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Abdominal CT scan with and without IV contrast
23%
5/22
24-hour urine free cortisol
0%
0/22
High dose dexamethasone suppression test
Plasma fractionated metanephrines
64%
14/22
Plasma aldosterone/renin ratio
5%
1/22
Select Answer to see Preferred Response
This patient with a familial and personal history of thyroid cancer, is now presenting with periods of diaphoresis, anxiety, palpitations, concerning for elevated catecholamines secondary to a pheochromocytoma in the setting of Multiple Endocrine Neoplasia Type II (MEN II). The first line test for diagnosing a pheochromocytoma is elevated plasma fractionated metanephrines. MEN IIa is caused by an autosomal dominant inherited germline RET mutation and is characterized by endocrine tumors. Patients are specifically at an elevated risk for developing medullary thyroid cancer, pheochromocytomas, and primary parathyroid hyperplasia. Medullary thyroid cancer is often the first presenting tumor, with peak incidence in the third decade of life. Pheochromocytomas are rarely the first tumor, but can be screened for if there is known familial inheritance. Treatment for pheochromocytomas is surgical excision. Brandi et. al. discuss the MEN syndromes in depth, and comment that most deaths from MEN II occur as a result of medullary thyroid cancer progression, not from pheochromocytomas. Early thyroidectomy in childhood, is thus recommended in suspected individuals with a familial history once a germline mutation is confirmed. Pappachan et. al. discuss the diagnosis and management of pheochromocytomas. They comment that the majority (85%) of pheochromocytomas occur in the adrenal medulla. Additionally, preoperative management with alpha blockade is critical for improved surgical outcomes. Incorrect Answers: Answer 1: Abdominal CT scan is important for surgical planning prior to resection of pheochromocytomas, in particular to assess for bilateral and extra adrenal disease. This is not the first line test for diagnosis, however. Answer 2: 24-hour free cortisol is a proper diagnostic test for patients suspected of Cushing's syndrome, not of pheochromocytomas. Answer 3: High dose dexamethasone suppression test in used to determine whether elevated ACTH is being produced centrally or peripherally in the setting of Cushing's syndrome. Answer 5: Plasma aldosterone/renin is the test of choice for the diagnosis of aldosterone secreting tumors.
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