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Coronary Steal Syndrome From A Cardiac Paraganglioma Tumor
Michael Gibson.
St Luke's Hospital of Kansas City, Kansas City, MO, USA.
Background: Paragangliomas are rare extra adrenal neuroendocrine tumors that account for <1% of cardiac tumors. When metabolically inactive they can be asymptomatic but are more commonly associated with symptoms of of catecholamine excess. Rarely, extensive vascularity can create parasitic arterial steal syndrome from the organ they derive their blood from.
Objective: We present a 74-year-old male who presented with symptoms and initial non-invasive cardiac imaging consistent with coronary ischemia.
Methods: A 5.6 x 4.5 cm metabolically active, mediastinal mass superior to the left atrium with extensive coronary collaterals causing coronary steal syndrome was diagnosed.
Results: 74-year-old mail present with symptoms consistent with angina. Non-invasive MPI imaging demonstrated anterior wall ischemia. Elective cardiac catheterization was performed (Fig 1A and B) demonstrating nonobstructive coronary disease with a large collateral off the circumflex and a second large collateral off the right coronary artery both feeding a large vascular mass. PET CTA demonstrated a hypermetabolic 5.6 x 5.5 mass (Fig 1C). After preoperative treatment with alpha and beta blockers, the tumor was successfully resected (Fig 1D) with hemostasis facilitated with the novel use of a Ligasure (Medtronic, Minneapolis, MN) device. Postoperative histochemistry(Fig 1E) and immunohistochemistry with S100 (Fig 1F) confirmed the diagnosis of a paraganlioma. Postoperative course was uneventful and subsequent cardiac nuclear imaging demonstrated complete resolution of the preoperative anterior wall ischemia.
Conclusions: Cardiac paragangliomas are rare catecholamine secreting neuroendocrine tumors. Extensive vascularity may create coronary steal that masquerades as cardiac ischemia. Management requires careful preoperative planning, and this case, the use of the Ligasure device facilitated the complete resection of the tumor.
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