Feocromocitoma y Paraganglioma: Más allá de la clínica

Palabras clave: Feocromocitoma, Paraganglioma, Cirugía, Quimioterapia, Metanefrina


Los feocromocitomas y paragangliomas son tumores raros que se originan en las células cromafines. Tienen un amplio espectro clínico desde el hallazgo incidental hasta manifestaciones dadas de la producción exagerada de catecolaminas. El diagnostico bioquímico se realiza mediante medición de metanefrinas libres en plasma o metanefrinas fraccionadas en orina; se recomienda asesoría y tamización genética a todos los pacientes. El tratamiento ideal es la escisión quirúrgica completa, previo al bloqueo α y β. En caso de enfermedad metastásica las opciones de tratamiento sistémico actuales son limitadas y con escasa tasa de respuesta; está en investigación el uso de inmunoterapia.


La descarga de datos todavía no está disponible.


Lam AK. Update on Adrenal Tumours in 2017 World Health Organization (WHO) of Endocrine Tumours. Endocr Pathol. 2017 Sep;28(3):213–27.

Berends AMA, Buitenwerf E, de Krijger RR, Veeger NJGM, van der Horst-Schrivers ANA, Links TP, et al. Incidence of pheochromocytoma and sympathetic paraganglioma in the Netherlands: A nationwide study and systematic review. Eur J Intern Med [Internet]. 2018 May;51:68–73. Available from: https://linkinghub.elsevier.com/retrieve/pii/S0953620518300153

Canu L, Parenti G, De Filpo G, Mannelli M. Pheochromocytomas and Paragangliomas as Causes of Endocrine Hypertension. Front Endocrinol (Lausanne) [Internet]. 2019 Jun 4;10. Available from: https://www.frontiersin.org/article/10.3389/fendo.2019.00333/full

Fishbein L. Pheochromocytoma and Paraganglioma. Hematol Oncol Clin North Am [Internet]. 2016 Feb;30(1):135–50. Available from: https://linkinghub.elsevier.com/retrieve/pii/S0889858815001410

Hamidi O. Metastatic pheochromocytoma and paraganglioma. Curr Opin Endocrinol Diabetes Obes [Internet]. 2019 Jun;26(3):146–54. Available from: http://journals.lww.com/01266029-201906000-00005

Maher ER, Eng C. The pressure rises: update on the genetics of phaeochromocytoma. Hum Mol Genet. 2002 Oct;11(20):2347–54.

Buffet A, Venisse A, Nau V, Roncellin I, Boccio V, Le Pottier N, et al. A decade (2001-2010) of genetic testing for pheochromocytoma and paraganglioma. Hormone and Metabolic Research. 2012.

Mercado-Asis LB, Wolf KI, Jochmanova I, Taïeb D. PHEOCHROMOCYTOMA: A GENETIC AND DIAGNOSTIC UPDATE. Endocr Pract [Internet]. 2018 Jan;24(1):78–90. Available from: http://journals.aace.com/doi/10.4158/EP-2017-0057

Alrezk R, Suarez A, Tena I, Pacak K. Update of Pheochromocytoma Syndromes: Genetics, Biochemical Evaluation, and Imaging. Front Endocrinol (Lausanne) [Internet]. 2018 Nov 27;9:515. Available from: https://www.frontiersin.org/article/10.3389/fendo.2018.00515/full

Fishbein L, Leshchiner I, Walter V, Danilova L, Robertson AG, Johnson AR, et al. Comprehensive Molecular Characterization of Pheochromocytoma and Paraganglioma. Cancer Cell. 2017/02/02. 2017 Feb;31(2):181–93.

Mulligan LM, Kwok JBJ, Healey CS, Elsdon MJ, Eng C, Gardner E, et al. Germ-line mutations of the RET proto-oncogene in multiple endocrine neoplasia type 2A. Nature [Internet]. 1993 Jun;363(6428):458–60. Available from: http://www.nature.com/articles/363458a0

Wallace M, Marchuk D, Andersen L, Letcher R, Odeh H, Saulino A, et al. Type 1 neurofibromatosis gene: identification of a large transcript disrupted in three NF1 patients. Science (80- ) [Internet]. 1990 Jul 13;249(4965):181–6. Available from: https://www.sciencemag.org/lookup/doi/10.1126/science.2134734

Crona J, Taïeb D, Pacak K. New Perspectives on Pheochromocytoma and Paraganglioma: Toward a Molecular Classification. Endocr Rev [Internet]. 2017 Dec 1;38(6):489–515. Available from: http://academic.oup.com/edrv/article/38/6/489/4064267

Jochmanova I, Yang C, Zhuang Z, Pacak K. Hypoxia-Inducible Factor Signaling in Pheochromocytoma: Turning the Rudder in the Right Direction. JNCI J Natl Cancer Inst [Internet]. 2013 Sep 4;105(17):1270–83. Available from: https://academic.oup.com/jnci/article-lookup/doi/10.1093/jnci/djt201

Jochmanova I, Pacak K. Genomic Landscape of Pheochromocytoma and Paraganglioma. Trends in Cancer [Internet]. 2018 Jan;4(1):6–9. Available from: https://linkinghub.elsevier.com/retrieve/pii/S2405803317302133

Farrugia F-A, Charalampopoulos A. Pheochromocytoma. Endocr Regul [Internet]. 2019 Jul 1;53(3):191–212. Available from: https://content.sciendo.com/doi/10.2478/enr-2019-0020

Cotesta D, Petramala L, Serra V, Pergolini M, Crescenzi E, Zinnamosca L, et al. Clinical experience with pheochromocytoma in a single centre over 16 years. High Blood Press Cardiovasc Prev. 2009;

Falhammar H, Kjellman M, Calissendorff J. Initial clinical presentation and spectrum of pheochromocytoma: A study of 94 cases from a single center. Endocr Connect. 2018;

Soltani A, Pourian M, Davani BM. Does this patient have Pheochromocytoma? a systematic review of clinical signs and symptoms. J Diabetes Metab Disord [Internet]. 2016 Dec 17;15(1):6. Available from: http://jdmdonline.biomedcentral.com/articles/10.1186/s40200-016-0226-x

Fassnacht M, Arlt W, Bancos I, Dralle H, Newell-Price J, Sahdev A, et al. Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors. European Journal of Endocrinology. 2016.

Gruber LM, Hartman RP, Thompson GB, McKenzie TJ, Lyden ML, Dy BM, et al. Pheochromocytoma Characteristics and Behavior Differ Depending on Method of Discovery. J Clin Endocrinol Metab [Internet]. 2019 May 1;104(5):1386–93. Available from: https://academic.oup.com/jcem/article/104/5/1386/5185198

Cohen JK, Cisco RM, Scholten A, Mitmaker E, Duh QY. Pheochromocytoma crisis resulting in acute heart failure and cardioembolic stroke in a 37-year-old man. Surg (United States). 2014;

Ronen JA, Gavin M, Ruppert MD, Peiris AN. Glycemic Disturbances in Pheochromocytoma and Paraganglioma. Cureus [Internet]. 2019 Apr 27;4. Available from: https://www.cureus.com/articles/19474-glycemic-disturbances-in-pheochromocytoma-and-paraganglioma

Eisenhofer G, Pacak K, Huynh T-T, Qin N, Bratslavsky G, Linehan WM, et al. Catecholamine metabolomic and secretory phenotypes in phaeochromocytoma. Endocr Relat Cancer [Internet]. 2010 Dec 21;18(1):97–111. Available from: https://erc.bioscientifica.com/doi/10.1677/ERC-10-0211

Lenders JWM, Pacak K, Walther MM, Linehan WM, Mannelli M, Friberg P, et al. Biochemical Diagnosis of Pheochromocytoma. JAMA [Internet]. 2002 Mar 20;287(11). Available from: http://jama.jamanetwork.com/article.aspx?doi=10.1001/jama.287.11.1427

Lenders JWM, Duh Q-Y, Eisenhofer G, Gimenez-Roqueplo A-P, Grebe SKG, Murad MH, et al. Pheochromocytoma and Paraganglioma: An Endocrine Society Clinical Practice Guideline. J Clin Endocrinol Metab. 2014 Jun 1;99(6):1915–42.

Därr R, Kuhn M, Bode C, Bornstein SR, Pacak K, Lenders JWM, et al. Accuracy of recommended sampling and assay methods for the determination of plasma-free and urinary fractionated metanephrines in the diagnosis of pheochromocytoma and paraganglioma: a systematic review. Endocrine [Internet]. 2017 Jun;56(3):495–503. Available from: http://www.ncbi.nlm.nih.gov/pubmed/28405881

Neary NM, King KS, Pacak K. Drugs and Pheochromocytoma — Don’t Be Fooled by Every Elevated Metanephrine. N Engl J Med [Internet]. 2011 Jun 9;364(23):2268–70. Available from: http://www.nejm.org/doi/abs/10.1056/NEJMc1101502

Oleaga A, Goñi F. Feocromocitoma: Actualización diagnóstica y terapéutica. Endocrinologia y Nutricion. 2008.

McHenry CM, Hunter SJ, McCormick MT, Russell CF, Smye MG, Atkinson AB. Evaluation of the clonidine suppression test in the diagnosis of phaeochromocytoma. J Hum Hypertens. 2011;25(7):451–6.

Whalen RK, Althausen AF, Daniels GH. Extra-adrenal pheochromocytoma. Journal of Urology. 1992.

Disick GIS, Palese MA. Extra-adrenal pheochromocytoma: Diagnosis and management. Curr Urol Rep. 2007;8(1):83–8.

Jalil ND, Pattou FN, Combemale F, Chapuis Y, Henry JF, Peix JL, et al. Effectiveness and limits of preoperative imaging studies for the localisation of pheochromocytomas and paragangliomas: A review of 282 cases. Eur J Surg. 1998;

Fassnacht M, Arlt W, Bancos I, Dralle H, Newell-Price J, Sahdev A, et al. Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors. European Journal of Endocrinology. 2016.

Jimenez C, Waguespack SG. Functional imaging for pheochromocytoma–paraganglioma: a step closer to understanding its place in clinical practice. Endocrine. 2015.

Brito JP, Asi N, Gionfriddo MR, Norman C, Leppin AL, Zeballos-Palacios C, et al. The incremental benefit of functional imaging in pheochromocytoma/paraganglioma: a systematic review. Endocrine. 2015;

Lynn MD, Shapiro B, Sisson JC, Beierwaltes WH, Meyers LJ, Ackerman R, et al. Pheochromocytoma and the normal adrenal medulla: improved visualization with I-123 MIBG scintigraphy. Radiology. 1985 Jun;155(3):789–92.

Havekes B, King K, Lai EW, Romijn JA, Corssmit EPM, Pacak K. New imaging approaches to phaeochromocytomas and paragangliomas. Clin Endocrinol (Oxf). 2010 Feb;72(2):137–45.

Timmers HJLM, Brouwers FM, Hermus ARMM, Sweep FCGJ, Verhofstad AAJ, Verbeek ALM, et al. Metastases but not cardiovascular mortality reduces life expectancy following surgical resection of apparently benign pheochromocytoma. Endocr Relat Cancer. 2008;

Amar L, Servais A, Gimenez-Roqueplo A-P, Zinzindohoue F, Chatellier G, Plouin P-F. Year of Diagnosis, Features at Presentation, and Risk of Recurrence in Patients with Pheochromocytoma or Secreting Paraganglioma. J Clin Endocrinol Metab. 2005 Apr;90(4):2110–6.

Angelousi A, Kassi E, Zografos G, Kaltsas G. Metastatic pheochromocytoma and paraganglioma. Eur J Clin Invest [Internet]. 2015 Sep;45(9):986–97. Available from: http://www.ncbi.nlm.nih.gov/pubmed/26183460

Jimenez P, Tatsui C, Jessop A, Thosani S, Jimenez C. Treatment for Malignant Pheochromocytomas and Paragangliomas: 5 Years of Progress. Curr Oncol Rep. 2017 Dec 28;19(12):83.

Bausch B, Tischler AS, Schmid KW, Leijon H, Eng C, Neumann HPH. Max Schottelius: Pioneer in Pheochromocytoma. J Endocr Soc [Internet]. 2017 Jul 1;1(7):957–64. Available from: https://academic.oup.com/jes/article/1/7/957/3933560

Pacak K. Preoperative Management of the Pheochromocytoma Patient. J Clin Endocrinol Metab. 2007 Nov;92(11):4069–79.

Suzuki K. Surgical management of pheochromocytoma. Biomed Pharmacother [Internet]. 2000 Jun;54:150s-156s. Available from: https://linkinghub.elsevier.com/retrieve/pii/S0753332200800335

Walz MK, Alesina PF, Wenger FA, Koch JA, Neumann HPH, Petersenn S, et al. Laparoscopic and Retroperitoneoscopic Treatment of Pheochromocytomas and Retroperitoneal Paragangliomas: Results of 161 Tumors in 126 Patients. World J Surg [Internet]. 2006 May 17;30(5):899–908. Available from: http://link.springer.com/10.1007/s00268-005-0373-6

Duh Q-Y. Evolving Surgical Management for Patients with Pheochromocytoma. J Clin Endocrinol Metab [Internet]. 2001 Mar;86(4):1477–9. Available from: https://academic.oup.com/jcem/article-lookup/doi/10.1210/jcem.86.4.7519

Brauckhoff M, Nguyen Thanh P, B�r A, Dralle H. Subtotale bilaterale Adrenalektomie mit adrenokortikalem Funktionserhalt. Der Chir [Internet]. 2003 Jul 1;74(7):646–51. Available from: http://link.springer.com/10.1007/s00104-002-0608-0

Adjallé R, Plouin PF, Pacak K, Lehnert H. Treatment of Malignant Pheochromocytoma. Horm Metab Res [Internet]. 2009 Sep 11;41(09):687–96. Available from: http://www.thieme-connect.de/DOI/DOI?10.1055/s-0029-1231025

Jimenez C, Rohren E, Habra MA, Rich T, Jimenez P, Ayala-Ramirez M, et al. Current and Future Treatments for Malignant Pheochromocytoma and Sympathetic Paraganglioma. Curr Oncol Rep [Internet]. 2013;15(4):356–71. Available from: https://doi.org/10.1007/s11912-013-0320-x

Jimenez C, Fazeli S, Román-Gonzalez A. Antiangiogenic therapies for pheochromocytoma and paraganglioma. Endocr Relat Cancer [Internet]. 2020 Jul;27(7):R239–54. Available from: https://erc.bioscientifica.com/view/journals/erc/27/7/ERC-20-0043.xml

Sisson JC, Shapiro B, Beierwaltes WH, Glowniak J V., Nakajo M, Mangner TJ, et al. Radiopharmaceutical treatment of malignant pheochromocytoma. J Nucl Med. 1984;

Ayala-Ramirez M, Feng L, Habra MA, Rich T, Dickson P V., Perrier N, et al. Clinical benefits of systemic chemotherapy for patients with metastatic pheochromocytomas or sympathetic extra-adrenal paragangliomas. Cancer [Internet]. 2012 Jun 1;118(11):2804–12. Available from: http://doi.wiley.com/10.1002/cncr.26577

Tanabe A, Naruse M, Nomura K, Tsuiki M, Tsumagari A, Ichihara A. Combination Chemotherapy with Cyclophosphamide, Vincristine, and Dacarbazine in Patients with Malignant Pheochromocytoma and Paraganglioma. Horm Cancer [Internet]. 2013 Apr 30;4(2):103–10. Available from: http://link.springer.com/10.1007/s12672-013-0133-2

Corssmit EPM, Snel M, Kapiteijn E. Malignant pheochromocytoma and paraganglioma. Curr Opin Oncol. 2020 Jan;32(1):20–6.

Hescot S, Leboulleux S, Amar L, Vezzosi D, Borget I, Bournaud-Salinas C, et al. One-Year Progression-Free Survival of Therapy-Naive Patients With Malignant Pheochromocytoma and Paraganglioma. J Clin Endocrinol Metab. 2013 Oct;98(10):4006–12.

Jimenez C. Treatment for Patients With Malignant Pheochromocytomas and Paragangliomas: A Perspective From the Hallmarks of Cancer. Front Endocrinol (Lausanne) [Internet]. 2018 May 28;9:277. Available from: https://www.frontiersin.org/article/10.3389/fendo.2018.00277/full

van Hulsteijn LT, Niemeijer ND, Dekkers OM, Corssmit EPM. 131 I-MIBG therapy for malignant paraganglioma and phaeochromocytoma: systematic review and meta-analysis. Clin Endocrinol (Oxf) [Internet]. 2014 Apr;80(4):487–501. Available from: http://doi.wiley.com/10.1111/cen.12341

Pryma DA, Chin BB, Noto RB, Dillon JS, Perkins S, Solnes L, et al. Efficacy and safety of high-specific-activity 131I-MIBG therapy in patients with advanced pheochromocytoma or paraganglioma. J Nucl Med. 2019;60(5):623–30.

Jimenez C, Erwin W, Chasen B. Targeted radionuclide therapy for patients with metastatic pheochromocytoma and paraganglioma: From low-specific-activity to high-specific-activity iodine-131 metaiodobenzylguanidine. Cancers. 2019.

Cómo citar
Salgado Sánchez, J.C., Amado Garzón, S. y Molina Pimienta, L. 2021. Feocromocitoma y Paraganglioma: Más allá de la clínica. Revista Colombiana de Cancerología. 25, 1 (feb. 2021). DOI:https://doi.org/10.35509/01239015.586.
Artículos de revisión