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Extraadrenal pheochromocytoma

MedGen UID:
263453
Concept ID:
C1257877
Neoplastic Process
Synonyms: Extra-Adrenal Pheochromocytoma; Extra-Adrenal Pheochromocytomas; Pheochromocytoma, Extra Adrenal; Pheochromocytoma, Extra-Adrenal; Pheochromocytomas, Extra-Adrenal
 
HPO: HP:0006737
Monarch Initiative: MONDO:0000550

Definition

Pheochromocytoma not originating from the adrenal medulla but from another source such as from chromaffin cells in or about sympathetic ganglia. [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

Rattenberry E, Vialard L, Yeung A, Bair H, McKay K, Jafri M, Canham N, Cole TR, Denes J, Hodgson SV, Irving R, Izatt L, Korbonits M, Kumar AV, Lalloo F, Morrison PJ, Woodward ER, Macdonald F, Wallis Y, Maher ER
J Clin Endocrinol Metab 2013 Jul;98(7):E1248-56. Epub 2013 May 10 doi: 10.1210/jc.2013-1319. PMID: 23666964

Recent clinical studies

Etiology

Vaze D, Trehan A, Saxena A, Joshi K, Narasimhan KL
Urology 2012 Oct;80(4):925-7. Epub 2012 Aug 15 doi: 10.1016/j.urology.2012.06.013. PMID: 22901822
Chen L, Li F, Zhuang H, Jing H, Du Y, Zeng Z
J Nucl Med 2009 Mar;50(3):397-400. Epub 2009 Feb 17 doi: 10.2967/jnumed.108.058693. PMID: 19223405
Phitayakorn R, McHenry CR
Am Surg 2008 Jan;74(1):37-42. PMID: 18274426
Kazaryan AM, Kuznetsov NS, Shulutko AM, Beltsevich DG, Edwin B
Surg Endosc 2004 Jun;18(6):937-41. Epub 2004 Apr 27 doi: 10.1007/s00464-003-9199-1. PMID: 15108109
Base J, Sváb J
Sb Ved Pr Lek Fak Karlovy Univerzity Hradci Kralove 1995;38(1):47-9. PMID: 7569727

Diagnosis

Rattenberry E, Vialard L, Yeung A, Bair H, McKay K, Jafri M, Canham N, Cole TR, Denes J, Hodgson SV, Irving R, Izatt L, Korbonits M, Kumar AV, Lalloo F, Morrison PJ, Woodward ER, Macdonald F, Wallis Y, Maher ER
J Clin Endocrinol Metab 2013 Jul;98(7):E1248-56. Epub 2013 May 10 doi: 10.1210/jc.2013-1319. PMID: 23666964
Vaze D, Trehan A, Saxena A, Joshi K, Narasimhan KL
Urology 2012 Oct;80(4):925-7. Epub 2012 Aug 15 doi: 10.1016/j.urology.2012.06.013. PMID: 22901822
Qiao HS, Feng XL, Yong L, Yong Z, Lian ZJ, Ling LB
Eur J Radiol 2007 Jun;62(3):335-41. Epub 2007 Apr 3 doi: 10.1016/j.ejrad.2007.02.041. PMID: 17408898
Elsayes KM, Narra VR, Leyendecker JR, Francis IR, Lewis JS Jr, Brown JJ
AJR Am J Roentgenol 2005 Mar;184(3):860-7. doi: 10.2214/ajr.184.3.01840860. PMID: 15728610
Naguib M, Caceres M, Thomas CR Jr, Herman TS, Eng TY
Am J Clin Oncol 2002 Feb;25(1):42-4. doi: 10.1097/00000421-200202000-00008. PMID: 11823694

Therapy

Thomaschewski M, Neeff H, Keck T, Neumann HPH, Strate T, von Dobschuetz E
Rev Endocr Metab Disord 2017 Dec;18(4):443-457. doi: 10.1007/s11154-017-9436-x. PMID: 29127554
Qiao HS, Feng XL, Yong L, Yong Z, Lian ZJ, Ling LB
Eur J Radiol 2007 Jun;62(3):335-41. Epub 2007 Apr 3 doi: 10.1016/j.ejrad.2007.02.041. PMID: 17408898
Kazaryan AM, Kuznetsov NS, Shulutko AM, Beltsevich DG, Edwin B
Surg Endosc 2004 Jun;18(6):937-41. Epub 2004 Apr 27 doi: 10.1007/s00464-003-9199-1. PMID: 15108109
Tohya T, Yoshimura T, Honda Y, Miyoshi J, Okamura H
Eur J Obstet Gynecol Reprod Biol 1999 Feb;82(2):217-8. doi: 10.1016/s0301-2115(98)00260-7. PMID: 10206419
Schlumberger M, Gicquel C, Lumbroso J, Tenenbaum F, Comoy E, Bosq J, Fonseca E, Ghillani PP, Aubert B, Travagli JP
J Endocrinol Invest 1992 Oct;15(9):631-42. doi: 10.1007/BF03345807. PMID: 1479146

Prognosis

Sakamaki Y, Yasukawa M, Kido T
Gen Thorac Cardiovasc Surg 2008 Oct;56(10):509-11. Epub 2008 Oct 15 doi: 10.1007/s11748-008-0282-3. PMID: 18854928
Qiao HS, Feng XL, Yong L, Yong Z, Lian ZJ, Ling LB
Eur J Radiol 2007 Jun;62(3):335-41. Epub 2007 Apr 3 doi: 10.1016/j.ejrad.2007.02.041. PMID: 17408898
Iijima T, Iwao Y, Ito Y
J Pediatr Surg 2006 Aug;41(8):e15-7. doi: 10.1016/j.jpedsurg.2006.04.007. PMID: 16863830
Naguib M, Caceres M, Thomas CR Jr, Herman TS, Eng TY
Am J Clin Oncol 2002 Feb;25(1):42-4. doi: 10.1097/00000421-200202000-00008. PMID: 11823694
Schlumberger M, Gicquel C, Lumbroso J, Tenenbaum F, Comoy E, Bosq J, Fonseca E, Ghillani PP, Aubert B, Travagli JP
J Endocrinol Invest 1992 Oct;15(9):631-42. doi: 10.1007/BF03345807. PMID: 1479146

Clinical prediction guides

Phitayakorn R, McHenry CR
Am Surg 2008 Jan;74(1):37-42. PMID: 18274426
Qiao HS, Feng XL, Yong L, Yong Z, Lian ZJ, Ling LB
Eur J Radiol 2007 Jun;62(3):335-41. Epub 2007 Apr 3 doi: 10.1016/j.ejrad.2007.02.041. PMID: 17408898
Benn DE, Gimenez-Roqueplo AP, Reilly JR, Bertherat J, Burgess J, Byth K, Croxson M, Dahia PL, Elston M, Gimm O, Henley D, Herman P, Murday V, Niccoli-Sire P, Pasieka JL, Rohmer V, Tucker K, Jeunemaitre X, Marsh DJ, Plouin PF, Robinson BG
J Clin Endocrinol Metab 2006 Mar;91(3):827-36. Epub 2005 Nov 29 doi: 10.1210/jc.2005-1862. PMID: 16317055
Kazaryan AM, Kuznetsov NS, Shulutko AM, Beltsevich DG, Edwin B
Surg Endosc 2004 Jun;18(6):937-41. Epub 2004 Apr 27 doi: 10.1007/s00464-003-9199-1. PMID: 15108109
Schlumberger M, Gicquel C, Lumbroso J, Tenenbaum F, Comoy E, Bosq J, Fonseca E, Ghillani PP, Aubert B, Travagli JP
J Endocrinol Invest 1992 Oct;15(9):631-42. doi: 10.1007/BF03345807. PMID: 1479146

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