Encyclopedia of Cancer

Living Edition
| Editors: Manfred Schwab


  • Amal Alhefdhi
  • Herbert Chen
Living reference work entry
DOI: https://doi.org/10.1007/978-3-642-27841-9_4519-7



Pheochromocytomas are rare neuroendocrine tumors. The name phios (dusky), chroma (color), and cytoma (tumor) refers to the color of the tumor cells when stained with chromium salts. Catecholamine-producing neuroendocrine cells are consequently known as chromaffin cells. Some authors restrict the use of pheochromocytoma to tumors of the adrenal medulla, while others use the term more broadly to include paraganglioma. The defining characteristic of pheochromocytoma in clinical practice is the autonomous production of catecholamines (Whiting and Doogue 2009). Sympathetic paraganglial tissue (chromaffin) is mainly located in the adrenal medulla but also in prevertebral and paravertebral thoracoabdominal and pelvic paraganglia or ganglia in the ovary, testis, vagina, urethra, prostate, bladder or liver, and the Zuckerkandl organ (Erlic and Neumann 2009).



Pheochromocytoma is a rare tumor with an approximate incidence of two to...


Multiple Endocrine Neoplasia Type Renal Clear Cell Carcinoma Adrenal Incidentaloma Malignant Pheochromocytoma Adrenal Pheochromocytoma 
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  1. Adler JT, Meyer-Rochow GY, Chen H et al (2008) Pheochromocytoma: current approaches and future directions. Oncologist 13:779–793CrossRefPubMedGoogle Scholar
  2. Anagnostis P, Karagiannis A, Tziomalos K et al (2009) Adrenal incidentaloma: a diagnostic challenge. Hormones 8(3):163–184CrossRefPubMedGoogle Scholar
  3. Chen H, Sippel RS, O’Dorisio MS, Vinik AI, Lloyd RV, Pacak K. The NANETS consensus guidelines for the diagnosis and management of neuroendocrine tumors: pheochromocytoma, paraganglioma, and medullary thyroid cancer. Pancreas (in press)Google Scholar
  4. Erlic Z, Neumann HP (2009) Familial pheochromocytoma. Hormones 8(1):29–38CrossRefPubMedGoogle Scholar
  5. Krawczyk A, Hasse-Lazar K, Pawlaczek A et al (2010) Germinal mutations of RET, SDHB, SDHD, and VHL genes in patients with apparently sporadic pheochromocytomas and paragangliomas. Endokrynol Pol/Pol J Endocrinol 61(1):43–48Google Scholar
  6. Lim YH, Rhee WJ, Choi SR et al (2011) Intraoperative hypertension in patient with undiagnosed pheochromocytoma under spinal anesthesia. Korean J Anesthesiol 61(5):439–440CrossRefPubMedPubMedCentralGoogle Scholar
  7. Mannelli M, Castellano M, Schiavi F (2009) Clinically guided genetic screening in a large cohort of Italian patients with pheochromocytomas and/or functional or nonfunctional paragangliomas. J Clin Endocrinol Metab 94(5):1541–1547CrossRefPubMedGoogle Scholar
  8. Whiting MJ, Doogue MP (2009) Advances in biochemical screening for pheochromocytoma using biogenic amines. Clin Biochem Rev 30:3–17PubMedPubMedCentralGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 2014

Authors and Affiliations

  1. 1.Department of SurgeryUniversity of WisconsinMadisonUSA
  2. 2.Department of SurgeryUniversity of Alabama - Birmingham (UAB) School of Medicine Surgeon-in-Chief, UAB Hospital and Health System Senior Advisor, University of Alabama Comprehensive Cancer CenterBirminghamUK