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We report a 15-year-old girl with a recent diagnosis of type 2 diabetes mellitus who presented in malignant hypertensive crisis (BP 210/120 mm Hg). Abdominal CT showed an 8.2 x 4.7 x 7.0 cm mass in the region of the organ of Zuckerkandl. MIBG scan showed a single paraganglioma without metastatic foci. Plasma total metanephrines were 232,176.4 pmol/l [263-1052] with normetanephrine predominance. Pre-operative course was complicated by ischemic stroke in the left MCA and right thalamic regions, acute renal failure, rhabdomyolysis and congestive heart failure. She required massive doses of propranolol, phenoxybenzamine, doxazosin and metyrosine prior to surgery.

Ischemic Stroke and Rhabdomyolysis in a 15 Year-old Girl with Paraganglioma due to an SDHB Exon 6 (Q214X) Mutation