What conditions are associated with pheochromocytoma

What conditions are associated with pheochromocytoma?

• von Recklinghausen disease (neurofibromatosis)

• Tuberous sclerosis (de Bourneville or Pringle disease)

• Sturge-Weber syndrome

• von Hippel-Lindau disease

• Ataxia telangiectasia

• MEN syndromes:

• MEN type 2 (or 2a): pheochromocytoma (usually bilateral, but seldom synchronous), parathyroid adenoma, medullary thyroid carcinoma (which is typically diagnosed first)

• MEN type 3 (or 2b): pheochromocytoma, medullary carcinoma of thyroid, mucosal neuromas, abdominal gangliomas, Marfanoid body habitus (the prevalence of this syndrome is only about 5% of that of MEN 2 or 2a)

• Familial paraganglioma (typically associated with mutations in the succinate dehydrogenase gene)

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