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Benign adrenal gland pheochromocytoma

MedGen UID:
317403
•Concept ID:
C1706920
•
Neoplastic Process
Synonyms: adrenal gland pheochromocytoma, benign; Benign Adrenal Gland Pheochromocytoma; benign adrenal gland pheochromocytoma; Non-Metastatic Adrenal Gland Pheochromocytoma; pheochromocytoma, benign
 
Monarch Initiative: MONDO:0006103

Definition

A sporadic or familial pheochromocytoma that is confined to the adrenal gland. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVBenign adrenal gland pheochromocytoma

Professional guidelines

PubMed

European Society of Endocrinology clinical practice guidelines on the management of adrenal incidentalomas, in collaboration with the European Network for the Study of Adrenal Tumors.
Fassnacht M, Tsagarakis S, Terzolo M, Tabarin A, Sahdev A, Newell-Price J, Pelsma I, Marina L, Lorenz K, Bancos I, Arlt W, Dekkers OM
Eur J Endocrinol 2023 Jul 20;189(1):G1-G42. doi: 10.1093/ejendo/lvad066. PMID: 37318239
Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors.
Fassnacht M, Arlt W, Bancos I, Dralle H, Newell-Price J, Sahdev A, Tabarin A, Terzolo M, Tsagarakis S, Dekkers OM
Eur J Endocrinol 2016 Aug;175(2):G1-G34. doi: 10.1530/EJE-16-0467. PMID: 27390021
Pheochromocytoma: presentation, diagnosis and treatment.
Reisch N, Peczkowska M, Januszewicz A, Neumann HP
J Hypertens 2006 Dec;24(12):2331-9. doi: 10.1097/01.hjh.0000251887.01885.54. PMID: 17082709

Recent clinical studies

Etiology

European Society of Endocrinology clinical practice guidelines on the management of adrenal incidentalomas, in collaboration with the European Network for the Study of Adrenal Tumors.
Fassnacht M, Tsagarakis S, Terzolo M, Tabarin A, Sahdev A, Newell-Price J, Pelsma I, Marina L, Lorenz K, Bancos I, Arlt W, Dekkers OM
Eur J Endocrinol 2023 Jul 20;189(1):G1-G42. doi: 10.1093/ejendo/lvad066. PMID: 37318239
American Association of Endocrine Surgeons Guidelines for Adrenalectomy: Executive Summary.
Yip L, Duh QY, Wachtel H, Jimenez C, Sturgeon C, Lee C, Velázquez-Fernández D, Berber E, Hammer GD, Bancos I, Lee JA, Marko J, Morris-Wiseman LF, Hughes MS, Livhits MJ, Han MA, Smith PW, Wilhelm S, Asa SL, Fahey TJ 3rd, McKenzie TJ, Strong VE, Perrier ND
JAMA Surg 2022 Oct 1;157(10):870-877. doi: 10.1001/jamasurg.2022.3544. PMID: 35976622Free 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
Management of adrenal incidentalomas: European Society of Endocrinology Clinical Practice Guideline in collaboration with the European Network for the Study of Adrenal Tumors.
Fassnacht M, Arlt W, Bancos I, Dralle H, Newell-Price J, Sahdev A, Tabarin A, Terzolo M, Tsagarakis S, Dekkers OM
Eur J Endocrinol 2016 Aug;175(2):G1-G34. doi: 10.1530/EJE-16-0467. PMID: 27390021
Pheochromocytoma of the Adrenal gland Scaled Score (PASS) to separate benign from malignant neoplasms: a clinicopathologic and immunophenotypic study of 100 cases.
Thompson LD
Am J Surg Pathol 2002 May;26(5):551-66. doi: 10.1097/00000478-200205000-00002. PMID: 11979086

Diagnosis

Adrenal Neoplasms: Lessons from Adrenal Multidisciplinary Tumor Boards.
Chung R, Garratt J, Remer EM, Navin P, Blake MA, Taffel MT, Hackett CE, Sharbidre KG, Tu W, Low G, Bara M, Carney BW, Corwin MT, Campbell MJ, Lee JT, Lee CY, Dueber JC, Shehata MA, Caoili EM, Schieda N, Elsayes KM
Radiographics 2023 Jul;43(7):e220191. doi: 10.1148/rg.220191. PMID: 37347698
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
Pheochromocytoma: An approach to diagnosis.
Sbardella E, Grossman AB
Best Pract Res Clin Endocrinol Metab 2020 Mar;34(2):101346. Epub 2019 Oct 22 doi: 10.1016/j.beem.2019.101346. PMID: 31708376
Pathological grading for predicting metastasis in phaeochromocytoma and paraganglioma.
Kimura N, Takayanagi R, Takizawa N, Itagaki E, Katabami T, Kakoi N, Rakugi H, Ikeda Y, Tanabe A, Nigawara T, Ito S, Kimura I, Naruse M; Phaeochromocytoma Study Group in Japan
Endocr Relat Cancer 2014 Jun;21(3):405-14. Epub 2014 May 6 doi: 10.1530/ERC-13-0494. PMID: 24521857
Pheochromocytoma: presentation, diagnosis and treatment.
Reisch N, Peczkowska M, Januszewicz A, Neumann HP
J Hypertens 2006 Dec;24(12):2331-9. doi: 10.1097/01.hjh.0000251887.01885.54. PMID: 17082709

Therapy

Systemic Radiopharmaceutical Therapy of Pheochromocytoma and Paraganglioma.
Carrasquillo JA, Chen CC, Jha A, Pacak K, Pryma DA, Lin FI
J Nucl Med 2021 Sep 1;62(9):1192-1199. doi: 10.2967/jnumed.120.259697. PMID: 34475242Free PMC Article
Somatostatin Receptors and Analogs in Pheochromocytoma and Paraganglioma: Old Players in a New Precision Medicine World.
Patel M, Tena I, Jha A, Taieb D, Pacak K
Front Endocrinol (Lausanne) 2021;12:625312. Epub 2021 Mar 29 doi: 10.3389/fendo.2021.625312. PMID: 33854479Free PMC Article
Emerging Treatments for Advanced/Metastatic Pheochromocytoma and Paraganglioma.
Ilanchezhian M, Jha A, Pacak K, Del Rivero J
Curr Treat Options Oncol 2020 Aug 29;21(11):85. doi: 10.1007/s11864-020-00787-z. PMID: 32862332Free PMC Article
Malignant pheochromocytoma and paraganglioma: management options.
Corssmit EPM, Snel M, Kapiteijn E
Curr Opin Oncol 2020 Jan;32(1):20-26. doi: 10.1097/CCO.0000000000000589. PMID: 31599769
Malignant and benign pheochromocytoma: chromaffin granule transmitters and the response to medical and surgical treatment.
Rao F, Keiser HR, O'Connor DT
Ann N Y Acad Sci 2002 Oct;971:530-2. doi: 10.1111/j.1749-6632.2002.tb04519.x. PMID: 12438175

Prognosis

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
New Insights into Pheochromocytoma Biology: the Role of the Insulin - Like Growth Factor 1 (IGF1) System.
Pennisi PA, Fernández MC, Martin A
Pediatr Endocrinol Rev 2020 Aug;17(4):331-342. doi: 10.17458/per.vol17.2020.kc.insightspheochromocytomabiology. PMID: 32780957
Pathological grading for predicting metastasis in phaeochromocytoma and paraganglioma.
Kimura N, Takayanagi R, Takizawa N, Itagaki E, Katabami T, Kakoi N, Rakugi H, Ikeda Y, Tanabe A, Nigawara T, Ito S, Kimura I, Naruse M; Phaeochromocytoma Study Group in Japan
Endocr Relat Cancer 2014 Jun;21(3):405-14. Epub 2014 May 6 doi: 10.1530/ERC-13-0494. PMID: 24521857
Pheochromocytoma of the Adrenal gland Scaled Score (PASS) to separate benign from malignant neoplasms: a clinicopathologic and immunophenotypic study of 100 cases.
Thompson LD
Am J Surg Pathol 2002 May;26(5):551-66. doi: 10.1097/00000478-200205000-00002. PMID: 11979086
Adrenal incidentaloma.
Arnaldi G, Masini AM, Giacchetti G, Taccaliti A, Faloia E, Mantero F
Braz J Med Biol Res 2000 Oct;33(10):1177-89. doi: 10.1590/s0100-879x2000001000007. PMID: 11004718

Clinical prediction guides

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
Malignant pheochromocytoma and paraganglioma: management options.
Corssmit EPM, Snel M, Kapiteijn E
Curr Opin Oncol 2020 Jan;32(1):20-26. doi: 10.1097/CCO.0000000000000589. PMID: 31599769
Pathological grading for predicting metastasis in phaeochromocytoma and paraganglioma.
Kimura N, Takayanagi R, Takizawa N, Itagaki E, Katabami T, Kakoi N, Rakugi H, Ikeda Y, Tanabe A, Nigawara T, Ito S, Kimura I, Naruse M; Phaeochromocytoma Study Group in Japan
Endocr Relat Cancer 2014 Jun;21(3):405-14. Epub 2014 May 6 doi: 10.1530/ERC-13-0494. PMID: 24521857
Pheochromocytoma of the Adrenal gland Scaled Score (PASS) to separate benign from malignant neoplasms: a clinicopathologic and immunophenotypic study of 100 cases.
Thompson LD
Am J Surg Pathol 2002 May;26(5):551-66. doi: 10.1097/00000478-200205000-00002. PMID: 11979086
Benign and malignant pheochromocytoma: diagnosis, treatment, and follow-Up.
Kebebew E, Duh QY
Surg Oncol Clin N Am 1998 Oct;7(4):765-89. PMID: 9735133

Recent systematic reviews

Presentation and outcomes of adrenal ganglioneuromas: A cohort study and a systematic review of literature.
Dages KN, Kohlenberg JD, Young WF Jr, Murad MH, Prokop L, Rivera M, Dy B, Foster T, Lyden M, McKenzie T, Thompson G, Bancos I
Clin Endocrinol (Oxf) 2021 Jul;95(1):47-57. Epub 2021 Mar 22 doi: 10.1111/cen.14460. PMID: 33721367Free PMC Article
Discrepancies in the Recommended Management of Adrenal Incidentalomas by Various Guidelines.
Maas M, Nassiri N, Bhanvadia S, Carmichael JD, Duddalwar V, Daneshmand S
J Urol 2021 Jan;205(1):52-59. Epub 2020 Aug 28 doi: 10.1097/JU.0000000000001342. PMID: 32856984
DIAGNOSIS OF ENDOCRINE DISEASE: The diagnostic performance of adrenal biopsy: a systematic review and meta-analysis.
Bancos I, Tamhane S, Shah M, Delivanis DA, Alahdab F, Arlt W, Fassnacht M, Murad MH
Eur J Endocrinol 2016 Aug;175(2):R65-80. Epub 2016 Jun 2 doi: 10.1530/EJE-16-0297. PMID: 27257146
Minimally invasive adrenalectomy in children.
Heloury Y, Muthucumaru M, Panabokke G, Cheng W, Kimber C, Leclair MD
J Pediatr Surg 2012 Feb;47(2):415-21. doi: 10.1016/j.jpedsurg.2011.08.003. PMID: 22325405
Exploration and management of adrenal incidentalomas. French Society of Endocrinology Consensus.
Tabarin A, Bardet S, Bertherat J, Dupas B, Chabre O, Hamoir E, Laurent F, Tenenbaum F, Cazalda M, Lefebvre H, Valli N, Rohmer V; French Society of Endocrinology Consensus
Ann Endocrinol (Paris) 2008 Dec;69(6):487-500. Epub 2008 Nov 20 doi: 10.1016/j.ando.2008.09.003. PMID: 19022420

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