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Paraganglioma(PGL1; PGL)

MedGen UID:
10571
Concept ID:
C0030421
Neoplastic Process
Synonyms: Gangliocytic Paraganglioma; Gangliocytic Paragangliomas; Paraganglioma, Gangliocytic; Paragangliomas; Paragangliomas 1; Paragangliomas, Familial, 1; Paragangliomas, Gangliocytic; Paragangliomata
SNOMED CT: Gangliocytic paraganglioma (72787006); [M]Gangliocytic paraganglioma (72787006); [M] Gangliocytic paraganglioma (72787006); Paraganglioma (803009); Neoplasm of paraganglion (127027008); Gangliocytic paraganglioma (253029009); Paraganglioma (302833002)
 
OMIM®: 168000

Definition

A rare, usually benign tumor that develops from cells of the paraganglia. Paraganglia are a collection of cells that came from embryonic nervous tissue, and are found near the adrenal glands and some blood vessels and nerves. Paragangliomas that develop in the adrenal gland are called pheochromocytomas. Those that develop outside of the adrenal glands near blood vessels or nerves are called glomus tumors or chemodectomas. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews
  • CROGParaganglioma

Recent clinical studies

Etiology

Toyoda H, Hirayama J, Sugimoto Y, Uchida K, Ohishi K, Hirayama M, Komada Y
Pediatrics 2014 Jun;133(6):e1787-91. Epub 2014 May 12 doi: 10.1542/peds.2013-2419. [Epub ahead of print] PMID: 24819565
Else T, Marvin ML, Everett JN, Gruber SB, Arts HA, Stoffel EM, Auchus RJ, Raymond VM
J Clin Endocrinol Metab 2014 Aug;99(8):E1482-6. Epub 2014 Apr 23 doi: 10.1210/jc.2013-3853. [Epub ahead of print] PMID: 24758179Free PMC Article
Hadoux J, Favier J, Scoazec JY, Leboulleux S, Al Ghuzlan A, Caramella C, Déandreis D, Borget I, Loriot C, Chougnet C, Letouzé E, Young J, Amar L, Bertherat J, Libé R, Dumont F, Deschamps F, Schlumberger M, Gimenez-Roqueplo AP, Baudin E
Int J Cancer 2014 Dec 1;135(11):2711-20. Epub 2014 May 5 doi: 10.1002/ijc.28913. [Epub ahead of print] PMID: 24752622
Phaeochromocytoma Study Group in Japan, Kimura N, Takayanagi R, Takizawa N, Itagaki E, Katabami T, Kakoi N, Rakugi H, Ikeda Y, Tanabe A, Nigawara T, Ito S, Kimura I, Naruse M
Endocr Relat Cancer 2014 Jun;21(3):405-14. Epub 2014 May 6 doi: 10.1530/ERC-13-0494. PMID: 24521857
Li Y, Guo A, Tang J, Li Q, Fei X, Zhang Y, Gao J
Ultrasound Med Biol 2014 Mar;40(3):478-84. Epub 2014 Jan 10 doi: 10.1016/j.ultrasmedbio.2013.10.014. [Epub ahead of print] PMID: 24412175

Diagnosis

Toyoda H, Hirayama J, Sugimoto Y, Uchida K, Ohishi K, Hirayama M, Komada Y
Pediatrics 2014 Jun;133(6):e1787-91. Epub 2014 May 12 doi: 10.1542/peds.2013-2419. [Epub ahead of print] PMID: 24819565
Li Y, Guo A, Tang J, Li Q, Fei X, Zhang Y, Gao J
Ultrasound Med Biol 2014 Mar;40(3):478-84. Epub 2014 Jan 10 doi: 10.1016/j.ultrasmedbio.2013.10.014. [Epub ahead of print] PMID: 24412175
Takahashi K, Yamamoto Y, Ohshima S, Morita Y, Takahashi S
Auris Nasus Larynx 2014 Feb;41(1):93-6. Epub 2013 May 18 doi: 10.1016/j.anl.2013.04.007. [Epub ahead of print] PMID: 23694736
Luiz HV, da Silva TN, Pereira BD, Santos JG, Gonçalves D, Manita I, Portugal J
Pediatrics 2013 Dec;132(6):e1709-14. Epub 2013 Nov 25 doi: 10.1542/peds.2013-0492. [Epub ahead of print] PMID: 24276837
Feng N, Li X, Gao HD, Liu ZL, Shi LJ, Liu WZ
Chin J Cancer 2013 Nov;32(11):624-8. Epub 2013 May 14 doi: 10.5732/cjc.012.10317. [Epub ahead of print] PMID: 23668927Free PMC Article

Therapy

Hadoux J, Favier J, Scoazec JY, Leboulleux S, Al Ghuzlan A, Caramella C, Déandreis D, Borget I, Loriot C, Chougnet C, Letouzé E, Young J, Amar L, Bertherat J, Libé R, Dumont F, Deschamps F, Schlumberger M, Gimenez-Roqueplo AP, Baudin E
Int J Cancer 2014 Dec 1;135(11):2711-20. Epub 2014 May 5 doi: 10.1002/ijc.28913. [Epub ahead of print] PMID: 24752622
Li Y, Guo A, Tang J, Li Q, Fei X, Zhang Y, Gao J
Ultrasound Med Biol 2014 Mar;40(3):478-84. Epub 2014 Jan 10 doi: 10.1016/j.ultrasmedbio.2013.10.014. [Epub ahead of print] PMID: 24412175
van Hulsteijn LT, Niemeijer ND, Dekkers OM, Corssmit EP
Clin Endocrinol (Oxf) 2014 Apr;80(4):487-501. Epub 2013 Nov 19 doi: 10.1111/cen.12341. [Epub ahead of print] PMID: 24118038
Pikis S, Cohen JE, Gomori JM, Fellig Y, Chrysostomou C, Barzilay Y, Kaplan L, Itshayek E, Hasharoni A
Clin J Pain 2013 Dec;29(12):e39-41. doi: 10.1097/AJP.0b013e31829a4cc6. PMID: 23765044
Tian H, Chen Z, Wang C, Xing G
Head Neck 2013 Oct;35(10):E304-7. Epub 2012 Sep 18 doi: 10.1002/hed.23164. [Epub ahead of print] PMID: 22987597

Prognosis

Toyoda H, Hirayama J, Sugimoto Y, Uchida K, Ohishi K, Hirayama M, Komada Y
Pediatrics 2014 Jun;133(6):e1787-91. Epub 2014 May 12 doi: 10.1542/peds.2013-2419. [Epub ahead of print] PMID: 24819565
Hadoux J, Favier J, Scoazec JY, Leboulleux S, Al Ghuzlan A, Caramella C, Déandreis D, Borget I, Loriot C, Chougnet C, Letouzé E, Young J, Amar L, Bertherat J, Libé R, Dumont F, Deschamps F, Schlumberger M, Gimenez-Roqueplo AP, Baudin E
Int J Cancer 2014 Dec 1;135(11):2711-20. Epub 2014 May 5 doi: 10.1002/ijc.28913. [Epub ahead of print] PMID: 24752622
van Hulsteijn LT, Niemeijer ND, Dekkers OM, Corssmit EP
Clin Endocrinol (Oxf) 2014 Apr;80(4):487-501. Epub 2013 Nov 19 doi: 10.1111/cen.12341. [Epub ahead of print] PMID: 24118038
Luiz HV, da Silva TN, Pereira BD, Santos JG, Gonçalves D, Manita I, Portugal J
Pediatrics 2013 Dec;132(6):e1709-14. Epub 2013 Nov 25 doi: 10.1542/peds.2013-0492. [Epub ahead of print] PMID: 24276837
Lau D, La Marca F, Camelo-Piragua S, Park P
Clin Neurol Neurosurg 2013 Sep;115(9):1571-4. Epub 2013 Feb 9 doi: 10.1016/j.clineuro.2013.01.006. [Epub ahead of print] PMID: 23398849

Clinical prediction guides

Hadoux J, Favier J, Scoazec JY, Leboulleux S, Al Ghuzlan A, Caramella C, Déandreis D, Borget I, Loriot C, Chougnet C, Letouzé E, Young J, Amar L, Bertherat J, Libé R, Dumont F, Deschamps F, Schlumberger M, Gimenez-Roqueplo AP, Baudin E
Int J Cancer 2014 Dec 1;135(11):2711-20. Epub 2014 May 5 doi: 10.1002/ijc.28913. [Epub ahead of print] PMID: 24752622
Li Y, Guo A, Tang J, Li Q, Fei X, Zhang Y, Gao J
Ultrasound Med Biol 2014 Mar;40(3):478-84. Epub 2014 Jan 10 doi: 10.1016/j.ultrasmedbio.2013.10.014. [Epub ahead of print] PMID: 24412175
Takahashi K, Yamamoto Y, Ohshima S, Morita Y, Takahashi S
Auris Nasus Larynx 2014 Feb;41(1):93-6. Epub 2013 May 18 doi: 10.1016/j.anl.2013.04.007. [Epub ahead of print] PMID: 23694736
Yang Y, Guo L, Yang F, Huang Q, Zhang F, Ma H, Li H, Yang K, Lou J, Liu C
Med Oncol 2013 Dec;30(4):659. Epub 2013 Aug 31 doi: 10.1007/s12032-013-0659-8. [Epub ahead of print] PMID: 23996239
Feng N, Li X, Gao HD, Liu ZL, Shi LJ, Liu WZ
Chin J Cancer 2013 Nov;32(11):624-8. Epub 2013 May 14 doi: 10.5732/cjc.012.10317. [Epub ahead of print] PMID: 23668927Free PMC Article

Recent systematic reviews

Lenders JW, Duh QY, Eisenhofer G, Gimenez-Roqueplo AP, Grebe SK, Murad MH, Naruse M, Pacak K, Young WF Jr; Endocrine Society
J Clin Endocrinol Metab 2014 Jun;99(6):1915-42. doi: 10.1210/jc.2014-1498. PMID: 24893135
van Hulsteijn LT, Niemeijer ND, Dekkers OM, Corssmit EP
Clin Endocrinol (Oxf) 2014 Apr;80(4):487-501. Epub 2013 Nov 19 doi: 10.1111/cen.12341. [Epub ahead of print] PMID: 24118038
Castellani MR, Aktolun C, Buzzoni R, Seregni E, Chiesa C, Maccauro M, Aliberti GL, Vellani C, Lorenzoni A, Bombardieri E
Q J Nucl Med Mol Imaging 2013 Jun;57(2):146-52. PMID: 23822990
Rufini V, Treglia G, Castaldi P, Perotti G, Giordano A
Q J Nucl Med Mol Imaging 2013 Jun;57(2):122-33. PMID: 23822989
Schalenbourg A, Moulin A, Guillou L, Zografos L
Ophthalmology 2011 Nov;118(11):2238-41. Epub 2011 Sep 9 doi: 10.1016/j.ophtha.2011.04.017. [Epub ahead of print] PMID: 21906814

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