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]
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Overview
Pheochromocytoma can be either benign or malignant and can be localized, regional, or metastatic.
Classification
Pheochromocytoma can be 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 paraganglioma is an autosomal dominant disorder characterized by skull, neck, thorax, abdomen and urinary bladder.
- Non-familial pheochromocytomas include cholelithiasis, renal artery stenosis, (gastrointestinal stromal tumor [GIST], paraganglioma, adrenal cortical adenoma
- Sporadic: Most catecholamine-secreting tumors are sporadic.
Pheochromocytoma can be classified on genetic basis into cluster 1 and cluster 2 :