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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
The North American Neuroendocrine Tumor Society Consensus Guidelines for Surveillance and Management of Metastatic and/or Unresectable Pheochromocytoma and Paraganglioma.
Fishbein L, Del Rivero J, Else T, Howe JR, Asa SL, Cohen DL, Dahia PLM, Fraker DL, Goodman KA, Hope TA, Kunz PL, Perez K, Perrier ND, Pryma DA, Ryder M, Sasson AR, Soulen MC, Jimenez C
Pancreas 2021 Apr 1;50(4):469-493. doi: 10.1097/MPA.0000000000001792. PMID: 33939658

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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