Pheochromocytoma classification
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ahmad Al Maradni, M.D. [2] Mohammed Abdelwahed M.D[3]
Overview
Pheochromocytoma may be classified by nature into benign and malignant and can be classified by spread into local, regional, and metastatic.Another classification by origin is familial, non-familial, and sporadic.
Classification
Pheochromocytoma may be classified by nature either:
- Malignant and benign tumors share the same biochemical and histological characters. The only difference is the ability of the malignant tumor to invade local and distant tissues according to WHO Classification of Tumours in 2004.[1]
- Most cases need to follow up for long durations.
Pheochromocytomas can be classified by spread ability either:
- Localized: 95% of pheochromocytomas are in the abdomen, 85 to 90 % are intra-adrenal and 5 to 10 percent are multiple, 10% are extra-adrenal and are referred to as catecholamine-secreting paragangliomas.
- Regional
- metastatic: to lung, bone, head, thorax, and liver
Pheochromocytoma can be either familial, non-familial, and sporadic:
Familial pheochromocytoma
- They are associated with other syndromes named Multiple endocrine neoplasia 2 (MEN2) Which are autosomal dominant syndromes controlled by RET gene. Pheochromocytoma occurs in 50% of patients with MEN2 as follows:
| MEN1 | MEN2 |
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Non-familial pheochromocytoma:
the majority of them are positive for KIT expression, some are not. A partial explanation was provided by the finding of activating mutations in another gene encoding an RTK, the platelet-derived growth factor receptor alpha (PDGFRA) gene in some KIT-negative GISTs:
Sporadic:
Most catecholamine-secreting tumors are sporadic. Mutations were identified in most of the sporadic cases. May be due to spontaneous mutation, decreased penetrance, maternal imprinting.[2] 50% of patients had a pathogenic mutation in SDHB, SDHD, or VHL.[3]
Pheochromocytoma can be classified on genetic basis into cluster 1 and cluster 2:
| Cluster 1 | Cluster 2 |
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. Patients with the succinate dehydrogenase B mutations are likely to develop a malignant disease.[4]
References
- ↑ Travis WD, Brambilla E, Nicholson AG, Yatabe Y, Austin JH, Beasley MB; et al. (2015). "The 2015 World Health Organization Classification of Lung Tumors: Impact of Genetic, Clinical and Radiologic Advances Since the 2004 Classification". J Thorac Oncol. 10 (9): 1243–60. doi:10.1097/JTO.0000000000000630. PMID 26291008.
- ↑ Buffet A, Venisse A, Nau V, Roncellin I, Boccio V, Le Pottier N; et al. (2012). "A decade (2001-2010) of genetic testing for pheochromocytoma and paraganglioma". Horm Metab Res. 44 (5): 359–66. doi:10.1055/s-0032-1304594. PMID 22517557.
- ↑ Jafri M, Whitworth J, Rattenberry E, Vialard L, Kilby G, Kumar AV; et al. (2013). "Evaluation of SDHB, SDHD and VHL gene susceptibility testing in the assessment of individuals with non-syndromic phaeochromocytoma, paraganglioma and head and neck paraganglioma". Clin Endocrinol (Oxf). 78 (6): 898–906. doi:10.1111/cen.12074. PMID 23072324.