Multidisciplinary practice guidelines for the diagnosis, genetic counseling and treatment of pheochromocytomas and paragangliomas.

{"article_title":"Multidisciplinary practice guidelines for the diagnosis, genetic counseling and treatment of pheochromocytomas and paragangliomas.","author":"Garcia-Carbonero R, Matute Teresa F, Mercader-Cidoncha E, Mitjavila-Casanovas M, Robledo M, Tena I, Alvarez-Escola C, Ar\u00edstegui M, Bella-Cueto MR, Ferrer-Albiach C, Hanzu FA","journal_title":"Clinical & translational oncology : official publication of the Federation of Spanish Oncology Societies and of the National Cancer Institute of Mexico","issn":"1699-3055","isbn":"","publication_date":"2021-10-01","volume":"23","issue":"10","first_page":"1995","page_count":"","accession_number":"33959901","doi":"","publisher":"Springer","doctype":"Journal Article","subjects":"Spain; Adrenal Gland Neoplasms diagnosis; Adrenal Gland Neoplasms therapy; Paraganglioma diagnosis; Paraganglioma therapy; Pheochromocytoma diagnosis; Pheochromocytoma therapy; Adrenal Gland Neoplasms genetics; Adrenal Gland Neoplasms pathology; Aftercare; Algorithms; Biomarkers, Tumor blood; Biomarkers, Tumor urine; Catecholamines antagonists & inhibitors; Diagnostic Imaging methods; Genetic Counseling; Genetic Predisposition to Disease; Genetic Testing; Humans; Neoplasm Staging; Paraganglioma genetics; Paraganglioma pathology; Pheochromocytoma genetics; Pheochromocytoma pathology; Societies, Medical; Spain epidemiology; Symptom Assessment methods","interest_area":["Endocrinology"," Oncology"],"abstract":"Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla and the sympathetic\/parasympathetic neural ganglia, respectively. The heterogeneity in its etiology makes PPGL diagnosis and treatment very complex. The aim of this article was to provide practical clinical guidelines for the diagnosis and treatment of PPGLs from a multidisciplinary perspective, with the involvement of the Spanish Societies of Endocrinology and Nutrition (SEEN), Medical Oncology (SEOM), Medical Radiology (SERAM), Nuclear Medicine and Molecular Imaging (SEMNIM), Otorhinolaryngology (SEORL), Pathology (SEAP), Radiation Oncology (SEOR), Surgery (AEC) and the Spanish National Cancer Research Center (CNIO). We will review the following topics: epidemiology; anatomy, pathology and molecular pathways; clinical presentation; hereditary predisposition syndromes and genetic counseling and testing; diagnostic procedures, including biochemical testing and imaging studies; treatment including catecholamine blockade, surgery, radiotherapy and radiometabolic therapy, systemic therapy, local ablative therapy and supportive care. Finally, we will provide follow-up recommendations. \u00a9 2021. The Author(s).","url":"https:\/\/search.ebscohost.com\/login.aspx?direct=true&db=mdl&AN=33959901","isPdfLink":true,"isSAML":false,"an":"33959901","number_other":"","type_pub":"","issn_electronic":"1699-3055","languages":"English","language":"eng","date_entry":"","date_update":"","titleSource":"Clinical & translational oncology : official publication of the Federation of Spanish Oncology Societies and of the National Cancer Institute of Mexico [Clin Transl Oncol] 2021 Oct; Vol. 23 (10), pp. 1995-2019. Date of Electronic Publication: 2021 May 06.","date_pub_cy":"","type_document":"","contract_publisher":"","authored_on":"2021-10-01","description":"Pheochromocytomas and paragangliomas (PPGLs) are rare neuroendocrine tumors that arise from chromaffin cells of the adrenal medulla and the sympathetic\/parasympathetic neural ganglia, respectively. The heterogeneity in its etiology makes PPGL diagnosis and treatment very complex. The aim of this article was to provide practical clinical guidelines for the diagnosis and treatment of PPGLs from a multidisciplinary perspective, with the involvement of the Spanish Societies of Endocrinology and Nutrition (SEEN), Medical Oncology (SEOM), Medical Radiology (SERAM), Nuclear Medicine and Molecular Imaging (SEMNIM), Otorhinolaryngology (SEORL), Pathology (SEAP), Radiation Oncology (SEOR), Surgery (AEC) and the Spanish National Cancer Research Center (CNIO). We will review the following topics: epidemiology; anatomy, pathology and molecular pathways; clinical presentation; hereditary predisposition syndromes and genetic counseling and testing; diagnostic procedures, including biochemical testing and imaging studies; treatment including catecholamine blockade, surgery, radiotherapy and radiometabolic therapy, systemic therapy, local ablative therapy and supportive care. Finally, we will provide follow-up recommendations.<br \/> (© 2021. The Author(s).)","upload_link":"https:\/\/search.ebscohost.com\/login.aspx?direct=true&site=eds-live&db=mdl&AN=33959901&authtype=shib&custid=ns346513&group=main&profile=eds","no_of_pages":"","authored_by":"Garcia-Carbonero R, Matute Teresa F, Mercader-Cidoncha E, Mitjavila-Casanovas M, Robledo M, Tena I, Alvarez-Escola C, Ar\u00edstegui M, Bella-Cueto MR, Ferrer-Albiach C, Hanzu FA","header":{"DbId":"mdl","DbLabel":"MEDLINE Ultimate","An":"33959901","RelevancyScore":"898","PubType":"Academic Journal","PubTypeId":"academicJournal","PreciseRelevancyScore":"897.524475097656"},"plink":"https:\/\/search.ebscohost.com\/login.aspx?direct=true&site=eds-live&db=mdl&AN=33959901&authtype=shib&custid=ns346513&group=main&profile=eds","physicalDescription":{"Pagination":{"StartPage":"1995"}},"additionalInfo":{"Authored_By":"Garcia-Carbonero R, Matute Teresa F, Mercader-Cidoncha E, Mitjavila-Casanovas M, Robledo M, Tena I, Alvarez-Escola C, Ar\u00edstegui M, Bella-Cueto MR, Ferrer-Albiach C, Hanzu FA","Journal_Info":"Publisher: Country of Publication: Italy NLM ID: 101247119 Publication Model: Print-Electronic Cited Medium: Internet ISSN: 1699-3055 (Electronic) Linking ISSN: 1699048X NLM ISO Abbreviation: Clin Transl Oncol Subsets: MEDLINE","Publication_Type":"Journal Article; Practice Guideline","Published_Date":"2021-10-01","Source":"Clinical & translational oncology : official publication of the Federation of Spanish Oncology Societies and of the National Cancer Institute of Mexico [Clin Transl Oncol] 2021 Oct; Vol. 23 (10), pp. 1995-2019. Date of Electronic Publication: 2021 May 06.","Languages":"English","Electronic_ISSN":"1699-3055","MeSH_Terms":"Adrenal Gland Neoplasms\/*diagnosis , Adrenal Gland Neoplasms\/*therapy , Paraganglioma\/*diagnosis , Paraganglioma\/*therapy , Pheochromocytoma\/*diagnosis , Pheochromocytoma\/*therapy, Adrenal Gland Neoplasms\/genetics ; Adrenal Gland Neoplasms\/pathology ; Aftercare ; Algorithms ; Biomarkers, Tumor\/blood ; Biomarkers, Tumor\/urine ; Catecholamines\/antagonists & inhibitors ; Diagnostic Imaging\/methods ; Genetic Counseling ; Genetic Predisposition to Disease ; Genetic Testing ; Humans ; Neoplasm Staging ; Paraganglioma\/genetics ; Paraganglioma\/pathology ; Pheochromocytoma\/genetics ; Pheochromocytoma\/pathology ; Societies, Medical ; Spain\/epidemiology ; Symptom Assessment\/methods","Subjects":"Adrenal Gland Neoplasms genetics, Adrenal Gland Neoplasms pathology, Aftercare, Algorithms, Biomarkers, Tumor blood, Biomarkers, Tumor urine, Catecholamines antagonists & inhibitors, Diagnostic Imaging methods, Genetic Counseling, Genetic Predisposition to Disease, Genetic Testing, Humans, Neoplasm Staging, Paraganglioma genetics, Paraganglioma pathology, Pheochromocytoma genetics, Pheochromocytoma pathology, Societies, Medical, Spain epidemiology, Symptom Assessment methods, Adrenal Gland Neoplasms diagnosis, Adrenal Gland Neoplasms therapy, Paraganglioma diagnosis, Paraganglioma therapy, Pheochromocytoma diagnosis, Pheochromocytoma therapy","Title_Abbreviations":"Clinical & translational oncology : official publication of the Federation of Spanish Oncology Societies and of the National Cancer Institute of Mexico","Volume":"23"}}