Pheochromocytoma

Pheochromocytoma
Other namesPhaeochromocytoma, adrenal medullary tumor, Chromaffin Cell Tumors, Paraganglioma
Normal remnant adrenal gland (left) with a pheochromocytoma (right) involving the adrenal medulla
Pronunciation
SpecialtyEndocrinology, oncology
SymptomsHypertension, tachycardia, sweating, headache, pallor
ComplicationsHypertensive crisis
Causes80% Genetic[2]
Diagnostic methodElevated plasma free metanephrines, plasma catecholamines, urinary catecholamines, MRI, PET Scan
TreatmentSurgery, chemotherapy, radiation, and pharmacologic agents
MedicationAlpha blocker Doxazosin Prazosin Terazosin
Frequency0.8 per 100,000 person-years [3]

Pheochromocytoma is a rare tumor of the adrenal medulla composed of chromaffin cells and is part of the paraganglioma (PGL) family of tumors, being defined as an intra-adrenal PGL .[2][4][5] These neuroendocrine tumors can be sympathetic, where they release catecholamines into the bloodstream which cause the most common symptoms, including hypertension (high blood pressure), tachycardia (fast heart rate), sweating, and headaches.[6][7] Some PGLs may secrete little to no catecholamines, or only secrete paroxysmally (episodically), and other than secretions, PGLs can still become clinically relevant through other secretions or mass effect (most common with head and neck PGL).[8] PGLs of the head and neck are typically parasympathetic and their sympathetic counterparts are predominantly located in the abdomen and pelvis, particularly concentrated at the organ of Zuckerkandl at the bifurcation of the aorta.[9]

  1. ^ "PHAEOCHROMOCYTOMA | Meaning & Definition for UK English". Lexico.com. Archived from the original on February 25, 2021. Retrieved 2022-08-24.
  2. ^ a b Gimenez-Roqueplo AP, Robledo M, Dahia PL (April 2023). "Update on the genetics of paragangliomas". Endocr Relat Cancer. 30 (4): e220373. doi:10.1530/ERC-22-0373. PMC 10029328. PMID 36748842.
  3. ^ Beard CM, Sheps SG, Kurland LT, Carney JA, Lie JT (December 1983). "Occurrence of pheochromocytoma in Rochester, Minnesota, 1950 through 1979". Mayo Clinic Proceedings. 58 (12): 802–4. PMID 6645626.
  4. ^ Lenders JW, Eisenhofer G, Mannelli M, Pacak K (20–26 August 2005). "Phaeochromocytoma". Lancet. 366 (9486): 665–75. doi:10.1016/S0140-6736(05)67139-5. PMID 16112304. S2CID 208788653.
  5. ^ Oyasu R, Yang XJ, Yoshida O, eds. (2008). "What is the difference between pheochromocytoma and paraganglioma? What are the familial syndromes that have pheochromocytoma as a component? What are the pathologic features of pheochromocytoma indicating malignancy?". Questions in Daily Urologic Practice: Updates for Urologists and Diagnostic Pathologists. Tokyo: Springer Japan. pp. 280–4. doi:10.1007/978-4-431-72819-1_49. ISBN 978-4-431-72819-1.
  6. ^ Lenders JW, Pacak K, Walther MM, Linehan WM, Mannelli M, Friberg P, et al. (March 2002). "Biochemical diagnosis of pheochromocytoma: which test is best?". JAMA. 287 (11): 1427–34. doi:10.1001/jama.287.11.1427. PMID 11903030.
  7. ^ "Internal Medicine". JAMA. 286 (8): 971. 2001-08-22. doi:10.1001/jama.286.8.971-jbk0822-2-1. ISSN 0098-7484.
  8. ^ Williams MD (September 2017). "Paragangliomas of the Head and Neck: An Overview from Diagnosis to Genetics". Head Neck Pathol. 11 (3): 278–287. doi:10.1007/s12105-017-0803-4. PMC 5550402. PMID 28321772.
  9. ^ Kellerman RD, Rakel D (2020). Conn's Current Therapy. Elsevier–Health Science. ISBN 978-0-323-79006-2. OCLC 1145315791.