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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
Advances in Adrenal and Extra-adrenal Paraganglioma: Practical Synopsis for Pathologists.
Juhlin CC, Mete O
Adv Anat Pathol 2023 Jan 1;30(1):47-57. Epub 2022 Sep 23 doi: 10.1097/PAP.0000000000000365. PMID: 36136370
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

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
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
Paragangliomas in Carney-Stratakis Syndrome.
Khurana A, Mei L, Faber AC, Smith SC, Boikos SA
Horm Metab Res 2019 Jul;51(7):437-442. Epub 2019 Jun 7 doi: 10.1055/a-0918-8340. PMID: 31174229
Pheochromocytoma and extra-adrenal paraganglioma: updates.
Tischler AS
Arch Pathol Lab Med 2008 Aug;132(8):1272-84. doi: 10.5858/2008-132-1272-PAEPU. PMID: 18684026

Therapy

Proton therapy for head and neck paragangliomas: A single institutional experience.
Kang KH, Lebow ES, Niemierko A, Bussière MR, Dewyer NA, Daly J, McKenna MJ, Lee DJ, Loeffler JS, Busse PM, Shih HA
Head Neck 2020 Apr;42(4):670-677. Epub 2019 Dec 18 doi: 10.1002/hed.26044. PMID: 31850601
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
Ectopic acromegaly due to growth hormone releasing hormone.
Ghazi AA, Amirbaigloo A, Dezfooli AA, Saadat N, Ghazi S, Pourafkari M, Tirgari F, Dhall D, Bannykh S, Melmed S, Cooper O
Endocrine 2013 Apr;43(2):293-302. Epub 2012 Sep 15 doi: 10.1007/s12020-012-9790-0. PMID: 22983831Free PMC Article

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
Orbital Paraganglioma.
Zhu B, Yan J
J Craniofac Surg 2019 Sep;30(6):e503-e506. doi: 10.1097/SCS.0000000000005408. PMID: 30896513
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 and metastatic paraganglioma of the cranial vault.
Kakodkar P, Houlihan LM, Preul M, Bermingham N, Lim C
Br J Neurosurg 2023 Oct;37(5):967-975. Epub 2021 Mar 19 doi: 10.1080/02688697.2021.1902477. PMID: 33739182
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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