Catecholamine Secreting Tumor Presenting With Stroke

Program: Abstracts - Orals, Featured Poster Presentations, and Posters
Session: MON 37-82-Pheochromocytoma & Paraganglioma
Monday, June 17, 2013: 1:45 PM-3:45 PM
Expo Halls ABC (Moscone Center)

Poster Board MON-81
Mansi Mehta*1, Alice Kit-wah Lee2 and Kenneth Howell Hupart3
1Nassau University Med Ctr, East Meadow, NY, 2Nassau University Medical Center, East Meadow, NY, 3Nassau Univ Med Ctr, East Meadow, NY
Introduction: Cerebrovascular accident(CVA) is a serious but rare complication in patients with catecholamine secreting tumors(CST); it has been reported in 2 to 5% of patients. We present a patient in whom we identified a norepinephrine(NE) secreting sporadic paraganglioma. Improved understanding of this potential consequence of CST may have lead to definitive therapy when our patient presented in her 40s with the first of her three CVAs.

Case: A 52-year-old woman presented with right sided weakness and uncontrolled hypertension. She also complained of headache, palpitations and sweating. History was significant for 2 prior CVAs (2008, 2010), hypertension treated with 3 medications, well controlled Type 2 DM, diastolic heart failure and seizures. Laboratory data revealed plasma free normetanephrine(NM) =1773 pg/ml (=<148) and total metanephrines(TM)=1814 pg/ml (=<205), with an elevated chromogranin A. 24-hour urine revealed NM=5149 μg (122-676), TM=5296 μg (224-832), and NE=794 μg (15-100). Adrenal MRI was normal. OctreoScan displayed focal uptake in the midline upper pelvis suggesting paraganglioma. Pelvic MRI identified a 4 cm retroperitoneal mass inferior to the aortic bifurcation. Succinate Dehydrogenase B and D mutations are negative. She has been referred for surgery.

Discussion: The diagnosis of CSTs should be suspected not only in patients with classic symptoms of persistent or paroxysmal catecholamine excess, but also in patients who present with evidence suggestive of secondary hypertension or unexpected consequences of hypertension.  Autopsy series point to the underdiagnosis of CSTs and the hazards of missing this cause of potentially reversible hypertension.

Our patient presented with CVA at a relatively young age. An investigation for causes of secondary hypertension at the time of her first CVA would have likely identified her CST. With the presentation of her third CVA, her laboratory investigation unambiguously identified the presence of catecholamine excess, the presence of high NE predicted the extra-adrenal tumor location. There are few case reports of patients with CVA from pheochromocytoma and fewer in patients with paraganglioma; most of the data is retrospective. Wider recognition of the profound morbidity that can arise from these conditions may sensitize clinicians to the importance of evaluating patients for secondary causes of hypertension; identification of familial CST would have benefits that extend to a patient’s relatives.

Nothing to Disclose: MM, AKWL, KHH

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