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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 chromaffin tumour; 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

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
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
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

Recent clinical studies

Etiology

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
Lenders JWM, Kerstens MN, Amar L, Prejbisz A, Robledo M, Taieb D, Pacak K, Crona J, Zelinka T, Mannelli M, Deutschbein T, Timmers HJLM, Castinetti F, Dralle H, Widimský J, Gimenez-Roqueplo AP, Eisenhofer G
J Hypertens 2020 Aug;38(8):1443-1456. doi: 10.1097/HJH.0000000000002438. PMID: 32412940Free PMC Article
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
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
Thompson LD
Am J Surg Pathol 2002 May;26(5):551-66. doi: 10.1097/00000478-200205000-00002. PMID: 11979086

Diagnosis

Kebebew E
N Engl J Med 2021 Apr 22;384(16):1542-1551. doi: 10.1056/NEJMcp2031112. PMID: 33882207
Pappachan JM, Tun NN, Arunagirinathan G, Sodi R, Hanna FWF
Curr Hypertens Rep 2018 Jan 22;20(1):3. doi: 10.1007/s11906-018-0804-z. PMID: 29356966
Mendiratta-Lala M, Avram A, Turcu AF, Dunnick NR
Endocrinol Metab Clin North Am 2017 Sep;46(3):741-759. Epub 2017 Jun 12 doi: 10.1016/j.ecl.2017.04.009. PMID: 28760236
Gunawardane PTK, Grossman A
Adv Exp Med Biol 2017;956:239-259. doi: 10.1007/5584_2016_76. PMID: 27888488
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

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
Corssmit EPM, Snel M, Kapiteijn E
Curr Opin Oncol 2020 Jan;32(1):20-26. doi: 10.1097/CCO.0000000000000589. PMID: 31599769
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
Bravo EL
Cardiol Rev 2002 Jan-Feb;10(1):44-50. doi: 10.1097/00045415-200201000-00009. PMID: 11790269
Eigelberger MS, Duh QY
Curr Treat Options Oncol 2001 Aug;2(4):321-9. doi: 10.1007/s11864-001-0025-5. PMID: 12057112

Prognosis

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
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
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
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
Thompson LD
Am J Surg Pathol 2002 May;26(5):551-66. doi: 10.1097/00000478-200205000-00002. PMID: 11979086

Clinical prediction guides

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
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
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
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
Thompson LD
Am J Surg Pathol 2002 May;26(5):551-66. doi: 10.1097/00000478-200205000-00002. PMID: 11979086

Recent systematic reviews

Ganni R, Torpy DJ, Falhammar H, Louise Rushworth R
J Clin Endocrinol Metab 2023 Aug 18;108(9):e885-e892. doi: 10.1210/clinem/dgad121. PMID: 36896586Free PMC Article
Sconfienza E, Tetti M, Forestiero V, Veglio F, Mulatero P, Monticone S
J Clin Endocrinol Metab 2023 Jun 16;108(7):1813-1823. doi: 10.1210/clinem/dgad044. PMID: 36718682
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
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
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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