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Pheochromocytomas and paragangliomas: An opportunity to apply new advances for optimizing clinical management
Feocromocitomas y paragangliomas: Una oportunidad para aplicar los nuevos avances para optimizar el manejo clínico
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Clínica Universidad Navarra, Madrid, Spain" "etiqueta" => "a" "identificador" => "aff0005" ] 1 => array:3 [ "entidad" => "Departamento de Endocrinología y Nutrición. Clínica Universidad Navarra, Pamplona, Spain" "etiqueta" => "b" "identificador" => "aff0010" ] ] "correspondencia" => array:1 [ 0 => array:3 [ "identificador" => "cor0005" "etiqueta" => "⁎" "correspondencia" => "Corresponding author." ] ] ] ] "titulosAlternativos" => array:1 [ "es" => array:1 [ "titulo" => "Feocromocitomas y paragangliomas: Una oportunidad para aplicar los nuevos avances para optimizar el manejo clínico" ] ] "textoCompleto" => "<span class="elsevierStyleSections"><p id="par0005" class="elsevierStylePara elsevierViewall">The study of pheochromocytomas and paragangliomas, jointly referred to as PPGLs, has undergone significant development in recent decades, with several notable discoveries that have improved clinical management in regard to their diagnosis, treatment and follow-up.</p><p id="par0010" class="elsevierStylePara elsevierViewall">The multicenter retrospective study on patients with pheochromocytomas surgically treated in 3 Spanish tertiary reference hospitals published in Revista Clinica Española<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> reported a large series of Spanish patients evaluated over a 37-year period, providing an excellent reflection of this progress.</p><p id="par0015" class="elsevierStylePara elsevierViewall">Although PPGLs are rare neuroendocrine tumors, they can cause significant cardiovascular morbidity and mortality. For this reason, their early diagnosis is of particular interest as it increases the chances for cure. However, PPGLs can be misdiagnosed because they can be confused with other diseases, leading to them being known as the "great masquerader."</p><p id="par0020" class="elsevierStylePara elsevierViewall">Most progress in managing PPGLs have resulted from elucidating their genetic origin. In 1990, the neurofibromin 1 (<span class="elsevierStyleItalic">NF1</span>) gene was the first driver gene reported to be related to the development of PPGL. To date, at least 10 more genes have been implicated as contributors through germline mutations, consolidating PPGL as the human neoplasia with the highest degree of heritability.<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> About 40 % of PPGLs develop in the context of three syndromes: <ul class="elsevierStyleList" id="lis0005"><li class="elsevierStyleListItem" id="lsti0005"><span class="elsevierStyleLabel">1)</span><p id="par0030" class="elsevierStylePara elsevierViewall">Von Hippel-Lindau disease (VHL) caused by <span class="elsevierStyleItalic">VHL</span> mutations.</p></li><li class="elsevierStyleListItem" id="lsti0010"><span class="elsevierStyleLabel">2)</span><p id="par0035" class="elsevierStylePara elsevierViewall">Multiple endocrine neoplasia type 2 caused by <span class="elsevierStyleItalic">RET</span> mutations.</p></li><li class="elsevierStyleListItem" id="lsti0015"><span class="elsevierStyleLabel">3)</span><p id="par0040" class="elsevierStylePara elsevierViewall">Familial PPGL, which includes: 3a) Hereditary PGLs, caused by mutations in succinate dehydrogenase (<span class="elsevierStyleItalic">SDH</span>), fumarate hydratase and malate dehydrogenase type 2 genes. 3b) Familial pheochromocytoma, caused by mutations in the transmembrane protein 127 or the Myc-associated factor X (<span class="elsevierStyleItalic">MAX</span>) genes.</p></li></ul></p><p id="par0045" class="elsevierStylePara elsevierViewall">The genetic scenario of PPGL changed in 2011 when it was reported that 14% of PPGLs could be caused by somatic mutations in <span class="elsevierStyleItalic">RET</span> and VHL genes.<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a> It has become clear that somatic mutations play an important role in PPGL, given that they have been reported in up to 40% of tumors.<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> Other mechanisms such as somatic mosaicism have also been reported in PPGL.</p><p id="par0050" class="elsevierStylePara elsevierViewall">A number of authors still discourage PPGL genetic screening claiming that, given the tumor is rare and mainly benign and its study is an expensive and time-consuming process with a high psychological impact on patients and their relatives. Between 2002 and 2009, various genetic studies have determined that 11–17% of apparently sporadic cases (single, not metastatic tumors with no family history) were carriers of germline mutations.<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> For this reason, the latest clinical practice guidelines from the Endocrine Society suggest considering genetic testing for all patients with PPGLs using a feature-driven diagnostic algorithm with the aim of prioritizing the order and which genes to study.<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5,7</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">Despite this heterogeneous genetic background, integrative genomic studies have provided evidence of a strong concordance between the mutated gene and the data dereived form different “omics” data (transcriptomic gene expression, copy number alterations, metabolomics signature, miRNA profiles, and DNA methylation). This concordance allows for the classification of PPGL tumors into 2 main clusters and 5 molecular subgroups, each displaying a set of genomic alterations and clinical characteristics (predominant biochemical secretion, useful immunohistochemistry and imaging tests, and risk of metastasis or associated syndromic features).<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">Knowing the type of mutation (somatic, germline or mosaicism) and specific mutated gene helps endocrinologists guide not only the diagnosis, early treatment, and follow-up of PPGL for patients and their relatives, but also the early diagnosis of other tumors that may appear to be associated with the mutated genes. This is the case, for example, of clear cell renal carcinoma and medullary thyroid carcinoma in succinate dehydrogenase complex subunit B (<span class="elsevierStyleItalic">SDHB)</span> and <span class="elsevierStyleItalic">RET</span> mutations, respectively.<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5,7</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">In clinical management, the classic rule of 10% attributed to PPGL (the percentage of familial, syndromic, or extra-adrenal cases as well as bilateral, multiple, pediatric, or metastatic cases) is now obsolete and the percentage depends mainly on the mutated gene. For example, the rate of metastasis varies between 10% and 60% of cases, depending on the mutated gene. Knowing this is important, given that in metastatic cases, 5-year survival is only 50%.<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5,7,9</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">The biochemical diagnosis is crucial not only to determining whether the tumor is silent but also to clarifying its predominant biochemical secretion (adrenergic, dopaminergic or noradrenergic).<a class="elsevierStyleCrossRef" href="#bib0050"><span class="elsevierStyleSup">10</span></a> In recent years, the measurement of catecholamines and vanillylmandelic acid has been substituted with plasma and/or 24-h urinary measurements of metanephrines.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> Knowing the predominant secretion helps guide the genetic study and the most appropriate functional imaging test for determining the possibility of multiple or metastatic tumors. The measurement of dopamine and its metabolite, 3-methoxytyramine, has been shown to be especially important, as high plasma concentrations have been linked to the presence of metastasis and a worse prognosis.<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">7,11</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">Despite the fact it is known that there are rare PPGL silent (non-secreting) tumors, imaging studies should be conducted once there is biochemical evidence of a PPGL, with abdominal computed tomography and magnetic resonance imaging, the most widely used imaging techniques for localizing the tumor. Functional imaging techniques are recommended for ruling out multiple and metastatic tumors in PPGLs that have the following characteristics: larger than 5 cm; extra-adrenal localization; increased 3-methoxytyramine levels; hereditary syndromes; <span class="elsevierStyleItalic">SDHB</span> carriers; and multifocal, recurrent, and silent tumors. Currently, <span class="elsevierStyleSup">123</span>I-metaiodobenzylguanidine scintigraphy is available in most centers, though it has lower sensitivity than the new positron emission tomography techniques.<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a></p><p id="par0080" class="elsevierStylePara elsevierViewall">The main techniques, after <span class="elsevierStyleSup">123</span>I- metaiodobenzylguanidine scintigraphy, are 18F-DOPA and <span class="elsevierStyleSup"><span class="elsevierStyleInf">68</span></span>gallium-labeled somatostatin analogs.<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5,7</span></a> Other techniques such as <span class="elsevierStyleItalic">in vivo</span> hydrogen 1 magnetic resonance spectroscopy have been used in cases of SDH mutations.<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">12</span></a></p><p id="par0085" class="elsevierStylePara elsevierViewall">Pathological findings confirm the biochemical diagnosis of PPGL. In the report, in addition to the main scales for adrenal tumors, such as the Pheochromocytoma of the Adrenal Scaled Score (PASS) and the Grading of Adrenal Pheochromocytoma and Paraganglioma (GAPP) scales, it is important to include the mitotic index, the Ki-67 index, and if the S-100 protein is detected. Immunochemistry of SDHB and other markers (SDHA, MAX, 2SC) can help guide genetic studies and predict the probability of metastasis.<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">13</span></a></p><p id="par0090" class="elsevierStylePara elsevierViewall">As shown by Iglesias et al.,<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a> the ideal preoperative medical management of patients with PPGL is currently unknown and there is no agreement as to whether phenoxybenzamine or doxazosin is the optimal alpha-adrenoreceptor antagonist to be administered prior to surgical resection of PPGLs.<a class="elsevierStyleCrossRefs" href="#bib0025"><span class="elsevierStyleSup">5,14</span></a></p><p id="par0095" class="elsevierStylePara elsevierViewall">In conclusion, a proper medical history, family history, and physical examination (searching for syndromic characteristics) are crucial for the proper management of PPGL. In terms of the biochemical study, the predominant biochemical secretion must be determined in order to guide which functional imaging techniques and genetic studies should be used. Finally, it is important that the pathology report includes Ki-67 and SDHB immunochemistry information, the latter in order to rule out involvement of SDH mutations that are associated with an increased risk of multiplicity and the presence of metastasis.</p></span>" "pdfFichero" => "main.pdf" "tienePdf" => true "NotaPie" => array:1 [ 0 => array:2 [ "etiqueta" => "☆" "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as: Curras-Freixes M and Salvador J. Feocromocitomas y paragangliomas: Una oportunidad para aplicar los nuevos avances para optimizar el manejo clínico. Rev Clin Esp. 2021;221:30–32.</p>" ] ] "bibliografia" => array:2 [ "titulo" => "References" "seccion" => array:1 [ 0 => array:2 [ "identificador" => "bibs0005" "bibliografiaReferencia" => array:14 [ 0 => array:3 [ "identificador" => "bib0005" "etiqueta" => "1" "referencia" => array:1 [ 0 => array:2 [ "contribucion" => array:1 [ 0 => array:2 [ "titulo" => "Feocromocitoma: una experiencia clínica de 3 décadas en un estudio multicéntrico" "autores" => array:1 [ 0 => array:2 [ "etal" => true "autores" => array:6 [ 0 => "P. Iglesias" 1 => "E. Santacruz" 2 => "P. García-Sancho" 3 => "E. Pia Marengo" 4 => "F. Guerrero-Pérez" 5 => "H. 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