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Parasympathetic paraganglioma

MedGen UID:
90779
•Concept ID:
C0334416
•
Neoplastic Process
Synonyms: paraganglioma of parasympathetic nervous system; Parasympathetic Extra-Adrenal Paraganglioma; parasympathetic Extra-adrenal paraganglioma; parasympathetic nervous system paraganglioma; Parasympathetic Paraganglioma; parasympathetic paraganglioma; Parasympathetic Paraganglionic Neoplasm; parasympathetic Paraganglionic neoplasm
SNOMED CT: Parasympathetic paraganglioma (52205002)
 
Monarch Initiative: MONDO:0021052

Definition

An extra-adrenal paraganglioma that arises from paraganglia located along the parasympathetic nerves. Representative examples include aorticopulmonary, carotid body, jugulotympanic, and mediastinal paragangliomas. [from NCI]

Professional guidelines

PubMed

Diagnosis and Management of Pheochromocytomas and Paragangliomas: A Guide for the Clinician.
Sharma S, Fishbein L
Endocr Pract 2023 Dec;29(12):999-1006. Epub 2023 Aug 15 doi: 10.1016/j.eprac.2023.07.027. PMID: 37586639
Multidisciplinary practice guidelines for the diagnosis, genetic counseling and treatment of pheochromocytomas and paragangliomas.
Garcia-Carbonero R, Matute Teresa F, Mercader-Cidoncha E, Mitjavila-Casanovas M, Robledo M, Tena I, Alvarez-Escola C, Arístegui M, Bella-Cueto MR, Ferrer-Albiach C, Hanzu FA
Clin Transl Oncol 2021 Oct;23(10):1995-2019. Epub 2021 May 6 doi: 10.1007/s12094-021-02622-9. PMID: 33959901Free PMC Article
Overview of primary adult retroperitoneal tumours.
Improta L, Tzanis D, Bouhadiba T, Abdelhafidh K, Bonvalot S
Eur J Surg Oncol 2020 Sep;46(9):1573-1579. Epub 2020 May 21 doi: 10.1016/j.ejso.2020.04.054. PMID: 32600897

Recent clinical studies

Etiology

High prevalence of occult paragangliomas in asymptomatic carriers of SDHD and SDHB gene mutations.
Heesterman BL, Bayley JP, Tops CM, Hes FJ, van Brussel BT, Corssmit EP, Hamming JF, van der Mey AG, Jansen JC
Eur J Hum Genet 2013 Apr;21(4):469-70. Epub 2012 Sep 5 doi: 10.1038/ejhg.2012.203. PMID: 22948026Free PMC Article
Functional consequences of succinate dehydrogenase mutations.
Opocher G, Schiavi F
Endocr Pract 2011 Jul-Aug;17 Suppl 3:64-71. doi: 10.4158/EP11070.RA. PMID: 21742608
p53 alterations and their relationship to SDHD mutations in parasympathetic paragangliomas.
van Nederveen FH, Dannenberg H, Sleddens HF, de Krijger RR, Dinjens WN
Mod Pathol 2003 Sep;16(9):849-56. doi: 10.1097/01.MP.0000084111.03922.4D. PMID: 13679447
Frequent germ-line succinate dehydrogenase subunit D gene mutations in patients with apparently sporadic parasympathetic paraganglioma.
Dannenberg H, Dinjens WN, Abbou M, Van Urk H, Pauw BK, Mouwen D, Mooi WJ, de Krijger RR
Clin Cancer Res 2002 Jul;8(7):2061-6. PMID: 12114404

Diagnosis

Ultrastructural features of tumor-associated mast cells in parasympathetic paragangliomas (chemodectomas) of the neck.
Chekmaryova I, Kalinin D, Kostin A, Buchwalow I, Tiemann M, Elieh-Ali-Komi D, Atiakshin D
Microsc Res Tech 2024 Jun;87(6):1373-1383. Epub 2024 Feb 21 doi: 10.1002/jemt.24523. PMID: 38380731
High prevalence of occult paragangliomas in asymptomatic carriers of SDHD and SDHB gene mutations.
Heesterman BL, Bayley JP, Tops CM, Hes FJ, van Brussel BT, Corssmit EP, Hamming JF, van der Mey AG, Jansen JC
Eur J Hum Genet 2013 Apr;21(4):469-70. Epub 2012 Sep 5 doi: 10.1038/ejhg.2012.203. PMID: 22948026Free PMC Article

Prognosis

Frequent germ-line succinate dehydrogenase subunit D gene mutations in patients with apparently sporadic parasympathetic paraganglioma.
Dannenberg H, Dinjens WN, Abbou M, Van Urk H, Pauw BK, Mouwen D, Mooi WJ, de Krijger RR
Clin Cancer Res 2002 Jul;8(7):2061-6. PMID: 12114404

Clinical prediction guides

Ultrastructural features of tumor-associated mast cells in parasympathetic paragangliomas (chemodectomas) of the neck.
Chekmaryova I, Kalinin D, Kostin A, Buchwalow I, Tiemann M, Elieh-Ali-Komi D, Atiakshin D
Microsc Res Tech 2024 Jun;87(6):1373-1383. Epub 2024 Feb 21 doi: 10.1002/jemt.24523. PMID: 38380731
p53 alterations and their relationship to SDHD mutations in parasympathetic paragangliomas.
van Nederveen FH, Dannenberg H, Sleddens HF, de Krijger RR, Dinjens WN
Mod Pathol 2003 Sep;16(9):849-56. doi: 10.1097/01.MP.0000084111.03922.4D. PMID: 13679447
Frequent germ-line succinate dehydrogenase subunit D gene mutations in patients with apparently sporadic parasympathetic paraganglioma.
Dannenberg H, Dinjens WN, Abbou M, Van Urk H, Pauw BK, Mouwen D, Mooi WJ, de Krijger RR
Clin Cancer Res 2002 Jul;8(7):2061-6. PMID: 12114404

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