Pheochromocytoma risk factors

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mohammed Abdelwahed M.D[2]

Overview

Pheochromocytoma is more common in third decades of life, people with family history of multiple endocrine neoplasias, Von Hippel-Lindau disease, neurofibromatosis type 1, hereditary paraganglioma syndromes.

Risk Factors

The most potent risk factors of pheochromocytoma are:

Age

• Occurs in third decades of life; the average age at diagnosis is 24.9 years in hereditary cases and 43.9 years in sporadic cases.

• Hereditary tumors present at a younger age than sporadic.

Family history

Ten percent of pheochromocytomas are linked to hereditary causes:

MEN1	MEN2
Medullary thyroid cancer Pheochromocytoma Primary hyperparathyroidism	Medullary thyroid cancer Pheochromocytoma Mucosal neuromas Marfanoid habitus

• Von Hippel-Lindau disease can result in tumors at multiple sites, including the central nervous system, endocrine system, pancreas and kidneys.
• Neurofibromatosis 1 results in multiple tumors in the skin (neurofibromas), pigmented skin spots and tumors of the optic nerve.
• Hereditary paraganglioma syndromes are inherited disorders that result in either pheochromocytomas or paragangliomas.