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Unresectable Adrenal Gland Pheochromocytoma

MedGen UID:
1681030
•Concept ID:
C4763427
•
Neoplastic Process

Definition

Adrenal gland pheochromocytoma that is not amenable to surgical resection. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVUnresectable Adrenal Gland Pheochromocytoma

Professional guidelines

PubMed

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
Management and outcome of metastatic pheochromocytomas/paragangliomas: an overview.
De Filpo G, Maggi M, Mannelli M, Canu L
J Endocrinol Invest 2021 Jan;44(1):15-25. Epub 2020 Jun 29 doi: 10.1007/s40618-020-01344-z. PMID: 32602077
Metastatic pheochromocytoma and paraganglioma: Management of endocrine manifestations, surgery and ablative procedures, and systemic therapies.
Jasim S, Jimenez C
Best Pract Res Clin Endocrinol Metab 2020 Mar;34(2):101354. Epub 2019 Oct 24 doi: 10.1016/j.beem.2019.101354. PMID: 31685417

Recent clinical studies

Etiology

Cabozantinib in patients with unresectable and progressive metastatic phaeochromocytoma or paraganglioma (the Natalie Trial): a single-arm, phase 2 trial.
Jimenez C, Habra MA, Campbell MT, Tamsen G, Cruz-Goldberg D, Long J, Bassett R, Dantzer R, Balderrama-Brondani V, Varghese J, Lu Y
Lancet Oncol 2024 May;25(5):658-667. Epub 2024 Apr 9 doi: 10.1016/S1470-2045(24)00133-5. PMID: 38608693
Efficacy and Safety of Tyrosine Kinase Inhibitors in Patients with Metastatic Pheochromocytomas/Paragangliomas.
Zhou Y, Cui Y, Zhang D, Tong A
J Clin Endocrinol Metab 2023 Feb 15;108(3):755-766. doi: 10.1210/clinem/dgac657. PMID: 36383456
Laparoscopic approach of pediatric adrenal tumors.
Oesterreich R, Varela MF, Moldes J, Lobos P
Pediatr Surg Int 2022 Oct;38(10):1435-1444. Epub 2022 Jul 25 doi: 10.1007/s00383-022-05188-x. PMID: 35876904
Multiple endocrine neoplasia type 2.
Gertner ME, Kebebew E
Curr Treat Options Oncol 2004 Aug;5(4):315-25. doi: 10.1007/s11864-004-0022-6. PMID: 15233908
Pediatric malignant pheochromocytoma.
Ein SH, Weitzman S, Thorner P, Seagram CG, Filler RM
J Pediatr Surg 1994 Sep;29(9):1197-201. doi: 10.1016/0022-3468(94)90799-4. PMID: 7807344

Diagnosis

Laparoscopic approach of pediatric adrenal tumors.
Oesterreich R, Varela MF, Moldes J, Lobos P
Pediatr Surg Int 2022 Oct;38(10):1435-1444. Epub 2022 Jul 25 doi: 10.1007/s00383-022-05188-x. PMID: 35876904
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
Management and outcome of metastatic pheochromocytomas/paragangliomas: an overview.
De Filpo G, Maggi M, Mannelli M, Canu L
J Endocrinol Invest 2021 Jan;44(1):15-25. Epub 2020 Jun 29 doi: 10.1007/s40618-020-01344-z. PMID: 32602077
Metastatic pheochromocytoma and paraganglioma.
Angelousi A, Kassi E, Zografos G, Kaltsas G
Eur J Clin Invest 2015 Sep;45(9):986-97. doi: 10.1111/eci.12495. PMID: 26183460
Malignant adrenal tumors.
Rescorla FJ
Semin Pediatr Surg 2006 Feb;15(1):48-56. doi: 10.1053/j.sempedsurg.2005.11.008. PMID: 16458846

Therapy

Cabozantinib in patients with unresectable and progressive metastatic phaeochromocytoma or paraganglioma (the Natalie Trial): a single-arm, phase 2 trial.
Jimenez C, Habra MA, Campbell MT, Tamsen G, Cruz-Goldberg D, Long J, Bassett R, Dantzer R, Balderrama-Brondani V, Varghese J, Lu Y
Lancet Oncol 2024 May;25(5):658-667. Epub 2024 Apr 9 doi: 10.1016/S1470-2045(24)00133-5. PMID: 38608693
Efficacy and Safety of Tyrosine Kinase Inhibitors in Patients with Metastatic Pheochromocytomas/Paragangliomas.
Zhou Y, Cui Y, Zhang D, Tong A
J Clin Endocrinol Metab 2023 Feb 15;108(3):755-766. doi: 10.1210/clinem/dgac657. PMID: 36383456
131I-metaiodobenzylguanidine and peptide receptor radionuclide therapy in pheochromocytoma and paraganglioma.
Jungels C, Karfis I
Curr Opin Oncol 2021 Jan;33(1):33-39. doi: 10.1097/CCO.0000000000000691. PMID: 33093336
Metastatic pheochromocytoma and paraganglioma: Management of endocrine manifestations, surgery and ablative procedures, and systemic therapies.
Jasim S, Jimenez C
Best Pract Res Clin Endocrinol Metab 2020 Mar;34(2):101354. Epub 2019 Oct 24 doi: 10.1016/j.beem.2019.101354. PMID: 31685417
Multiple endocrine neoplasia type 2.
Gertner ME, Kebebew E
Curr Treat Options Oncol 2004 Aug;5(4):315-25. doi: 10.1007/s11864-004-0022-6. PMID: 15233908

Prognosis

Management and outcome of metastatic pheochromocytomas/paragangliomas: an overview.
De Filpo G, Maggi M, Mannelli M, Canu L
J Endocrinol Invest 2021 Jan;44(1):15-25. Epub 2020 Jun 29 doi: 10.1007/s40618-020-01344-z. PMID: 32602077
Metastatic pheochromocytoma and paraganglioma.
Angelousi A, Kassi E, Zografos G, Kaltsas G
Eur J Clin Invest 2015 Sep;45(9):986-97. doi: 10.1111/eci.12495. PMID: 26183460
Malignant adrenal tumors.
Rescorla FJ
Semin Pediatr Surg 2006 Feb;15(1):48-56. doi: 10.1053/j.sempedsurg.2005.11.008. PMID: 16458846
Multiple endocrine neoplasia type 2.
Gertner ME, Kebebew E
Curr Treat Options Oncol 2004 Aug;5(4):315-25. doi: 10.1007/s11864-004-0022-6. PMID: 15233908
Pediatric malignant pheochromocytoma.
Ein SH, Weitzman S, Thorner P, Seagram CG, Filler RM
J Pediatr Surg 1994 Sep;29(9):1197-201. doi: 10.1016/0022-3468(94)90799-4. PMID: 7807344

Clinical prediction guides

Efficacy and Safety of Tyrosine Kinase Inhibitors in Patients with Metastatic Pheochromocytomas/Paragangliomas.
Zhou Y, Cui Y, Zhang D, Tong A
J Clin Endocrinol Metab 2023 Feb 15;108(3):755-766. doi: 10.1210/clinem/dgac657. PMID: 36383456
Prognostic value of [(18)F]FDG-PET prior to [(131)I]MIBG treatment for pheochromocytoma and paraganglioma (PPGL).
Takenaka J, Watanabe S, Abe T, Hirata K, Uchiyama Y, Kimura R, Shinohara N, Kudo K
Ann Nucl Med 2023 Jan;37(1):10-17. Epub 2022 Oct 27 doi: 10.1007/s12149-022-01798-6. PMID: 36301465
SDHx-related pheochromocytoma/paraganglioma - genetic, clinical, and treatment outcomes in a series of 30 patients from a single center.
Donato S, Simões H, Pinto AT, M Cavaco B, Leite V
Endocrine 2019 Aug;65(2):408-415. Epub 2019 May 18 doi: 10.1007/s12020-019-01953-6. PMID: 31104306
Metastatic pheochromocytoma and paraganglioma.
Angelousi A, Kassi E, Zografos G, Kaltsas G
Eur J Clin Invest 2015 Sep;45(9):986-97. doi: 10.1111/eci.12495. PMID: 26183460
Role of VEGF-A and its receptors in sporadic and MEN2-associated pheochromocytoma.
Ferreira CV, Siqueira DR, Romitti M, Ceolin L, Brasil BA, Meurer L, Capp C, Maia AL
Int J Mol Sci 2014 Mar 26;15(4):5323-36. doi: 10.3390/ijms15045323. PMID: 24675699Free PMC Article

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