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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

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
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 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

Diagnosis and Management of Pheochromocytomas and Paragangliomas: A Guide for the Clinician.
Sharma S, Fishbein L
Endocr Pract 2023 Dec;29(12):999-1006. Epub 2023 Aug 15 doi: 10.1016/j.eprac.2023.07.027. PMID: 37586639
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
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 Incidentaloma.
Kebebew E
N Engl J Med 2021 Apr 22;384(16):1542-1551. doi: 10.1056/NEJMcp2031112. PMID: 33882207
Adrenal Imaging.
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
Phaeochromocytoma and Paraganglioma.
Gunawardane PTK, Grossman A
Adv Exp Med Biol 2017;956:239-259. doi: 10.1007/5584_2016_76. PMID: 27888488
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

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
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
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
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

Clinical prediction guides

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
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
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

Recent systematic reviews

Adrenal Medullary Hyperplasia: A Systematic Review and Meta-analysis.
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
Silent pheochromocytoma and paraganglioma: Systematic review and proposed definitions for standardized terminology.
Constantinescu G, Preda C, Constantinescu V, Siepmann T, Bornstein SR, Lenders JWM, Eisenhofer G, Pamporaki C
Front Endocrinol (Lausanne) 2022;13:1021420. Epub 2022 Oct 17 doi: 10.3389/fendo.2022.1021420. PMID: 36325453Free PMC Article
Composite phaeochromocytomas-a systematic review of published literature.
Dhanasekar K, Visakan V, Tahir F, Balasubramanian SP
Langenbecks Arch Surg 2022 Mar;407(2):517-527. Epub 2021 Mar 2 doi: 10.1007/s00423-021-02129-5. PMID: 33651160Free PMC Article
Ectopic ACTH- and/or CRH-Producing Pheochromocytomas.
Elliott PF, Berhane T, Ragnarsson O, Falhammar H
J Clin Endocrinol Metab 2021 Jan 23;106(2):598-608. doi: 10.1210/clinem/dgaa488. PMID: 32710791
European Society of Endocrinology Clinical Practice Guideline for long-term follow-up of patients operated on for a phaeochromocytoma or a paraganglioma.
Plouin PF, Amar L, Dekkers OM, Fassnacht M, Gimenez-Roqueplo AP, Lenders JW, Lussey-Lepoutre C, Steichen O; Guideline Working Group
Eur J Endocrinol 2016 May;174(5):G1-G10. doi: 10.1530/EJE-16-0033. PMID: 27048283

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