Pheochromocytoma remains a clinical challenge to diagnose and manage. In addition, the association of
multiple endocrine neoplasia syndromes with
pheochromocytoma require the clinician's awareness to evaluate patients with
pheochromocytoma (especially when bilateral) for abnormalities in thyroidal C-cell function with serum
calcitonin determinations. The authors present a case of a 42-year-old woman initially diagnosed with, and treated for, cranial artery
vasculitis because she had a
stroke and a history of
rheumatoid arthritis and
asthma. Subsequent evaluation of episodic blood pressure increases,
headache, and
tachycardia revealed biochemical evidence of
catecholamine overproduction. Bilateral adrenal masses were found on computed tomography scanning, and the functional nature of the adrenal masses was confirmed by a
meta-Iodobenzylguanidine scan. Upon further evaluation, an elevated serum
calcitonin concentration was demonstrated, which increased greatly with
pentagastrin stimulation. C-cell
hyperplasia was demonstrated by subsequent
thyroidectomy, confirming the diagnosis of
multiple endocrine neoplasia 2A. The difficulty in arriving at a correct diagnosis, the subsequent management, including bilateral
adrenalectomy and
thyroidectomy, and newer insight into the genetic abnormalities of
multiple endocrine neoplasia 2A are discussed.