 2022 Avances en la investigaci\u00f3n del craneofaringioma\">Seguir leyendo<\/a><\/p>","protected":false},"author":6,"featured_media":146,"comment_status":"closed","ping_status":"open","sticky":false,"template":"","format":"standard","meta":{"_acf_changed":false,"footnotes":""},"categories":[35,20,1],"tags":[],"class_list":["post-145","post","type-post","status-publish","format-standard","has-post-thumbnail","hentry","category-craniopharyngioma","category-latest-research","category-uncategorized","generate-columns","tablet-grid-50","mobile-grid-100","grid-parent","grid-33","no-featured-image-padding"],"acf":[],"yoast_head":"<!-- This site is optimized with the Yoast SEO plugin v27.3 - https:\/\/yoast.com\/product\/yoast-seo-wordpress\/ -->\n<title>2022 Research Progress on Craniopharyngioma - Raymond A. 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