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Malignant adrenal gland pheochromocytoma

MedGen UID:
87238
•Concept ID:
C0334419
•
Neoplastic Process
Synonyms: adrenal gland pheochromocytoma, malignant; Adrenal medullary paraganglioma, malignant; Malignant Adrenal Gland Chromaffin Neoplasm; malignant adrenal gland chromaffin neoplasm; Malignant Adrenal Gland Chromaffin Paraganglioma; malignant adrenal gland chromaffin paraganglioma; Malignant Adrenal Gland Chromaffin Tumor; malignant adrenal gland chromaffin tumor; Malignant Adrenal Gland Chromaffinoma; malignant adrenal gland Chromaffinoma; Malignant Adrenal Gland Paraganglioma; malignant adrenal gland paraganglioma; Malignant Adrenal Gland Pheochromocytoma; malignant adrenal gland pheochromocytoma; Malignant Adrenal Medullary Paraganglioma; malignant adrenal medullary paraganglioma; Malignant Adrenal Medullary Pheochromocytoma; malignant adrenal medullary pheochromocytoma; Malignant Adrenal Pheochromocytoma; malignant adrenal pheochromocytoma; Malignant Pheochromocytoma; Malignant pheochromocytoma; malignant pheochromocytoma; Metastatic Adrenal Gland Pheochromocytoma; Metastatic Pheochromocytoma; Pheochromoblastoma; pheochromoblastoma; Pheochromocytoma, malignant; pheochromocytoma, malignant
SNOMED CT: Malignant pheochromocytoma (29370006); Adrenal medullary paraganglioma, malignant (29370006); Malignant pheochromocytoma (21851000119103); Pheochromocytoma, malignant (29370006); Pheochromoblastoma (29370006)
 
Monarch Initiative: MONDO:0006288

Definition

A pheochromocytoma that metastasizes to other anatomic sites. Common sites of metastasis include lymph nodes, bones, liver, and lung. Morphologic features associated with malignant pheochromocytomas include: atypical mitotic figures, capsular and vascular invasion, tumor cell necrosis, and high mitotic activity. [from NCI]

Term Hierarchy

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

Professional guidelines

PubMed

Personalized Management of Pheochromocytoma and Paraganglioma.
Nölting S, Bechmann N, Taieb D, Beuschlein F, Fassnacht M, Kroiss M, Eisenhofer G, Grossman A, Pacak K
Endocr Rev 2022 Mar 9;43(2):199-239. doi: 10.1210/endrev/bnab019. PMID: 34147030Free PMC Article
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 and paraganglioma: an endocrine society clinical practice guideline.
Lenders JW, Duh QY, Eisenhofer G, Gimenez-Roqueplo AP, Grebe SK, Murad MH, Naruse M, Pacak K, Young WF Jr; Endocrine Society
J Clin Endocrinol Metab 2014 Jun;99(6):1915-42. doi: 10.1210/jc.2014-1498. PMID: 24893135

Recent clinical studies

Etiology

Adrenal pheochromocytoma: Keys to radiologic diagnosis.
Corral de la Calle MA, Encinas de la Iglesia J, Fernández-Pérez GC, Repollés Cobaleda M, Fraino A
Radiologia (Engl Ed) 2022 Jul-Aug;64(4):348-367. doi: 10.1016/j.rxeng.2022.07.002. PMID: 36030082
Personalized Management of Pheochromocytoma and Paraganglioma.
Nölting S, Bechmann N, Taieb D, Beuschlein F, Fassnacht M, Kroiss M, Eisenhofer G, Grossman A, Pacak K
Endocr Rev 2022 Mar 9;43(2):199-239. doi: 10.1210/endrev/bnab019. PMID: 34147030Free PMC Article
Pheochromocytoma and paraganglioma-an update on diagnosis, evaluation, and management.
Jain A, Baracco R, Kapur G
Pediatr Nephrol 2020 Apr;35(4):581-594. Epub 2019 Jan 2 doi: 10.1007/s00467-018-4181-2. PMID: 30603807
Pheochromocytoma, diagnosis and treatment: Review of the literature.
Farrugia FA, Martikos G, Tzanetis P, Charalampopoulos A, Misiakos E, Zavras N, Sotiropoulos D
Endocr Regul 2017 Jul 1;51(3):168-181. doi: 10.1515/enr-2017-0018. PMID: 28858847
Phaeochromocytoma and Paraganglioma.
Gunawardane PTK, Grossman A
Adv Exp Med Biol 2017;956:239-259. doi: 10.1007/5584_2016_76. PMID: 27888488

Diagnosis

Adrenal pheochromocytoma: Keys to radiologic diagnosis.
Corral de la Calle MA, Encinas de la Iglesia J, Fernández-Pérez GC, Repollés Cobaleda M, Fraino A
Radiologia (Engl Ed) 2022 Jul-Aug;64(4):348-367. doi: 10.1016/j.rxeng.2022.07.002. PMID: 36030082
Adrenal Incidentaloma.
Kebebew E
N Engl J Med 2021 Apr 22;384(16):1542-1551. doi: 10.1056/NEJMcp2031112. PMID: 33882207
Phaeochromocytoma and Paraganglioma.
Gunawardane PTK, Grossman A
Adv Exp Med Biol 2017;956:239-259. doi: 10.1007/5584_2016_76. PMID: 27888488
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
Pheochromocytoma.
Manger WM, Gifford RW
J Clin Hypertens (Greenwich) 2002 Jan-Feb;4(1):62-72. doi: 10.1111/j.1524-6175.2002.01452.x. PMID: 11821644Free PMC Article

Therapy

Targeted Therapies in Pheochromocytoma and Paraganglioma.
Wang K, Crona J, Beuschlein F, Grossman AB, Pacak K, Nölting S
J Clin Endocrinol Metab 2022 Nov 23;107(11):2963-2972. doi: 10.1210/clinem/dgac471. PMID: 35973976Free PMC Article
Personalized drug testing in human pheochromocytoma/paraganglioma primary cultures.
Wang K, Schütze I, Gulde S, Bechmann N, Richter S, Helm J, Lauseker M, Maurer J, Reul A, Spoettl G, Klink B, William D, Knösel T, Friemel J, Bihl M, Weber A, Fankhauser M, Schober L, Vetter D, Broglie Däppen M, Ziegler CG, Ullrich M, Pietzsch J, Bornstein SR, Lottspeich C, Kroiss M, Fassnacht M, Wenter VUJ, Ladurner R, Hantel C, Reincke M, Eisenhofer G, Grossman AB, Pacak K, Beuschlein F, Auernhammer CJ, Pellegata NS, Nölting S
Endocr Relat Cancer 2022 May 9;29(6):285-306. doi: 10.1530/ERC-21-0355. PMID: 35324454
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
Pheochromocytoma, diagnosis and treatment: Review of the literature.
Farrugia FA, Martikos G, Tzanetis P, Charalampopoulos A, Misiakos E, Zavras N, Sotiropoulos D
Endocr Regul 2017 Jul 1;51(3):168-181. doi: 10.1515/enr-2017-0018. PMID: 28858847
Adrenal autotransplantation.
Srougi M, Gittes RF
Urol Surv 1978 Apr;28(2):41-8. PMID: 209598

Prognosis

Personalized Management of Pheochromocytoma and Paraganglioma.
Nölting S, Bechmann N, Taieb D, Beuschlein F, Fassnacht M, Kroiss M, Eisenhofer G, Grossman A, Pacak K
Endocr Rev 2022 Mar 9;43(2):199-239. doi: 10.1210/endrev/bnab019. PMID: 34147030Free PMC Article
Metastatic Pheochromocytomas and Abdominal Paragangliomas.
Granberg D, Juhlin CC, Falhammar H
J Clin Endocrinol Metab 2021 Apr 23;106(5):e1937-e1952. doi: 10.1210/clinem/dgaa982. PMID: 33462603Free PMC Article
Predicting Metastatic Potential in Pheochromocytoma and Paraganglioma: A Comparison of PASS and GAPP Scoring Systems.
Wachtel H, Hutchens T, Baraban E, Schwartz LE, Montone K, Baloch Z, LiVolsi V, Krumeich L, Fraker DL, Nathanson KL, Cohen DL, Fishbein L
J Clin Endocrinol Metab 2020 Dec 1;105(12):e4661-70. doi: 10.1210/clinem/dgaa608. PMID: 32877928Free 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
Phaeochromocytoma and Paraganglioma.
Gunawardane PTK, Grossman A
Adv Exp Med Biol 2017;956:239-259. doi: 10.1007/5584_2016_76. PMID: 27888488

Clinical prediction guides

Pediatric Metastatic Pheochromocytoma and Paraganglioma: Clinical Presentation and Diagnosis, Genetics, and Therapeutic Approaches.
Kuo MJM, Nazari MA, Jha A, Pacak K
Front Endocrinol (Lausanne) 2022;13:936178. Epub 2022 Jul 12 doi: 10.3389/fendo.2022.936178. PMID: 35903274Free PMC Article
Metastatic Pheochromocytomas and Abdominal Paragangliomas.
Granberg D, Juhlin CC, Falhammar H
J Clin Endocrinol Metab 2021 Apr 23;106(5):e1937-e1952. doi: 10.1210/clinem/dgaa982. PMID: 33462603Free PMC Article
Predicting Metastatic Potential in Pheochromocytoma and Paraganglioma: A Comparison of PASS and GAPP Scoring Systems.
Wachtel H, Hutchens T, Baraban E, Schwartz LE, Montone K, Baloch Z, LiVolsi V, Krumeich L, Fraker DL, Nathanson KL, Cohen DL, Fishbein L
J Clin Endocrinol Metab 2020 Dec 1;105(12):e4661-70. doi: 10.1210/clinem/dgaa608. PMID: 32877928Free PMC Article
Clinical Characterization of the Pheochromocytoma and Paraganglioma Susceptibility Genes SDHA, TMEM127, MAX, and SDHAF2 for Gene-Informed Prevention.
Bausch B, Schiavi F, Ni Y, Welander J, Patocs A, Ngeow J, Wellner U, Malinoc A, Taschin E, Barbon G, Lanza V, Söderkvist P, Stenman A, Larsson C, Svahn F, Chen JL, Marquard J, Fraenkel M, Walter MA, Peczkowska M, Prejbisz A, Jarzab B, Hasse-Lazar K, Petersenn S, Moeller LC, Meyer A, Reisch N, Trupka A, Brase C, Galiano M, Preuss SF, Kwok P, Lendvai N, Berisha G, Makay Ö, Boedeker CC, Weryha G, Racz K, Januszewicz A, Walz MK, Gimm O, Opocher G, Eng C, Neumann HPH; European-American-Asian Pheochromocytoma-Paraganglioma Registry Study Group
JAMA Oncol 2017 Sep 1;3(9):1204-1212. doi: 10.1001/jamaoncol.2017.0223. PMID: 28384794Free PMC Article
Phaeochromocytoma and Paraganglioma.
Gunawardane PTK, Grossman A
Adv Exp Med Biol 2017;956:239-259. doi: 10.1007/5584_2016_76. PMID: 27888488

Recent systematic reviews

Comparison of the effectiveness and safety of robotic-assisted and laparoscopic in adrenalectomy: A systematic review and meta-analysis.
Gan L, Peng L, Li J, Meng C, Li K, Wu J, Zhang Z, Li Y
Int J Surg 2022 Sep;105:106853. Epub 2022 Sep 6 doi: 10.1016/j.ijsu.2022.106853. PMID: 36075556
Safety and effectiveness of minimally invasive adrenalectomy versus open adrenalectomy in patients with large adrenal tumors (≥5 cm): A meta-analysis and systematic review.
Gan L, Meng C, Li K, Lei Peng, Li J, Wu J, Li Y
Int J Surg 2022 Aug;104:106779. Epub 2022 Jul 20 doi: 10.1016/j.ijsu.2022.106779. PMID: 35870758
Maternal and fetal outcomes in phaeochromocytoma and pregnancy: a multicentre retrospective cohort study and systematic review of literature.
Bancos I, Atkinson E, Eng C, Young WF Jr, Neumann HPH; International Pheochromocytoma and Pregnancy Study Group
Lancet Diabetes Endocrinol 2021 Jan;9(1):13-21. Epub 2020 Nov 26 doi: 10.1016/S2213-8587(20)30363-6. PMID: 33248478Free PMC Article
The natural progression and outcomes of adrenal incidentaloma: a systematic review and meta-analysis.
Loh HH, Yee A, Loh HS, Sukor N, Kamaruddin NA
Minerva Endocrinol 2017 Mar;42(1):77-87. Epub 2015 Dec 23 doi: 10.23736/S0391-1977.16.02394-4. PMID: 26698544
Acute and Chronic Pheochromocytoma-Induced Cardiomyopathies: Different Prognoses?: A Systematic Analytical Review.
Batisse-Lignier M, Pereira B, Motreff P, Pierrard R, Burnot C, Vorilhon C, Maqdasy S, Roche B, Desbiez F, Clerfond G, Citron B, Lusson JR, Tauveron I, Eschalier R
Medicine (Baltimore) 2015 Dec;94(50):e2198. doi: 10.1097/MD.0000000000002198. PMID: 26683930Free PMC Article

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