Positions at NIH
- 1990-1995: Visiting Fellow, National Institute of Neurological Disorders and Stroke (NINDS), NIH
- 1997-1999: Clinical Associate, Inter-Institute Endocrinology Training Program in Endocrinology, Diabetes, and Metabolism, NICHD, NIH
- 1990-200: Senior Staff Fellow, NICHD, NIH
- 2000-2005: Chief, Unit on Clinical Neuroendocrinology, tenure-track, NICHD, NIH
- 2005-2024: Chief, Senior Investigator, Section on Medical Neuroendocrinology, tenured, NICHD, NIH
- 2015-2019: Head, Developmental Endocrinology and Tumor Genetics Affinity Group, NICHD, NIH
- 2019-2024: Head, Developmental Endocrinology, Metabolism, Genetics, and Endocrine Oncology Affinity Group, NICHD, NIH
- 2024-present: Scientist Emeritus, NICHD, NIH
Biography
Karel Pacak graduated summa cum laude from Charles University in the Czech Republic, where he received his M.D., Ph.D., and D.Sc. degrees (and the last two degrees were based on scientific work at NIH). He established a nationally and internationally recognized patient-oriented pheochromocytoma and paraganglioma research program at NICHD in Bethesda, MD, where his laboratory had major findings and publications over the past 25 years.
His early scientific career focused on refuting the Selye’s theory of stressor non-specificity and describing stressor-specific pathways in the brain. After finishing his residency in Internal Medicine and a fellowship in Endocrinology, Diabetes, and Metabolism, he turned his clinical and scientific career towards studying chromaffin cell tumors, focusing on pheochromocytoma and paraganglioma. Later, Dr. Pacak contributed to discoveries related to patients with neuroendocrine tumors, specifically pheochromocytoma and paraganglioma. By establishing major initiatives for chromaffin cell tumors worldwide based on his work at NICHD, Dr. Pacak made a significant impact on the diagnosis and treatment of patients with these tumors.
Research Accomplishments
Together with other colleagues, Dr. Pacak pioneered the current biochemical and imaging diagnoses for pheochromocytoma and paraganglioma, implementing plasma metanephrines and 3-methoxytyramine, as well as 18F-fluorodopamine and 68Ga-DOTATATE positron emission tomography, into clinical practice. His team discovered new HIF2A, PHD1, IRP1, and SUCLG2 gene mutations that play an important role in the pathogenesis of these tumors. Together with Dr. Z. Zhuang, a new syndrome of paraganglioma and somatostatinoma associated with polycythemia in females, termed Pacak-Zhuang syndrome, was discovered.
As a team effort under the Cancer Genome Atlas, Dr. Pacak also described a new Wnt-altered subtype driven by MAML3 and CSDE1 genes in pheochromocytoma and paraganglioma.
Dr. Pacak was the leading expert participating in the Pheochromocytoma Task Force of the U.S. Endocrine Society in 2014 and co-chair of the National Cancer Institute’s Cancer Genome Atlas Program study on pheochromocytoma from 2014 to 2016. He was also a co-lead of the team that launched guidelines for radionuclide imaging of pheochromocytoma and paraganglioma for the European Association of Nuclear Medicine and the Society of Nuclear Medicine and Molecular Imaging. In 2023 to 2024, he served as the co-chair for the international consensus statement/guidelines for SDHB/D pheochromocytoma and paraganglioma. Since 2023, he served as Chair of the Medical Advisory Board of the Pheo Para Alliance.
Dr. Pacak has received several awards for his clinical and scientific work: Outstanding Clinical Investigator from the American Association of Clinical Endocrinologists (2015), H. Friesen Award from the Canadian Society of Endocrinology and Metabolism (2016), Best Research Team Award by the NIH Inter-Institute Endocrinology Training Program (2018), Science Award from SDHB Coalition (2018), Purkyne Medal from the Czech Medical Society (2018), and American Association for Cancer Research Team Science Award for TCGA (2020). In 2022, Dr. Pacak received the Outstanding Clinical Investigator Award from the U.S. Endocrine Society and Frontiers in Science and Distinction in Endocrinology Award from AACE. In 2023, he presented the Annual Irene Tamagna Lecture in Hypertension at George Washington University, Washington D.C. In 2024, he gave the Thompson Lecture from Baylor Scott & White Health, and the Basic Science Lecture from the European Society of Endocrine Surgeons. In 2024, he was elected to the Learned Society of the Czech Republic.
Selected Publications
- Pacak, K., & Palkovits, M. (2001). Stressor specificity of central neuroendocrine responses: Implications for stress-related Disorders. Endocr. Rev. 22:502-548. PMID: 11493581
- Lenders, J., Pacak, K., Walther, M., Linehan, W., Mannelli, M., Friberg, P. et al. (2002). Biochemical diagnosis of pheochromocytoma: which test is best? JAMA 287:1427. PMID: 11903030
- Lenders, J., Eisenhofer, G., Mannelli, M., & Pacak, K. (2005). Phaeochromocytoma. Lancet, 366:665 PMID: 16112304
- Eisenhofer, G., Lenders, J., Siegert, G., Bornstein, S., Friberg, P., Milosevic, D.,…Pacak, K. (2012). Plasma methoxytyramine: A novel biomarker of metastatic pheochromocytoma and paraganglioma in relation to established risk factors of tumour size, location and SDHB mutation status. Eur. J Cancer 48:1739. PMID: 22036874
- Zhuang, Z., Yang, C., Lorenzo, F., Merino, M., Fojo, T., Kebebew, E.,…Pacak, K. (2012). Somatic HIF2A gain-of-function mutations in paraganglioma with polycythemia. New Engl. J. Med. 367:922. PMID: 22931260
- Pacak, K., Jochmanova, I., Prodanov, T., Yang, C., Merino, M., & Fojo, T.,…Pacak, K. (2013). New syndrome of paraganglioma and somatostatinoma associated with polycythemia. J. Clin. Oncol. 31:1690. PMID: 23509317
- Lenders, J., Duh, Q., Eisenhofer, G., Gimenez-Roqueplo, A., Grebe, S., Murad, M., Pacak, K. et al. (2014). Pheochromocytoma and Paraganglioma: An Endocrine Society Clinical Practice Guideline. J Clin. Endocrinol. Metab. 99:1915. PMID: 24893135
- Janssen, I., Blanchet, E., Adams, K., Chen, C., Millo, C., Herscovitch, P.…Pacak, K. (2015). Superiority of [68Ga]-DOTATATE PET/CT to other functional imaging modalities in the localization of SDHB-associated metastatic pheochromocytoma and paraganglioma. Clin. Cancer Res. 21:3888. PMID: 25873086
- Fishbein, L., Leshchiner, I., Walter, V., Danilova, L., Robertson, G., Johnson, A. et al. (2017). Comprehensive molecular characterization of pheochromocytoma and paraganglioma. Cancer Cell 31;181. (TCGA initiative: Chairs: Pacak, K., Nathanson, K., Wilkerson, M.). PMID: 28162975
- Taieb, D., Hicks, R., Hindie, E., Guillet, A., Avram, A., Ghedini, P.,…Pacak, K. (2019). European Association of Nuclear Medicine Practice Guideline/Society of Nuclear Medicine and Molecular Imaging Procedure Standard 2019 for radionuclide imaging of phaechromocytoma and paraganglioma. Eur. J. Nucl. Med. Mol. Imaging 46:2112. PMID: 31254038
- Hadrava Vanova, K., Pang, Y., Krobova, L., Kraus, M., Nahacka, Z., Noukalova, S……Pacak, K. (2022). Germline SUCLG2 variants in patients with pheochromocytoma and paraganglioma. J. Natl. Cancer Inst., 114:130. PMID: 34415331
- Taieb D., Pacak, K. (2023). Clinical guideline on the management of pheochromocytoma and paraganglioma in patients harboring germline pathogenic variants in the succinate dehydrogenase subunit D gene. Lancet Diabet. Endcrinol. 11:345. PMID: 37011647
- Taieb D.,...Pacak, K. (2024). Management of phaeochromocytoma and paraganglioma in patients with germline SDHB pathogenic variants: an international expert Consensus statement. Nat. Rev. Endocrinol. 20:168. PMID: 38097671
- Uher, O., Hadrava-Vanova, K., Taieb, D., Calsina, B., Robledo, M., Clifton-Bligh, R., Pacak, K (2024). The immune landscape of pheochromocytoma and paraganglioma: Current Advances and Perspectives. Endocr. Rev. 45:521 PMID: 38377172
Access Dr. Pacak’s full publications list from the National Library of Medicine’s PubMed® database.