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Children presenting with pheochromocytoma should be tested for:

a) von Hipple-Lindau disease

b) Neurofibromatosis type 1

c) Multiple endocrine neoplasia, type 2

d) Succinate dehydrogenase mutations

e) All of the above

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Answer

Answer e. 40% of pheochromocytomas in children are associated with these genetic conditions. All young patients (under 50) should be offered genetic screening, especially as the succinate dehydrogenase mutations are associated with a high risk of malignancy.

Notes

This question originally printed in the Pediatric Anesthesiology Review Topics kindle book series, and appears courtesy of Naerthwyn Press, LLC.

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