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. 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
Medullary thyroid cancer, Pheochromocytoma Primary hyperparathyroidism	Medullary thyroid cancer Pheochromocytoma Mucosal neuromas Marfanoid habitus

• 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 :

Cluster 1 tumorsare noradrenergic. Cluster 2 tumors are adrenergic.^[3]

Cluster 1	Cluster 2
Succinate dehydrogenase (SDH) subunit genes Von Hippel-Lindau (VHL) disease Fumarate hydratase gene mutations	Multiple endocrine neoplasia type 2A Multiple endocrine neoplasia type 2B Neurofibromatosis type 1 (NF1)

. Patients with the succinate dehydrogenase B mutations are likely to develop malignant disease.^[4]

References

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