SESSION TITLE: Cardiovascular Student/Resident Case Report Posters I
SESSION TYPE: Medical Student/Resident Case Report
PRESENTED ON: Tuesday, October 29, 2013 at 01:30 PM - 02:30 PM
INTRODUCTION: Paragangliomas are rare tumors of neural crest cells of the autonomic ganglia of the head and neck, the heart, and the viscera. We report a case of a patient who presented with a syncopal event, who was ultimately found to have an invasive neoplastic left atrial cardiac paraganglioma.
CASE PRESENTATION: A 45-year-old white male presented with syncope. He had no precipitating symptoms. He had a glomus jugulare tumor removed from the right side of the neck in 1993. Postoperatively, he suffered a stroke with left upper extremity paralysis and left lower extremity partial paralysis. His physical examination only demonstrated a facial laceration and the post-surgical changes from his tumor resection. Head and maxillofacial CT suggested neoplastic involvement of the right mandible/temporal bone. CT angiography of the head and neck demonstrated a large area of abnormal enhancement along the distal right internal jugular vein region. MRI of the brain showed a large abnormal enhancing mass within the right petrous bone. MRI of the neck showed a enhancing mass surrounding the distal cervical carotid artery, which was suspected to be recurrence of the original tumor. Trans-thoracic and trans-esophageal echocardiogram demonstrated a well-circumscribed 4.6 x 5.4 cm sessile mass in the right atrium near the inter-atrial septum. He was taken to surgery for removal of the mass, which involved the lateral wall of the left atrium and the inter-atrial septum. His post-operative recovery was uncomplicated other than atrial fibrillation. Resection of his glomus tumor was planned for the future. Histological examination demonstrated sheets of neoplastic, polygonal eosinophilic cells with cords and nests and a delicate fibrous network of scattered irregular vessels. The cells exhibited oval nuclei, fine chromatin and small nucleoli. Chromogranin A, synaptophysin, and CD056 were positive, and consistent with a neuroendocrine cardiac paraganglioma.
DISCUSSION: Paragangliomas are rare tumors originating from neural crest cells of the autonomic nervous system which have the ability to secrete neurohormones and produce a mass effect. Head and neck paragangliomas are rare, and cardiac paragangliomas are extremely rare. Review of the literature demonstrates paraganglioma recurrence are rarer still. We report a case of left atrial paraganglioma that was diagnosed during evaluation of a syncopal event, in a patient with a history of recurrent glomus jugulare tumors.
CONCLUSIONS: This typical presentation of syncope occurred from an extremely rare and abnormal cause of recurrent paragangliomas.
Reference #1: Cleveland D.C., Westaby S., Karp R.B. Treatments of intraaterial cardiac tumors. JAMA 1983;249:2799-802.
Reference #2: Cane M.E., Berrizbeitia L.D., Yang S.S., Mahapatro D., McGrath L.B. Paraganglioma of the Intraatrial Septum. Ann Thorac Surg 1996;61:1845-7.
DISCLOSURE: The following authors have nothing to disclose: Gary Brooks, David Polizzi, Gerald Park, Victor Test
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