pheochromocytoma/paraganglioma syndrome 4

A paraganglioma characterized by the development of neuroendocrine tumors, usually in adulthood that has material_basis_in heterozygous mutation in the SDHB gene, which encodes the iron sulfur subunit of succinate dehydrogenase, on chromosome 1p36.

Signs and symptoms

• Extraadrenal pheochromocytoma
• Cranial nerve paralysis
• Recurrent paroxysmal headache
• Pulsatile tinnitus
• Hypertension associated with pheochromocytoma
• Glomus jugular tumor
• Renal cell carcinoma
• Elevated urinary catecholamine level
• Hyperhidrosis
• Tachycardia