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Adrenal pheochromocytoma

MedGen UID:
1636437
Concept ID:
C4551683
Neoplastic Process
Synonym: Adrenal gland pheochromocytoma
SNOMED CT: Chromaffin tumor (302835009); Chromaffinoma (302835009); Chromaffin paraganglioma (302835009); Pheochromocytoma (85583005); Chromaffin paraganglioma (85583005); Chromaffin tumor (85583005); Chromaffinoma (85583005); Adrenal medullary paraganglioma (85583005); Adrenal medullary paraganglioma (302835009); Chromaffin cell neoplasm (85583005); Pheochromocytoma (302835009)
 
HPO: HP:0006748
Monarch Initiative: MONDO:0004974

Definition

Pheochromocytoma originating from the adrenal medulla. [from HPO]

Conditions with this feature

Paragangliomas 3
MedGen UID:
340200
Concept ID:
C1854336
Disease or Syndrome
Hereditary paraganglioma-pheochromocytoma (PGL/PCC) syndromes are characterized by paragangliomas (tumors that arise from neuroendocrine tissues distributed along the paravertebral axis from the base of the skull to the pelvis) and pheochromocytomas (paragangliomas that are confined to the adrenal medulla). Sympathetic paragangliomas cause catecholamine excess; parasympathetic paragangliomas are most often nonsecretory. Extra-adrenal parasympathetic paragangliomas are located predominantly in the skull base and neck (referred to as head and neck PGL [HNPGL]) and sometimes in the upper mediastinum; approximately 95% of such tumors are nonsecretory. In contrast, sympathetic extra-adrenal paragangliomas are generally confined to the lower mediastinum, abdomen, and pelvis, and are typically secretory. Pheochromocytomas, which arise from the adrenal medulla, typically lead to catecholamine excess. Symptoms of PGL/PCC result from either mass effects or catecholamine hypersecretion (e.g., sustained or paroxysmal elevations in blood pressure, headache, episodic profuse sweating, forceful palpitations, pallor, and apprehension or anxiety). The risk for developing metastatic disease is greater for extra-adrenal sympathetic paragangliomas than for pheochromocytomas.
Paragangliomas 4
MedGen UID:
349380
Concept ID:
C1861848
Disease or Syndrome
Hereditary paraganglioma-pheochromocytoma (PGL/PCC) syndromes are characterized by paragangliomas (tumors that arise from neuroendocrine tissues distributed along the paravertebral axis from the base of the skull to the pelvis) and pheochromocytomas (paragangliomas that are confined to the adrenal medulla). Sympathetic paragangliomas cause catecholamine excess; parasympathetic paragangliomas are most often nonsecretory. Extra-adrenal parasympathetic paragangliomas are located predominantly in the skull base and neck (referred to as head and neck PGL [HNPGL]) and sometimes in the upper mediastinum; approximately 95% of such tumors are nonsecretory. In contrast, sympathetic extra-adrenal paragangliomas are generally confined to the lower mediastinum, abdomen, and pelvis, and are typically secretory. Pheochromocytomas, which arise from the adrenal medulla, typically lead to catecholamine excess. Symptoms of PGL/PCC result from either mass effects or catecholamine hypersecretion (e.g., sustained or paroxysmal elevations in blood pressure, headache, episodic profuse sweating, forceful palpitations, pallor, and apprehension or anxiety). The risk for developing metastatic disease is greater for extra-adrenal sympathetic paragangliomas than for pheochromocytomas.
Paragangliomas 1
MedGen UID:
488134
Concept ID:
C3494181
Neoplastic Process
Hereditary paraganglioma-pheochromocytoma (PGL/PCC) syndromes are characterized by paragangliomas (tumors that arise from neuroendocrine tissues distributed along the paravertebral axis from the base of the skull to the pelvis) and pheochromocytomas (paragangliomas that are confined to the adrenal medulla). Sympathetic paragangliomas cause catecholamine excess; parasympathetic paragangliomas are most often nonsecretory. Extra-adrenal parasympathetic paragangliomas are located predominantly in the skull base and neck (referred to as head and neck PGL [HNPGL]) and sometimes in the upper mediastinum; approximately 95% of such tumors are nonsecretory. In contrast, sympathetic extra-adrenal paragangliomas are generally confined to the lower mediastinum, abdomen, and pelvis, and are typically secretory. Pheochromocytomas, which arise from the adrenal medulla, typically lead to catecholamine excess. Symptoms of PGL/PCC result from either mass effects or catecholamine hypersecretion (e.g., sustained or paroxysmal elevations in blood pressure, headache, episodic profuse sweating, forceful palpitations, pallor, and apprehension or anxiety). The risk for developing metastatic disease is greater for extra-adrenal sympathetic paragangliomas than for pheochromocytomas.

Professional guidelines

PubMed

Laks S, van Leeuwaarde R, Patel D, Keutgen XM, Hammel P, Nilubol N, Links TP, Halfdanarson TR, Daniels AB, Tirosh A; Pancreatic Manifestations Recommendations Development Subcommittee of the VHL Alliance
Cancer 2022 Feb 1;128(3):435-446. Epub 2021 Nov 4 doi: 10.1002/cncr.33978. PMID: 34735022
Guo Z, Lloyd RV
Adv Anat Pathol 2015 Sep;22(5):283-93. doi: 10.1097/PAP.0000000000000086. PMID: 26262510
Disick GI, Palese MA
Curr Urol Rep 2007 Jan;8(1):83-8. doi: 10.1007/s11934-007-0025-5. PMID: 17239321

Curated

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Neuroendocrine and Adrenal Tumors, 2023

Recent clinical studies

Etiology

Sada A, McKenzie TJ
Curr Opin Endocrinol Diabetes Obes 2023 Jun 1;30(3):161-166. Epub 2023 Apr 14 doi: 10.1097/MED.0000000000000810. PMID: 37057653
Wachtel H, Hutchens T, Baraban E, Schwartz LE, Montone K, Baloch Z, LiVolsi V, Krumeich L, Fraker DL, Nathanson KL, Cohen DL, Fishbein L
J Clin Endocrinol Metab 2020 Dec 1;105(12):e4661-70. doi: 10.1210/clinem/dgaa608. PMID: 32877928Free PMC Article
Koh JM, Ahn SH, Kim H, Kim BJ, Sung TY, Kim YH, Hong SJ, Song DE, Lee SH
PLoS One 2017;12(11):e0187398. Epub 2017 Nov 8 doi: 10.1371/journal.pone.0187398. PMID: 29117221Free PMC Article
Nozaki T, Iida H, Morii A, Fujiuchi Y, Okumura A, Fuse H
J Endourol 2013 Jul;27(7):862-8. Epub 2013 Jun 6 doi: 10.1089/end.2012.0745. PMID: 23469777
Opocher G, Schiavi F, Iacobone M, Toniato A, Sattarova S, Erlic Z, Martella M, Mian C, Merante Boschin I, Zambonin L, De Lazzari P, Murgia A, Pelizzo MR, Favia G, Mantero F
Ann N Y Acad Sci 2006 Aug;1073:149-55. doi: 10.1196/annals.1353.015. PMID: 17102081

Diagnosis

Szatko A, Glinicki P, Gietka-Czernel M
Front Endocrinol (Lausanne) 2023;14:1204851. Epub 2023 Jul 13 doi: 10.3389/fendo.2023.1204851. PMID: 37522121Free PMC Article
Ambrosini V, Kunikowska J, Baudin E, Bodei L, Bouvier C, Capdevila J, Cremonesi M, de Herder WW, Dromain C, Falconi M, Fani M, Fanti S, Hicks RJ, Kabasakal L, Kaltsas G, Lewington V, Minozzi S, Cinquini M, Öberg K, Oyen WJG, O'Toole D, Pavel M, Ruszniewski P, Scarpa A, Strosberg J, Sundin A, Taïeb D, Virgolini I, Wild D, Herrmann K, Yao J
Eur J Cancer 2021 Mar;146:56-73. Epub 2021 Feb 12 doi: 10.1016/j.ejca.2021.01.008. PMID: 33588146Free PMC Article
Wachtel H, Hutchens T, Baraban E, Schwartz LE, Montone K, Baloch Z, LiVolsi V, Krumeich L, Fraker DL, Nathanson KL, Cohen DL, Fishbein L
J Clin Endocrinol Metab 2020 Dec 1;105(12):e4661-70. doi: 10.1210/clinem/dgaa608. PMID: 32877928Free PMC Article
Tevosian SG, Ghayee HK
Endocrinol Metab Clin North Am 2019 Dec;48(4):727-750. doi: 10.1016/j.ecl.2019.08.006. PMID: 31655773
Whalen RK, Althausen AF, Daniels GH
J Urol 1992 Jan;147(1):1-10. doi: 10.1016/s0022-5347(17)37119-7. PMID: 1729490

Therapy

Gu J, Yan C, Yin S, Wu H, Liu C, Xue A, Lei X, Zhang N, Geng F
J Control Release 2024 Feb;366:448-459. Epub 2024 Jan 11 doi: 10.1016/j.jconrel.2023.12.030. PMID: 38128884
Ambrosini V, Kunikowska J, Baudin E, Bodei L, Bouvier C, Capdevila J, Cremonesi M, de Herder WW, Dromain C, Falconi M, Fani M, Fanti S, Hicks RJ, Kabasakal L, Kaltsas G, Lewington V, Minozzi S, Cinquini M, Öberg K, Oyen WJG, O'Toole D, Pavel M, Ruszniewski P, Scarpa A, Strosberg J, Sundin A, Taïeb D, Virgolini I, Wild D, Herrmann K, Yao J
Eur J Cancer 2021 Mar;146:56-73. Epub 2021 Feb 12 doi: 10.1016/j.ejca.2021.01.008. PMID: 33588146Free PMC Article
Zhang W, Shi YB, Zhuang ZX, Wang JP, Sun LJ, Fu YF
Surg Laparosc Endosc Percutan Tech 2019 Oct;29(5):409-412. doi: 10.1097/SLE.0000000000000677. PMID: 31107857
Araki S, Kijima T, Waseda Y, Komai Y, Nakanishi Y, Uehara S, Yasuda Y, Yoshida S, Yokoyama M, Ishioka J, Matsuoka Y, Saito K, Kihara K, Nakano Y, Yoshimoto T, Uchida T, Fujii Y
Int J Urol 2019 Feb;26(2):273-277. Epub 2018 Nov 22 doi: 10.1111/iju.13864. PMID: 30467902
Hirai H, Midorikawa S, Suzuki S, Sasano H, Watanabe T, Satoh H
Intern Med 2016;55(20):2985-2991. Epub 2016 Oct 15 doi: 10.2169/internalmedicine.55.7071. PMID: 27746437Free PMC Article

Prognosis

Szatko A, Glinicki P, Gietka-Czernel M
Front Endocrinol (Lausanne) 2023;14:1204851. Epub 2023 Jul 13 doi: 10.3389/fendo.2023.1204851. PMID: 37522121Free PMC Article
Wachtel H, Hutchens T, Baraban E, Schwartz LE, Montone K, Baloch Z, LiVolsi V, Krumeich L, Fraker DL, Nathanson KL, Cohen DL, Fishbein L
J Clin Endocrinol Metab 2020 Dec 1;105(12):e4661-70. doi: 10.1210/clinem/dgaa608. PMID: 32877928Free PMC Article
Koh JM, Ahn SH, Kim H, Kim BJ, Sung TY, Kim YH, Hong SJ, Song DE, Lee SH
PLoS One 2017;12(11):e0187398. Epub 2017 Nov 8 doi: 10.1371/journal.pone.0187398. PMID: 29117221Free PMC Article
Nozaki T, Iida H, Morii A, Fujiuchi Y, Okumura A, Fuse H
J Endourol 2013 Jul;27(7):862-8. Epub 2013 Jun 6 doi: 10.1089/end.2012.0745. PMID: 23469777
Whalen RK, Althausen AF, Daniels GH
J Urol 1992 Jan;147(1):1-10. doi: 10.1016/s0022-5347(17)37119-7. PMID: 1729490

Clinical prediction guides

Chung R, O'Shea A, Sweeney AT, Mercaldo ND, McDermott S, Blake MA
AJR Am J Roentgenol 2022 Jul;219(1):97-109. Epub 2022 Jan 26 doi: 10.2214/AJR.21.26918. PMID: 35080458
Wachtel H, Hutchens T, Baraban E, Schwartz LE, Montone K, Baloch Z, LiVolsi V, Krumeich L, Fraker DL, Nathanson KL, Cohen DL, Fishbein L
J Clin Endocrinol Metab 2020 Dec 1;105(12):e4661-70. doi: 10.1210/clinem/dgaa608. PMID: 32877928Free PMC Article
Zhang W, Shi YB, Zhuang ZX, Wang JP, Sun LJ, Fu YF
Surg Laparosc Endosc Percutan Tech 2019 Oct;29(5):409-412. doi: 10.1097/SLE.0000000000000677. PMID: 31107857
Koh JM, Ahn SH, Kim H, Kim BJ, Sung TY, Kim YH, Hong SJ, Song DE, Lee SH
PLoS One 2017;12(11):e0187398. Epub 2017 Nov 8 doi: 10.1371/journal.pone.0187398. PMID: 29117221Free PMC Article
Nozaki T, Iida H, Morii A, Fujiuchi Y, Okumura A, Fuse H
J Endourol 2013 Jul;27(7):862-8. Epub 2013 Jun 6 doi: 10.1089/end.2012.0745. PMID: 23469777

Supplemental Content

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    Curated

    • NCCN, 2023
      NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines®), Neuroendocrine and Adrenal Tumors, 2023

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