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Advanced Paraganglioma

MedGen UID:
1690182
•Concept ID:
C5205607
•
Neoplastic Process

Definition

A paraganglioma that has spread extensively to other anatomic sites or is no longer responding to treatment. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAdvanced Paraganglioma

Professional guidelines

PubMed

Clinical consensus guideline on the management of phaeochromocytoma and paraganglioma in patients harbouring germline SDHD pathogenic variants.
Taïeb D, Wanna GB, Ahmad M, Lussey-Lepoutre C, Perrier ND, Nölting S, Amar L, Timmers HJLM, Schwam ZG, Estrera AL, Lim M, Pollom EL, Vitzthum L, Bourdeau I, Casey RT, Castinetti F, Clifton-Bligh R, Corssmit EPM, de Krijger RR, Del Rivero J, Eisenhofer G, Ghayee HK, Gimenez-Roqueplo AP, Grossman A, Imperiale A, Jansen JC, Jha A, Kerstens MN, Kunst HPM, Liu JK, Maher ER, Marchioni D, Mercado-Asis LB, Mete O, Naruse M, Nilubol N, Pandit-Taskar N, Sebag F, Tanabe A, Widimsky J, Meuter L, Lenders JWM, Pacak K
Lancet Diabetes Endocrinol 2023 May;11(5):345-361. Epub 2023 Mar 31 doi: 10.1016/S2213-8587(23)00038-4. PMID: 37011647Free PMC Article
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
Adrenocortical carcinomas and malignant phaeochromocytomas: ESMO-EURACAN Clinical Practice Guidelines for diagnosis, treatment and follow-up.
Fassnacht M, Assie G, Baudin E, Eisenhofer G, de la Fouchardiere C, Haak HR, de Krijger R, Porpiglia F, Terzolo M, Berruti A; ESMO Guidelines Committee. Electronic address: clinicalguidelines@esmo.org
Ann Oncol 2020 Nov;31(11):1476-1490. Epub 2020 Aug 27 doi: 10.1016/j.annonc.2020.08.2099. PMID: 32861807

Recent clinical studies

Diagnosis

Malignant phenotype and two SDHD mutations in a family with paraganglioma syndrome type 1.
Leidenz FB, Bastos-Rodrigues L, Oliveira M, Mamede M, Sarquis M, Friedman E, de Marco L
Genet Res (Camb) 2015 Mar 30;97:e3. doi: 10.1017/S0016672315000063. PMID: 25819804Free PMC Article
Locally advanced paraganglioma of the urinary bladder: a case report.
Beilan J, Lawton A, Hajdenberg J, Rosser CJ
BMC Res Notes 2013 Apr 18;6:156. doi: 10.1186/1756-0500-6-156. PMID: 23594404Free PMC Article
Brief report: Advanced paraganglioma: A role for chemotherapy?
Fitoussi O, Debled M, Masson B, Coindre JM, Kantor G, Bui BN
Med Pediatr Oncol 1999 Aug;33(2):129-31. doi: 10.1002/(sici)1096-911x(199908)33:2<129::aid-mpo15>3.0.co;2-x. PMID: 10398192

Therapy

Radiolabeled DOTATOC in patients with advanced paraganglioma and pheochromocytoma.
Forrer F, Riedweg I, Maecke HR, Mueller-Brand J
Q J Nucl Med Mol Imaging 2008 Dec;52(4):334-40. Epub 2008 May 16 PMID: 18480742
Brief report: Advanced paraganglioma: A role for chemotherapy?
Fitoussi O, Debled M, Masson B, Coindre JM, Kantor G, Bui BN
Med Pediatr Oncol 1999 Aug;33(2):129-31. doi: 10.1002/(sici)1096-911x(199908)33:2<129::aid-mpo15>3.0.co;2-x. PMID: 10398192

Prognosis

Malignant phenotype and two SDHD mutations in a family with paraganglioma syndrome type 1.
Leidenz FB, Bastos-Rodrigues L, Oliveira M, Mamede M, Sarquis M, Friedman E, de Marco L
Genet Res (Camb) 2015 Mar 30;97:e3. doi: 10.1017/S0016672315000063. PMID: 25819804Free PMC Article
Locally advanced paraganglioma of the urinary bladder: a case report.
Beilan J, Lawton A, Hajdenberg J, Rosser CJ
BMC Res Notes 2013 Apr 18;6:156. doi: 10.1186/1756-0500-6-156. PMID: 23594404Free PMC Article

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