Pheochromocytoma classification: Difference between revisions
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==Classification== | ==Classification== | ||
=== '''Pheochromocytoma can be either [[benign]] or [[malignant]]:''' === | === '''Pheochromocytoma can be classified either [[benign]] or [[malignant]]:''' === | ||
Malignant and benign tumours are the same on biochemical and histological bases. The only difference is the ability of the malignant one to invade local and distatant tissues (pathology and Genetics of Tumours of the Endocrine Organs. WHO Classification of Tumours, DeLellis RA, Lloyd RV, Heitz PU, Eng C. (Eds), IARC press, Lyon, France 2004). So, most cases need follow up for long durations. 10% of pheochromocytomas are malignant. | Malignant and benign tumours are the same on biochemical and histological bases. The only difference is the ability of the malignant one to invade local and distatant tissues (pathology and Genetics of Tumours of the Endocrine Organs. WHO Classification of Tumours, DeLellis RA, Lloyd RV, Heitz PU, Eng C. (Eds), IARC press, Lyon, France 2004). So, most cases need follow up for long durations. 10% of pheochromocytomas are malignant. | ||
Revision as of 17:24, 7 July 2017
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Risk calculators and risk factors for Pheochromocytoma classification |
Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Ahmad Al Maradni, M.D. [2]
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Overview
Pheochromocytoma can be either benign or malignant and can be localized, regional, or metastatic. It can be familial, non-familial and sporadic.
Classification
Pheochromocytoma can be classified either benign or malignant:
Malignant and benign tumours are the same on biochemical and histological bases. The only difference is the ability of the malignant one to invade local and distatant tissues (pathology and Genetics of Tumours of the Endocrine Organs. WHO Classification of Tumours, DeLellis RA, Lloyd RV, Heitz PU, Eng C. (Eds), IARC press, Lyon, France 2004). So, most cases need follow up for long durations. 10% of pheochromocytomas are malignant.
Pheochromocytomas can be localized, regional, or metastatic:
- 95 *% of pheochromocytomas are in the abdomen, 85 to 90 % are intraadrenal and 5 to 10 percent are multiple, 10% are extraadrenal and are referred to as catecholamine-secreting paragangliomas.
Pheochromocytoma can be either familial,non-familial or sporadic:
Familial pheochromocytoma is an autosomal dominant disorder characterized by skull, neck, thorax, abdomen and urinary bladder. They are associated with other syndromes named Multiple endocrien neoplasia (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 pheochromocytomas: include cholelithiasis, renal artery stenosis, (gastrointestinal stromal tumor [GIST], paraganglioma, adrenal cortical adenoma 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 sporadic cases. May be due to spontaneous mutation, decreased penetrance, maternal imprinting.[1] 50% of patients had a pathogenic mutation in SDHB, SDHD, or VHL[2]
Pheochromocytoma can be classified on genetic basis into cluster 1 and cluster 2 :
Cluster 1 tumorsare noradrenergic. Cluster 2 tumors are adrenergic.[3]
| Cluster 1 | Cluster 2 |
|---|---|
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. Patients with the succinate dehydrogenase B mutations are likely to develop malignant disease.[4]
References
- ↑ 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.