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Metastatic Adrenal Gland Composite Pheochromocytoma

MedGen UID:
457126
•Concept ID:
C2984900
•
Neoplastic Process
Synonym: Malignant Adrenal Gland Composite Pheochromocytoma

Definition

An adrenal gland composite pheochromocytoma that metastasizes to other anatomic sites. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMetastatic Adrenal Gland Composite Pheochromocytoma

Professional guidelines

PubMed

Utility of GATA3 in the differential diagnosis of pheochromocytoma.
Perrino CM, Ho A, Dall CP, Zynger DL
Histopathology 2017 Sep;71(3):475-479. Epub 2017 Jun 5 doi: 10.1111/his.13229. PMID: 28374498
Differential diagnosis of pheochromocytomas and paragangliomas.
McNichol AM
Endocr Pathol 2001 Winter;12(4):407-15. doi: 10.1385/ep:12:4:407. PMID: 11914474

Recent clinical studies

Etiology

Advances in Adrenal and Extra-adrenal Paraganglioma: Practical Synopsis for Pathologists.
Juhlin CC, Mete O
Adv Anat Pathol 2023 Jan 1;30(1):47-57. Epub 2022 Sep 23 doi: 10.1097/PAP.0000000000000365. PMID: 36136370
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
The utility of (68)Ga-DOTATATE PET/CT in localizing primary/metastatic pheochromocytoma and paraganglioma in children and adolescents - a single-center experience.
Jaiswal SK, Sarathi V, Malhotra G, Hira P, Shah R, Patil VA, Dalvi A, Prakash G, Lila AR, Shah NS, Bandgar T
J Pediatr Endocrinol Metab 2021 Jan 27;34(1):109-119. Epub 2020 Nov 12 doi: 10.1515/jpem-2020-0354. PMID: 33180042
Update on Adrenal Tumours in 2017 World Health Organization (WHO) of Endocrine Tumours.
Lam AK
Endocr Pathol 2017 Sep;28(3):213-227. doi: 10.1007/s12022-017-9484-5. PMID: 28477311
Utility of GATA3 in the differential diagnosis of pheochromocytoma.
Perrino CM, Ho A, Dall CP, Zynger DL
Histopathology 2017 Sep;71(3):475-479. Epub 2017 Jun 5 doi: 10.1111/his.13229. PMID: 28374498

Diagnosis

Advances in Adrenal and Extra-adrenal Paraganglioma: Practical Synopsis for Pathologists.
Juhlin CC, Mete O
Adv Anat Pathol 2023 Jan 1;30(1):47-57. Epub 2022 Sep 23 doi: 10.1097/PAP.0000000000000365. PMID: 36136370
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
Utility of GATA3 in the differential diagnosis of pheochromocytoma.
Perrino CM, Ho A, Dall CP, Zynger DL
Histopathology 2017 Sep;71(3):475-479. Epub 2017 Jun 5 doi: 10.1111/his.13229. PMID: 28374498
Composite pheochromocytoma with a malignant peripheral nerve sheath tumor: Case report and review of the literature.
Namekawa T, Utsumi T, Imamoto T, Kawamura K, Oide T, Tanaka T, Nihei N, Suzuki H, Nakatani Y, Ichikawa T
Asian J Surg 2016 Jul;39(3):187-90. Epub 2013 Feb 20 doi: 10.1016/j.asjsur.2012.11.003. PMID: 27338175
Differential diagnosis of pheochromocytomas and paragangliomas.
McNichol AM
Endocr Pathol 2001 Winter;12(4):407-15. doi: 10.1385/ep:12:4:407. PMID: 11914474

Therapy

The utility of (68)Ga-DOTATATE PET/CT in localizing primary/metastatic pheochromocytoma and paraganglioma in children and adolescents - a single-center experience.
Jaiswal SK, Sarathi V, Malhotra G, Hira P, Shah R, Patil VA, Dalvi A, Prakash G, Lila AR, Shah NS, Bandgar T
J Pediatr Endocrinol Metab 2021 Jan 27;34(1):109-119. Epub 2020 Nov 12 doi: 10.1515/jpem-2020-0354. PMID: 33180042
Pheochromocytoma with histologic transformation to composite type, complicated by watery diarrhea, hypokalemia, and achlorhydria syndrome.
Kikuchi Y, Wada R, Sakihara S, Suda T, Yagihashi S
Endocr Pract 2012 Jul-Aug;18(4):e91-6. doi: 10.4158/EP11370.CR. PMID: 22440998

Prognosis

Composite pheochromocytoma of the adrenal gland-a review of published cases.
Costa J, Jácome M, Souteiro P, Santos C, Cunha AL
Virchows Arch 2023 Aug;483(2):267-275. Epub 2023 Jan 16 doi: 10.1007/s00428-023-03492-y. PMID: 36645463
Advances in Adrenal and Extra-adrenal Paraganglioma: Practical Synopsis for Pathologists.
Juhlin CC, Mete O
Adv Anat Pathol 2023 Jan 1;30(1):47-57. Epub 2022 Sep 23 doi: 10.1097/PAP.0000000000000365. PMID: 36136370
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
Update on Adrenal Tumours in 2017 World Health Organization (WHO) of Endocrine Tumours.
Lam AK
Endocr Pathol 2017 Sep;28(3):213-227. doi: 10.1007/s12022-017-9484-5. PMID: 28477311
Composite pheochromocytoma with a malignant peripheral nerve sheath tumor: Case report and review of the literature.
Namekawa T, Utsumi T, Imamoto T, Kawamura K, Oide T, Tanaka T, Nihei N, Suzuki H, Nakatani Y, Ichikawa T
Asian J Surg 2016 Jul;39(3):187-90. Epub 2013 Feb 20 doi: 10.1016/j.asjsur.2012.11.003. PMID: 27338175

Clinical prediction guides

Validation and Evaluation of 5 Scoring Systems for Predicting Metastatic Risk in Pheochromocytoma and Paraganglioma.
Li Q, Lan Z, Jiang Y, Wang R, Li Z, Jiang X
Am J Surg Pathol 2024 Jul 1;48(7):855-865. Epub 2024 May 7 doi: 10.1097/PAS.0000000000002238. PMID: 38712603
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
COPPS, a composite score integrating pathological features, PS100 and SDHB losses, predicts the risk of metastasis and progression-free survival in pheochromocytomas/paragangliomas.
Pierre C, Agopiantz M, Brunaud L, Battaglia-Hsu SF, Max A, Pouget C, Nomine C, Lomazzi S, Vignaud JM, Weryha G, Oussalah A, Gauchotte G, Busby-Venner H
Virchows Arch 2019 Jun;474(6):721-734. Epub 2019 Mar 13 doi: 10.1007/s00428-019-02553-5. PMID: 30868297
Update on Adrenal Tumours in 2017 World Health Organization (WHO) of Endocrine Tumours.
Lam AK
Endocr Pathol 2017 Sep;28(3):213-227. doi: 10.1007/s12022-017-9484-5. PMID: 28477311
Localization and prediction of malignant potential in recurrent pheochromocytoma/paraganglioma (PCC/PGL) using 18F-FDG PET/CT.
Fikri AS, Kroiss A, Ahmad AZ, Zanariah H, Lau WF, Uprimny C, Donnemiller E, Kendler D, Nordin AJ, Virgolini IJ
Acta Radiol 2014 Jun;55(5):631-40. Epub 2013 Sep 14 doi: 10.1177/0284185113504330. PMID: 24037430

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