Abstract
Introduction: Pheochromocytomas and paragangliomas are rare neuroendocrine tumors arising from the chromaffin cells of the adrenal medulla or extra-adrenal para-ganglionic tissues. 10% to 20% of tumors are detected before the age of 18 years. Approximately 20%–50% of these tumors are malignant. Up to 24% of the cases may be associated with genetic syndromes like MEN 2, succinate dehydrogenase subunit B (SDHB) gene mutations and VHL syndrome. SDHB is one of the four subunits of the SDH complex. Germline mutations in SDHB gene increase the risk of PGLs/PHEOs and RCC with 38%-83% risk of malignancy.
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