endosergЭндокринная хирургияEndocrine Surgery2306-35132310-3965Типография «Печатных дел Мастер»10.14341/serg2015315-33endoserg-7768Research ArticleНациональные клинические рекомендацииNational clinical recomendationsКлинические рекомендации Российской ассоциации эндокринологов по диагностике и лечению феохромоцитомы/параганглиомыRussian Association of Endocrinologists clinical practice guidelines for diagnosis and treatment of pheochromocytoma and paragangliomaМельниченкоГалина АфанасьевнаMel'nichenkoGalina Afanas'evna

MD, PhD, Professor

teofrast2000@mail.ruТрошинаЕкатерина АнатольевнаTroshinaEkaterina Anatol'evna

MD, PhD, Professor

troshina@inbox.ruБельцевичДмитрий ГермановичBel'tsevichDmitriy Germanovich

MD, PhD

beltsevich@rambler.ruКузнецовНиколай СергеевичKuznetsovNikolay Sergeevich

MD, PhD, Professor

kuznetsov-enc@yandex.ruЮкинаМарина ЮрьевнаYukinaMarina Yur'evna

MD, PhD

Kuronova@yandex.ruФГБУ “Эндокринологический научный центр” Минздрава России, ГБОУ ВПО “Первый МГМУ им. И.М. Сеченова”РоссияEndocrinology Research Centre; Sechenov First Moscow State Medical UniversityRussian FederationФГБУ “Эндокринологический научный центр” Минздрава РоссииРоссияEndocrinology Research CentreRussian Federation201518122015931533Copyright © Мельниченко Г.А., Трошина Е.А., Бельцевич Д.Г., Кузнецов Н.С., Юкина М.Ю., 20152015Мельниченко Г.А., Трошина Е.А., Бельцевич Д.Г., Кузнецов Н.С., Юкина М.Ю.Mel'nichenko G.A., Troshina E.A., Bel'tsevich D.G., Kuznetsov N.S., Yukina M.Y.Данная работа распространяется под лицензией Creative Commons Attribution 4.0.This work is licensed under a Creative Commons Attribution 4.0 License.https://www.surg-endojournals.ru/jour/article/view/7768

В Клинических рекомендациях обсуждаются современные подходы к диагностике и лечению феохромоцитомы/параганглиомы.

Russian clinical practice guidelines are dedicated to the management of patients with pheochromocytoma and paraganglioma.

феохромоцитомапараганглиномаскринингметанефриныжидкостная хроматографиямасс-спектрометриякомпьютерная томографиямагнитно-резонансная томографияметайодбензилгуанидинсцинтиграфияпозитронно-эмиссионная томографияpheochromocytomaparagangliomascreeningplasma free metanephrinesurinary fractionated metanephrinesliquid chromatographymass spectrometrycomputed tomographymagnetic res-onance imaging123I-metaiodobenzylguanidine18F-fluorodeoxyglucoseReferencesDeLellis RA, Lloyd RV, Heitz PU, Eng C. Pathology and Genetics of Tumours of Endocrine Organs (IARC WHO Classification of Tumours). Lyon, France: World Health Organization; 2004.DeLellis RA, Lloyd RV, Heitz PU, Eng C. Pathology and Genetics of Tumours of Endocrine Organs (IARC WHO Classification of Tumours). Lyon, France: World Health Organization; 2004.Юкина М.Ю., Трошина Е.А., Бельцевич Д.Г., и др. Феохромоцитома/параганглиома: клинико-генетические аспекты. // Проблемы эндокринологии. – 2013. – Т. 59. – №3 – C. 19–26. [Iukina MIu, Troshina EA, Bel'tsevich DG, et al. Pheochromocytoma/paraganglioma: clinical and genetic aspects. Probl Endokrinol (Mosk). 2013;59(3):19-26. (In Russ).] doi: 10.14341/probl201359319-26.Юкина М.Ю., Трошина Е.А., Бельцевич Д.Г., и др. Феохромоцитома/параганглиома: клинико-генетические аспекты. // Проблемы эндокринологии. – 2013. – Т. 59. – №3 – C. 19–26. [Iukina MIu, Troshina EA, Bel'tsevich DG, et al. Pheochromocytoma/paraganglioma: clinical and genetic aspects. Probl Endokrinol (Mosk). 2013;59(3):19-26. (In Russ).] doi: 10.14341/probl201359319-26.Stolk RF, Bakx C, Mulder J, et al. Is the excess cardiovascular morbidity in pheochromocytoma related to blood pressure or to catecholamines? J Clin Endocrinol Metab. 2013;98(3):1100-1106. doi: 10.1210/jc.2012-3669.Stolk RF, Bakx C, Mulder J, et al. Is the excess cardiovascular morbidity in pheochromocytoma related to blood pressure or to catecholamines? J Clin Endocrinol Metab. 2013;98(3):1100-1106. doi: 10.1210/jc.2012-3669.Бельцевич Д.Г., Трошина Е.А., Юкина М.Ю., и др. Инциденталома надпочечников. Диагностика и дифференциальная диагностика. Методические рекомендации для врачей первичного звена. / Под ред. Дедова И.И., Мельниченко Г.А. – М.; 2014. [Bel'cevich DG, Troshina EA, Iukina MJu, et al. Incidentaloma nadpochechnikov. Diagnostika i differencial'naja diagnostika. Metodicheskie rekomendacii dlja vrachej pervichnogo zvena. Ed. by Dedov I.I., Mel'nichenko G.A. Moscow; 2014. (In Russ).]Бельцевич Д.Г., Трошина Е.А., Юкина М.Ю., и др. Инциденталома надпочечников. Диагностика и дифференциальная диагностика. Методические рекомендации для врачей первичного звена. / Под ред. Дедова И.И., Мельниченко Г.А. – М.; 2014. [Bel'cevich DG, Troshina EA, Iukina MJu, et al. Incidentaloma nadpochechnikov. Diagnostika i differencial'naja diagnostika. Metodicheskie rekomendacii dlja vrachej pervichnogo zvena. Ed. by Dedov I.I., Mel'nichenko G.A. Moscow; 2014. (In Russ).]Дедов И.И., Бельцевич Д.Г., Кузнецов Н.С., Мельниченко Г.А. Феохромоцитома. – М.: Практическая медицина; 2005. [Dedov II, Bel'cevich DG, Kuznecov NS, Mel'nichenko GA. Pheochromocytoma. Moscow: Prakticheskaja medicina; 2005. (In Russ).]Дедов И.И., Бельцевич Д.Г., Кузнецов Н.С., Мельниченко Г.А. Феохромоцитома. – М.: Практическая медицина; 2005. [Dedov II, Bel'cevich DG, Kuznecov NS, Mel'nichenko GA. Pheochromocytoma. Moscow: Prakticheskaja medicina; 2005. (In Russ).]Lenders JWM, Duh Q-Y, Eisenhofer G, et al. Pheochromocytoma and paraganglioma: An endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2014;99(6):1915-1942. doi: 10.1210/jc.2014-1498.Lenders JWM, Duh Q-Y, Eisenhofer G, et al. Pheochromocytoma and paraganglioma: An endocrine society clinical practice guideline. J Clin Endocrinol Metab. 2014;99(6):1915-1942. doi: 10.1210/jc.2014-1498.Lenders JWM, Pacak K, Walther MM, et al. Biochemical diagnosis of pheochromocytoma. JAMA. 2002;287(11). doi: 10.1001/jama.287.11.1427.Lenders JWM, Pacak K, Walther MM, et al. Biochemical diagnosis of pheochromocytoma. JAMA. 2002;287(11). doi: 10.1001/jama.287.11.1427.Peitzsch M, Pelzel D, Glцckner S, et al. Simultaneous liquid chromatography tandem mass spectrometric determination of urinary free metanephrines and catecholamines, with comparisons of free and deconjugated metabolites. Clinica Chimica Acta. 2013;418:50-58. doi: 10.1016/j.cca.2012.12.031.Peitzsch M, Pelzel D, Glцckner S, et al. Simultaneous liquid chromatography tandem mass spectrometric determination of urinary free metanephrines and catecholamines, with comparisons of free and deconjugated metabolites. Clinica Chimica Acta. 2013;418:50-58. doi: 10.1016/j.cca.2012.12.031.Mullins F, O’shea P, Fitzgerald R, et al. Enzyme-linked immunoassay for plasma-free metanephrines in the biochemical diagnosis of phaeochromocytoma in adults is not ideal. Clin Chem Lab Med. 2012;50(1). doi: 10.1515/cclm.2011.742.Mullins F, O’shea P, Fitzgerald R, et al. Enzyme-linked immunoassay for plasma-free metanephrines in the biochemical diagnosis of phaeochromocytoma in adults is not ideal. Clin Chem Lab Med. 2012;50(1). doi: 10.1515/cclm.2011.742.Lenders JWM, Willemsen JJ, Eisenhofer G, et al. Is supine rest necessary before blood sampling for plasma metanephrines? Clin Chem. 2006;53(2):352-354. doi: 10.1373/clinchem.2006.076489.Lenders JWM, Willemsen JJ, Eisenhofer G, et al. Is supine rest necessary before blood sampling for plasma metanephrines? Clin Chem. 2006;53(2):352-354. doi: 10.1373/clinchem.2006.076489.Yu R, Wei M. False positive test results for pheochromocytoma from 2000 to 2008. Exp Clin Endocrinol Diabetes. 2010;118:577-585. doi: 10.1055/s-0029-1237699.Yu R, Wei M. False positive test results for pheochromocytoma from 2000 to 2008. Exp Clin Endocrinol Diabetes. 2010;118:577-585. doi: 10.1055/s-0029-1237699.Neary NM, King KS, Pacak K. Drugs and pheochromocytoma – don’t be fooled by every elevated metanephrine. N Engl J Med.2011;364:2268-2270. doi: 10.1056/NEJMc1101502.Neary NM, King KS, Pacak K. Drugs and pheochromocytoma – don’t be fooled by every elevated metanephrine. N Engl J Med.2011;364:2268-2270. doi: 10.1056/NEJMc1101502.Algeciras-Schimnich A, Preissner CM, Young WF, et al. Plasma chromogranin a or urine fractionated metanephrines follow-up testing improves the diagnostic accuracy of plasma fractionated metanephrines for pheochromocytoma. J Clin Endocrinol Metab. 2008;93(1):91-95. doi: 10.1210/jc.2007-1354.Algeciras-Schimnich A, Preissner CM, Young WF, et al. Plasma chromogranin a or urine fractionated metanephrines follow-up testing improves the diagnostic accuracy of plasma fractionated metanephrines for pheochromocytoma. J Clin Endocrinol Metab. 2008;93(1):91-95. doi: 10.1210/jc.2007-1354.De Jong WHA, Eisenhofer G, Post WJ, et al. Dietary influences on plasma and urinary metanephrines: Implications for diagnosis of catecholamine-producing tumors. J Clin Endocrinol Metab. 2009;94(8):2841-2849. doi: 10.1210/jc.2009-0303.De Jong WHA, Eisenhofer G, Post WJ, et al. Dietary influences on plasma and urinary metanephrines: Implications for diagnosis of catecholamine-producing tumors. J Clin Endocrinol Metab. 2009;94(8):2841-2849. doi: 10.1210/jc.2009-0303.Gimenez-Roqueplo A-P, Caumont-Prim A, Houzard C, et al. Imaging work-up for screening of paraganglioma and pheochromocytoma in SDHX mutation carriers: A multicenter prospective study from the pgl.Eva investigators. J Clin Endocrinol Metab. 2013;98(1):E162-E173. doi: 10.1210/jc.2012-2975.Gimenez-Roqueplo A-P, Caumont-Prim A, Houzard C, et al. Imaging work-up for screening of paraganglioma and pheochromocytoma in SDHX mutation carriers: A multicenter prospective study from the pgl.Eva investigators. J Clin Endocrinol Metab. 2013;98(1):E162-E173. doi: 10.1210/jc.2012-2975.Luster M, Karges W, Zeich K, et al. Clinical value of 18F-fluorodihydroxyphenylalanine positron emission tomography/computed tomography (18F-dopa pet/ct) for detecting pheochromocytoma. Eur J Nucl Med Mol Imaging. 2009;37(3):484-493. doi: 10.1007/s00259-009-1294-7.Luster M, Karges W, Zeich K, et al. Clinical value of 18F-fluorodihydroxyphenylalanine positron emission tomography/computed tomography (18F-dopa pet/ct) for detecting pheochromocytoma. Eur J Nucl Med Mol Imaging. 2009;37(3):484-493. doi: 10.1007/s00259-009-1294-7.Lev I, Kelekar G, Waxman A, et al. Clinical use and utility of metaiodobenzylguanidine scintigraphy in pheochromocytoma diagnosis. Endocr Pract. 2010;16(3):398-407. doi: 10.4158/ep09302.or.Lev I, Kelekar G, Waxman A, et al. Clinical use and utility of metaiodobenzylguanidine scintigraphy in pheochromocytoma diagnosis. Endocr Pract. 2010;16(3):398-407. doi: 10.4158/ep09302.or.Fonte JS, Robles JF, Chen CC, et al. False-negative 123I-MIBG spect is most commonly found in SDHB-related pheochromocytoma or paraganglioma with high frequency to develop metastatic disease. Endocr Relat Cancer. 2011;19(1):83-93. doi: 10.1530/erc-11-0243.Fonte JS, Robles JF, Chen CC, et al. False-negative 123I-MIBG spect is most commonly found in SDHB-related pheochromocytoma or paraganglioma with high frequency to develop metastatic disease. Endocr Relat Cancer. 2011;19(1):83-93. doi: 10.1530/erc-11-0243.Shulkin BL, Thompson NW, Shapiro B, et al. Pheochromocytomas: Imaging with 2-[fluorine-18]fluoro-2-deoxy-d-glucose PET1. Radiology. 1999;212(1):35-41. doi: 10.1148/radiology.212.1.r99jl3035.Shulkin BL, Thompson NW, Shapiro B, et al. Pheochromocytomas: Imaging with 2-[fluorine-18]fluoro-2-deoxy-d-glucose PET1. Radiology. 1999;212(1):35-41. doi: 10.1148/radiology.212.1.r99jl3035.Timmers HJLM, Chen CC, Carrasquillo JA, et al. Staging and functional characterization of pheochromocytoma and paraganglioma by 18F-fluorodeoxyglucose (18F-fdg) positron emission tomography. J Natl Cancer Inst. 2012;104(9):700-708. doi: 10.1093/jnci/djs188.Timmers HJLM, Chen CC, Carrasquillo JA, et al. Staging and functional characterization of pheochromocytoma and paraganglioma by 18F-fluorodeoxyglucose (18F-fdg) positron emission tomography. J Natl Cancer Inst. 2012;104(9):700-708. doi: 10.1093/jnci/djs188.Neumann HPH, Bausch B, Mcwhinney SR, et al. Germline mutations in nonsyndromic pheochromocytoma. N Engl J Med. 2002;346(19):1459-1466. doi: 10.1056/NEJMoa020152.Neumann HPH, Bausch B, Mcwhinney SR, et al. Germline mutations in nonsyndromic pheochromocytoma. N Engl J Med. 2002;346(19):1459-1466. doi: 10.1056/NEJMoa020152.Jafri M, Whitworth J, Rattenberry E, et al. Evaluation of SDHB, SDHD and VHL gene susceptibility testing in the assessment of individuals with non-syndromic phaeochromocytoma, paraganglioma and head and neck paraganglioma. Clin Endocrinol (Oxf). 2013;78(6):898-906. doi: 10.1111/cen.12074.Jafri M, Whitworth J, Rattenberry E, et al. Evaluation of SDHB, SDHD and VHL gene susceptibility testing in the assessment of individuals with non-syndromic phaeochromocytoma, paraganglioma and head and neck paraganglioma. Clin Endocrinol (Oxf). 2013;78(6):898-906. doi: 10.1111/cen.12074.Brito JP, Asi N, Bancos I, et al. Testing for germline mutations in sporadic pheochromocytoma/paraganglioma: A systematic review. Clin Endocrinol (Oxf). 2015;82(3):338-345. doi: 10.1111/cen.12530.Brito JP, Asi N, Bancos I, et al. Testing for germline mutations in sporadic pheochromocytoma/paraganglioma: A systematic review. Clin Endocrinol (Oxf). 2015;82(3):338-345. doi: 10.1111/cen.12530.Pasini B, Stratakis CA. SDH mutations in tumorigenesis and inherited endocrine tumours: Lesson from the phaeochromocytoma-paraganglioma syndromes. J Intern Med. 2009; 266(1):19-42. doi: 10.1111/j.1365-2796.2009.02111.x.Pasini B, Stratakis CA. SDH mutations in tumorigenesis and inherited endocrine tumours: Lesson from the phaeochromocytoma-paraganglioma syndromes. J Intern Med. 2009; 266(1):19-42. doi: 10.1111/j.1365-2796.2009.02111.x.Cascon A, Inglada-Perez L, Comino-Mendez I, et al. Genetics of pheochromocytoma and paraganglioma in spanish pediatric patients. Endocr Relat Cancer. 2013;20(3):L1-L6. doi: 10.1530/erc-12-0339.Cascon A, Inglada-Perez L, Comino-Mendez I, et al. Genetics of pheochromocytoma and paraganglioma in spanish pediatric patients. Endocr Relat Cancer. 2013;20(3):L1-L6. doi: 10.1530/erc-12-0339.Eisenhofer G, Lenders JWM, Timmers H, et al. Measurements of plasma methoxytyramine, normetanephrine, and metanephrine as discriminators of different hereditary forms of pheochromocytoma. Clin Chem. 2011;57(3):411-420. doi: 10.1373/clinchem.2010.153320.Eisenhofer G, Lenders JWM, Timmers H, et al. Measurements of plasma methoxytyramine, normetanephrine, and metanephrine as discriminators of different hereditary forms of pheochromocytoma. Clin Chem. 2011;57(3):411-420. doi: 10.1373/clinchem.2010.153320.Burnichon N, Cascon A, Schiavi F, et al. MAX mutations cause hereditary and sporadic pheochromocytoma and paraganglioma. Clin Cancer Res. 2012;18(10):2828-2837. doi: 10.1158/1078-0432.ccr-12-0160.Burnichon N, Cascon A, Schiavi F, et al. MAX mutations cause hereditary and sporadic pheochromocytoma and paraganglioma. Clin Cancer Res. 2012;18(10):2828-2837. doi: 10.1158/1078-0432.ccr-12-0160.Houdayer C, Caux-Moncoutier V, Krieger S, et al. Guidelines for splicing analysis in molecular diagnosis derived from a set of 327 combined in silico/in vitro studies on brca1 and brca2 variants. Hum Mutat. 2012;33(8):1228-1238. doi: 10.1002/humu.22101.Houdayer C, Caux-Moncoutier V, Krieger S, et al. Guidelines for splicing analysis in molecular diagnosis derived from a set of 327 combined in silico/in vitro studies on brca1 and brca2 variants. Hum Mutat. 2012;33(8):1228-1238. doi: 10.1002/humu.22101.Shao Y, Chen R, Shen Z-J, et al. Preoperative alpha blockade for normotensive pheochromocytoma. J Hypertens. 2011; 29(12):2429-2432. doi: 10.1097/HJH.0b013e32834d24d9.Shao Y, Chen R, Shen Z-J, et al. Preoperative alpha blockade for normotensive pheochromocytoma. J Hypertens. 2011; 29(12):2429-2432. doi: 10.1097/HJH.0b013e32834d24d9.Jankovic RJ, Konstantinovic SM, Milic DJ, et al. Can a patient be successfully prepared for pheochromocytoma surgery in three days? A case report. Minerva Anestesiol. 2007; 73:245-248.Jankovic RJ, Konstantinovic SM, Milic DJ, et al. Can a patient be successfully prepared for pheochromocytoma surgery in three days? A case report. Minerva Anestesiol. 2007; 73:245-248.Agarwal G, Sadacharan D, Aggarwal V, et al. Surgical management of organcontained unilateral pheochromocytoma: Comparative outcomes of laparoscopic and conventional open surgical procedures in a large singleinstitution series. Langenbecks Arch Surg. 2011;397(7):1109-1116. doi: 10.1007/s00423-011-0879-3.Agarwal G, Sadacharan D, Aggarwal V, et al. Surgical management of organcontained unilateral pheochromocytoma: Comparative outcomes of laparoscopic and conventional open surgical procedures in a large singleinstitution series. Langenbecks Arch Surg. 2011;397(7):1109-1116. doi: 10.1007/s00423-011-0879-3.Goers TA, Abdo M, Moley JF, et al. Outcomes of resection of extraadrenal pheochromocytomas/paragangliomas in the laparoscopic era: A comparison with adrenal pheochromocytoma. Surg Endosc. 2012;27(2):428-433. doi: 10.1007/s00464-012-2451-9.Goers TA, Abdo M, Moley JF, et al. Outcomes of resection of extraadrenal pheochromocytomas/paragangliomas in the laparoscopic era: A comparison with adrenal pheochromocytoma. Surg Endosc. 2012;27(2):428-433. doi: 10.1007/s00464-012-2451-9.Alesina PF, Hinrichs J, Meier B, et al. Minimally invasive cortical-sparing surgery for bilateral pheochromocytomas. Langenbecks Arch Surg. 2011;397(2):233-238. doi: 10.1007/s00423-011-0851-2.Alesina PF, Hinrichs J, Meier B, et al. Minimally invasive cortical-sparing surgery for bilateral pheochromocytomas. Langenbecks Arch Surg. 2011;397(2):233-238. doi: 10.1007/s00423-011-0851-2.Amar L, Fassnacht M, Gimenez-Roqueplo AP, et al. Long-term postoperative follow-up in patients with apparently benign pheochromocytoma and paraganglioma. Horm Metab Res. 2012;44(05):385-389. doi: 10.1055/s-0031-1301339.Amar L, Fassnacht M, Gimenez-Roqueplo AP, et al. Long-term postoperative follow-up in patients with apparently benign pheochromocytoma and paraganglioma. Horm Metab Res. 2012;44(05):385-389. doi: 10.1055/s-0031-1301339.

The authors declare that there are no conflicts of interest present.