A rare case of cardiac paraganglioma presenting as anginal pain: a case report

Abstract

Introduction: Primary cardiac paraganglioma is a very rare tumor with less than sixty reported cases in the literature. The clinical presentation is variable, but is most commonly manifested by hypertension and symptoms related to the catecholamine excess.

Case report: We report a case of a 35 year old man who presented with anginal pain and hypertension. He was found to have a cardiac mass on the computed tomographic scan and echocardiogram. He underwent surgical exploration of the mass which on biopsy was found to be a 'Cardiac Paraganglioma'. Surgical resection of the tumor was successfully done and the patient is doing well five years after the surgery without any evidence of recurrence. His blood pressure, however, failed to normalize and needed single agent antihypertensive therapy.

Conclusion: Cardiac paragangliomas have a relatively favorable outcome if diagnosed and resected in time. We briefly review the literature regarding the diagnosis, treatment and prognosis of this rare tumor.

Figure 1

Cardiac catheterization image: showing the "tumor blush" (denoted by "a") with neovascularization seen projecting from the left circumflex artery (arrow) to the "tumor blush."

Figure 2

CT scan: image showing a solid and likely necrotic mass (arrows) extending posterior to the left atrium.

Figure 3

Trans-esophageal echocardiogram: TEE shows a large mass, likely extrinsic to the heart, compressing the left atrium (LA). RA denotes Right Atrium; RV denotes Right Ventricle; LV denotes Left Ventricle; P denotes the Cardiac Paraganglioma.

Figure 4

Biopsy from the cardiac mass: photomicrograph of the biopsy specimen taken from the tumor showing tumor cells arranged in a nest-like (Zellballen) pattern, in a background of thick collagenous stroma, separated by thin vascular network. (Original magnification ×40; H&E stain).