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Adrenal Gland Composite Pheochromocytoma

MedGen UID:
317355
•Concept ID:
C1706728
•
Neoplastic Process

Definition

A neoplasm that arises from the adrenal medulla and combines morphologic characteristics of pheochromocytoma and neuroectodermal tumors such as neuroblastoma, ganglioneuroma, ganglioneuroblastoma, or peripheral nerve sheath tumor. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAdrenal Gland Composite Pheochromocytoma

Professional guidelines

PubMed

Impact of body composition and genotype on haemodynamics during surgery for pheochromocytoma and paraganglioma.
Pang Y, Li M, Jiang J, Chen X, Fu Y, Wang C, He Y, Zhao Y, Wang Y, Guan X, Zhang L, Xu X, Gan Y, Liu Y, Xie Y, Tang T, Wang J, Xie B, Liang Z, Chen D, Liu H, Chen C, Eisenhofer G, Liu L, Yi X, Chen BT
J Cachexia Sarcopenia Muscle 2022 Dec;13(6):2843-2853. Epub 2022 Sep 6 doi: 10.1002/jcsm.13071. PMID: 36068986Free PMC Article
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
Impact of body composition and genotype on haemodynamics during surgery for pheochromocytoma and paraganglioma.
Pang Y, Li M, Jiang J, Chen X, Fu Y, Wang C, He Y, Zhao Y, Wang Y, Guan X, Zhang L, Xu X, Gan Y, Liu Y, Xie Y, Tang T, Wang J, Xie B, Liang Z, Chen D, Liu H, Chen C, Eisenhofer G, Liu L, Yi X, Chen BT
J Cachexia Sarcopenia Muscle 2022 Dec;13(6):2843-2853. Epub 2022 Sep 6 doi: 10.1002/jcsm.13071. PMID: 36068986Free 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
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
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

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
Composite pheochromocytoma with spindle cell sarcoma - a rare tumor of the adrenal gland: case report.
Turk Y, Karimov Z, Ozdemir M, Ertan Y, Makay O
Hormones (Athens) 2022 Sep;21(3):507-511. Epub 2022 Mar 18 doi: 10.1007/s42000-022-00363-0. PMID: 35304732
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
Adrenal pheochromocytoma: is it all or the tip of the iceberg?
Wang K, Tang G, Peng Y, Li C, Fu W, Li R, Guan J
Jpn J Radiol 2022 Feb;40(2):120-134. Epub 2021 Sep 21 doi: 10.1007/s11604-021-01199-1. PMID: 34546504
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

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
Change of skeletal muscle mass in patients with pheochromocytoma.
Lee SH, Kwak MK, Ahn SH, Kim H, Cho YY, Suh S, Song KH, Koh JM, Kim JH, Kim BJ
J Bone Miner Metab 2019 Jul;37(4):694-702. Epub 2018 Sep 20 doi: 10.1007/s00774-018-0959-3. PMID: 30238430
Changes in energy metabolism in pheochromocytoma.
Petrák O, Haluzíková D, Kaválková P, Štrauch B, Rosa J, Holaj R, Brabcová Vránková A, Michalsky D, Haluzík M, Zelinka T, Widimsky J Jr
J Clin Endocrinol Metab 2013 Apr;98(4):1651-8. Epub 2013 Feb 22 doi: 10.1210/jc.2012-3625. PMID: 23436923
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
Impact of body composition and genotype on haemodynamics during surgery for pheochromocytoma and paraganglioma.
Pang Y, Li M, Jiang J, Chen X, Fu Y, Wang C, He Y, Zhao Y, Wang Y, Guan X, Zhang L, Xu X, Gan Y, Liu Y, Xie Y, Tang T, Wang J, Xie B, Liang Z, Chen D, Liu H, Chen C, Eisenhofer G, Liu L, Yi X, Chen BT
J Cachexia Sarcopenia Muscle 2022 Dec;13(6):2843-2853. Epub 2022 Sep 6 doi: 10.1002/jcsm.13071. PMID: 36068986Free 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
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

Clinical prediction guides

Ensemble Machine Learning Model Incorporating Radiomics and Body Composition for Predicting Intraoperative HDI in PPGL.
Fu Y, Wang X, Yi X, Guan X, Chen C, Han Z, Gong G, Yin H, Liu L, Chen BT
J Clin Endocrinol Metab 2024 Jan 18;109(2):351-360. doi: 10.1210/clinem/dgad543. PMID: 37708346
Impact of body composition and genotype on haemodynamics during surgery for pheochromocytoma and paraganglioma.
Pang Y, Li M, Jiang J, Chen X, Fu Y, Wang C, He Y, Zhao Y, Wang Y, Guan X, Zhang L, Xu X, Gan Y, Liu Y, Xie Y, Tang T, Wang J, Xie B, Liang Z, Chen D, Liu H, Chen C, Eisenhofer G, Liu L, Yi X, Chen BT
J Cachexia Sarcopenia Muscle 2022 Dec;13(6):2843-2853. Epub 2022 Sep 6 doi: 10.1002/jcsm.13071. PMID: 36068986Free PMC Article
The immune cell infiltrate in the tumour microenvironment of phaeochromocytomas and paragangliomas.
Tufton N, Hearnden RJ, Berney DM, Drake WM, Parvanta L, Chapple JP, Akker SA
Endocr Relat Cancer 2022 Nov 1;29(11):589-598. Epub 2022 Sep 19 doi: 10.1530/ERC-22-0020. PMID: 35975974Free 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
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

Recent systematic reviews

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
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

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