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Extra-Adrenal Paraganglioma

MedGen UID:
18289
•Concept ID:
C0030422
•
Neoplastic Process
Synonyms: Chemodectoma; Chemodectomas; Extra-Adrenal Paragangliomas; Non-Chromaffin Paraganglioma; Non-Chromaffin Paragangliomas; Nonchromaffin Paraganglioma; Nonchromaffin Paragangliomas; Paraganglioma, Extra Adrenal; Paraganglioma, Extra-Adrenal; Paraganglioma, Non Chromaffin; Paraganglioma, Non-Chromaffin; Paraganglioma, Nonchromaffin; Paragangliomas, Extra-Adrenal; Paragangliomas, Familial Nonchromaffin, 1; Paragangliomas, Non-Chromaffin; Paragangliomas, Nonchromaffin
SNOMED CT: Non-chromaffin paraganglioma (302834008); Chemodectoma (302834008); Extra-adrenal paraganglioma (302834008); Extra-adrenal paraganglioma (51747000); Chemodectoma (51747000); Nonchromaffin paraganglioma (51747000)
 
OMIM®: 168000

Definition

A paraganglioma arising from sympathetic or parasympathetic paraganglia outside the adrenal gland. [from NCI]

Professional guidelines

PubMed

Outcomes of Carotid Body Tumor Management with Active Surveillance.
Lyle DA, Lopez A, Osofsky R, Wiemann B, Boyd N, Olson G, Rana MA
Ann Otol Rhinol Laryngol 2023 May;132(5):551-557. Epub 2022 Jun 20 doi: 10.1177/00034894221105833. PMID: 35723203
Clinical evaluation and treatment of phaeochromocytoma.
Davison AS, Jones DM, Ruthven S, Helliwell T, Shore SL
Ann Clin Biochem 2018 Jan;55(1):34-48. Epub 2017 Nov 8 doi: 10.1177/0004563217739931. PMID: 29027806
Management of carotid body tumors and familial paragangliomas: review of 30 years' experience.
Unlü Y, Becit N, Ceviz M, Koçak H
Ann Vasc Surg 2009 Sep-Oct;23(5):616-20. doi: 10.1016/j.avsg.2009.06.014. PMID: 19747612

Recent clinical studies

Etiology

Paraganglioma of the Head and Neck: A Review.
Sandow L, Thawani R, Kim MS, Heinrich MC
Endocr Pract 2023 Feb;29(2):141-147. Epub 2022 Oct 15 doi: 10.1016/j.eprac.2022.10.002. PMID: 36252779Free PMC Article
Overview of the 2022 WHO Classification of Paragangliomas and Pheochromocytomas.
Mete O, Asa SL, Gill AJ, Kimura N, de Krijger RR, Tischler A
Endocr Pathol 2022 Mar;33(1):90-114. Epub 2022 Mar 13 doi: 10.1007/s12022-022-09704-6. PMID: 35285002
Paragangliomas of the head and neck.
Valero C, Ganly I
J Oral Pathol Med 2022 Nov;51(10):897-903. Epub 2022 Feb 23 doi: 10.1111/jop.13286. PMID: 35178777
Clinical Characterization of the Pheochromocytoma and Paraganglioma Susceptibility Genes SDHA, TMEM127, MAX, and SDHAF2 for Gene-Informed Prevention.
Bausch B, Schiavi F, Ni Y, Welander J, Patocs A, Ngeow J, Wellner U, Malinoc A, Taschin E, Barbon G, Lanza V, Söderkvist P, Stenman A, Larsson C, Svahn F, Chen JL, Marquard J, Fraenkel M, Walter MA, Peczkowska M, Prejbisz A, Jarzab B, Hasse-Lazar K, Petersenn S, Moeller LC, Meyer A, Reisch N, Trupka A, Brase C, Galiano M, Preuss SF, Kwok P, Lendvai N, Berisha G, Makay Ö, Boedeker CC, Weryha G, Racz K, Januszewicz A, Walz MK, Gimm O, Opocher G, Eng C, Neumann HPH; European-American-Asian Pheochromocytoma-Paraganglioma Registry Study Group
JAMA Oncol 2017 Sep 1;3(9):1204-1212. doi: 10.1001/jamaoncol.2017.0223. PMID: 28384794Free PMC Article
Gastrointestinal stromal tumour.
Joensuu H, Hohenberger P, Corless CL
Lancet 2013 Sep 14;382(9896):973-83. Epub 2013 Apr 24 doi: 10.1016/S0140-6736(13)60106-3. PMID: 23623056

Diagnosis

Paraganglioma of the Head and Neck: A Review.
Sandow L, Thawani R, Kim MS, Heinrich MC
Endocr Pract 2023 Feb;29(2):141-147. Epub 2022 Oct 15 doi: 10.1016/j.eprac.2022.10.002. PMID: 36252779Free PMC Article
Paragangliomas of the head and neck.
Valero C, Ganly I
J Oral Pathol Med 2022 Nov;51(10):897-903. Epub 2022 Feb 23 doi: 10.1111/jop.13286. PMID: 35178777
Recent advances in the management of pheochromocytoma and paraganglioma.
Tanabe A, Naruse M
Hypertens Res 2020 Nov;43(11):1141-1151. Epub 2020 Aug 11 doi: 10.1038/s41440-020-0531-0. PMID: 32778780
Pheochromocytoma and Paraganglioma.
Neumann HPH, Young WF Jr, Eng C
N Engl J Med 2019 Aug 8;381(6):552-565. doi: 10.1056/NEJMra1806651. PMID: 31390501
PHEOCHROMOCYTOMA: A GENETIC AND DIAGNOSTIC UPDATE.
Mercado-Asis LB, Wolf KI, Jochmanova I, Taïeb D
Endocr Pract 2018 Jan;24(1):78-90. Epub 2017 Nov 16 doi: 10.4158/EP-2017-0057. PMID: 29144820

Therapy

Update on Pheochromocytoma and Paraganglioma from the SSO Endocrine and Head and Neck Disease Site Working Group, Part 2 of 2: Perioperative Management and Outcomes of Pheochromocytoma and Paraganglioma.
Patel D, Phay JE, Yen TWF, Dickson PV, Wang TS, Garcia R, Yang AD, Kim LT, Solórzano CC
Ann Surg Oncol 2020 May;27(5):1338-1347. Epub 2020 Feb 28 doi: 10.1245/s10434-020-08221-2. PMID: 32112213Free PMC Article
Preoperative tumor embolization.
Ashour R, Aziz-Sultan A
Neurosurg Clin N Am 2014 Jul;25(3):607-17. Epub 2014 May 20 doi: 10.1016/j.nec.2014.04.015. PMID: 24994094
Gastrointestinal stromal tumour.
Joensuu H, Hohenberger P, Corless CL
Lancet 2013 Sep 14;382(9896):973-83. Epub 2013 Apr 24 doi: 10.1016/S0140-6736(13)60106-3. PMID: 23623056
Carney's triad.
Noushif M, Chandra A, Gupta V, Kumar A
Indian J Gastroenterol 2013 Jul;32(4):281-2. Epub 2013 Mar 5 doi: 10.1007/s12664-013-0310-9. PMID: 23460241
Pheochromocytomas and extra-adrenal paragangliomas detected by screening in patients with SDHD-associated head-and-neck paragangliomas.
Havekes B, van der Klaauw AA, Weiss MM, Jansen JC, van der Mey AG, Vriends AH, Bonsing BA, Romijn JA, Corssmit EP
Endocr Relat Cancer 2009 Jun;16(2):527-36. Epub 2009 Mar 16 doi: 10.1677/ERC-09-0024. PMID: 19289533

Prognosis

Vagal paragangliomas.
Mottie L, Meulemans J, Vander Poorten V
Curr Opin Otolaryngol Head Neck Surg 2023 Apr 1;31(2):146-154. Epub 2023 Feb 9 doi: 10.1097/MOO.0000000000000876. PMID: 36912227
Paraganglioma of the Head and Neck: A Review.
Sandow L, Thawani R, Kim MS, Heinrich MC
Endocr Pract 2023 Feb;29(2):141-147. Epub 2022 Oct 15 doi: 10.1016/j.eprac.2022.10.002. PMID: 36252779Free PMC Article
Overview of the 2022 WHO Classification of Paragangliomas and Pheochromocytomas.
Mete O, Asa SL, Gill AJ, Kimura N, de Krijger RR, Tischler A
Endocr Pathol 2022 Mar;33(1):90-114. Epub 2022 Mar 13 doi: 10.1007/s12022-022-09704-6. PMID: 35285002
Succinate Dehydrogenase-Deficient Gastrointestinal Stromal Tumors.
Ibrahim A, Chopra S
Arch Pathol Lab Med 2020 May;144(5):655-660. Epub 2019 Jun 6 doi: 10.5858/arpa.2018-0370-RS. PMID: 31169996
Clinical Characterization of the Pheochromocytoma and Paraganglioma Susceptibility Genes SDHA, TMEM127, MAX, and SDHAF2 for Gene-Informed Prevention.
Bausch B, Schiavi F, Ni Y, Welander J, Patocs A, Ngeow J, Wellner U, Malinoc A, Taschin E, Barbon G, Lanza V, Söderkvist P, Stenman A, Larsson C, Svahn F, Chen JL, Marquard J, Fraenkel M, Walter MA, Peczkowska M, Prejbisz A, Jarzab B, Hasse-Lazar K, Petersenn S, Moeller LC, Meyer A, Reisch N, Trupka A, Brase C, Galiano M, Preuss SF, Kwok P, Lendvai N, Berisha G, Makay Ö, Boedeker CC, Weryha G, Racz K, Januszewicz A, Walz MK, Gimm O, Opocher G, Eng C, Neumann HPH; European-American-Asian Pheochromocytoma-Paraganglioma Registry Study Group
JAMA Oncol 2017 Sep 1;3(9):1204-1212. doi: 10.1001/jamaoncol.2017.0223. PMID: 28384794Free PMC Article

Clinical prediction guides

Paraganglioma of the Head and Neck: A Review.
Sandow L, Thawani R, Kim MS, Heinrich MC
Endocr Pract 2023 Feb;29(2):141-147. Epub 2022 Oct 15 doi: 10.1016/j.eprac.2022.10.002. PMID: 36252779Free PMC Article
Overview of the 2022 WHO Classification of Paragangliomas and Pheochromocytomas.
Mete O, Asa SL, Gill AJ, Kimura N, de Krijger RR, Tischler A
Endocr Pathol 2022 Mar;33(1):90-114. Epub 2022 Mar 13 doi: 10.1007/s12022-022-09704-6. PMID: 35285002
Paragangliomas of the head and neck.
Valero C, Ganly I
J Oral Pathol Med 2022 Nov;51(10):897-903. Epub 2022 Feb 23 doi: 10.1111/jop.13286. PMID: 35178777
Recent advances in the management of pheochromocytoma and paraganglioma.
Tanabe A, Naruse M
Hypertens Res 2020 Nov;43(11):1141-1151. Epub 2020 Aug 11 doi: 10.1038/s41440-020-0531-0. PMID: 32778780
Clinical Characterization of the Pheochromocytoma and Paraganglioma Susceptibility Genes SDHA, TMEM127, MAX, and SDHAF2 for Gene-Informed Prevention.
Bausch B, Schiavi F, Ni Y, Welander J, Patocs A, Ngeow J, Wellner U, Malinoc A, Taschin E, Barbon G, Lanza V, Söderkvist P, Stenman A, Larsson C, Svahn F, Chen JL, Marquard J, Fraenkel M, Walter MA, Peczkowska M, Prejbisz A, Jarzab B, Hasse-Lazar K, Petersenn S, Moeller LC, Meyer A, Reisch N, Trupka A, Brase C, Galiano M, Preuss SF, Kwok P, Lendvai N, Berisha G, Makay Ö, Boedeker CC, Weryha G, Racz K, Januszewicz A, Walz MK, Gimm O, Opocher G, Eng C, Neumann HPH; European-American-Asian Pheochromocytoma-Paraganglioma Registry Study Group
JAMA Oncol 2017 Sep 1;3(9):1204-1212. doi: 10.1001/jamaoncol.2017.0223. PMID: 28384794Free PMC Article

Recent systematic reviews

Primary Paraganglioma of the Spine: A Systematic Review of Clinical Features and Surgical Management in Cauda Equina versus Non-Cauda Equina Lesions.
Palmisciano P, Sagoo NS, Haider AS, Ogasawara C, Ogasawara M, Bin Alamer O, Heidari KS, Raj KM, Scalia G, Umana GE, Krishnaney AA, Aoun SG, Passias PG, Vira S
World Neurosurg 2022 May;161:190-197.e20. Epub 2022 Feb 3 doi: 10.1016/j.wneu.2022.01.110. PMID: 35123022
Imaging pediatric gastrointestinal stromal tumor (GIST).
Herzberg M, Beer M, Anupindi S, Vollert K, Kröncke T
J Pediatr Surg 2018 Sep;53(9):1862-1870. Epub 2018 Mar 29 doi: 10.1016/j.jpedsurg.2018.03.022. PMID: 29685489

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