Paraganglioma - Cardiac Involvement

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Can J Cardiol. 2000 Mar;16(3):383-5.
A left atrial paraganglioma patient presenting with compressive dysphagia.
Tekin UN, Khan IA, Singh N, Nair VM, Vasavada BC, Sacchi TJ.
Division of Cardiology, Long Island College Hospital, Brooklyn, NY, USA.

Paragangliomas are rare neoplasms of neural crest origin arising in the chromaffin (pheochromocytoma) and chemoreceptor (chemodectoma) tissues. Only a few cases of paragangliomas have been reported in the heart. Most of the cardiac paragangliomas are loca ted in, or adjacent to, the left atrium. The biological activity indicative of catecholamine production has rarely been shown in cardiac paragangliomas. Patients with cardiac paragangliomas may present with hypertension, or with various obstructive or com pressive symptoms, depending on the location of the tumour. A left atrial paraganglioma compressing the esophagus causing dysphagia is presented. A 37-year-old female patient underwent work-up for dysphagia to solids. Transesophageal echocardiography show ed the presence of a large mass in the dilated left atrium. The tumour was removed completely intact and was a benign noncatecholamine-secreting paraganglioma. The patient's dysphagia was relieved. The clinical perspectives of cardiac paraganglioma and ca rdiac dysphagia are discussed.

Saudi Med J. 2002 Oct;23(10):1278-80.
Intrapericardial paraganglioma.
Boumzebra DA, Charifchefchaouni ZS, Belhadj SA, Maazouzi WA, Al-Halees ZY.
Cardiovascular Surgery Unit, Ibn Sina Hospital, Rabat, Morocco.

We report a case of intrapericardial paraganglioma accidentally discovered during surgery for mixed mitral valve disease. The 6 cm tumor was located in the aorta-caval groove, adherent to the roof of the left atrium and compressing the superior vena cava. A biopsy of the mass established the histological diagnosis of a non pressor secreting paraganglioma. The tumor was resected 2 months after the original mitral valve surgery. The patient made an uneventful recovery and remains well 2 years after surgery.

Eur J Cardiothorac Surg. 1996;10(4):287-9.
Intrapericardial paraganglioma.
Casanova J, Moura CS, Torres JP, Vouga L, Graca AS, Gomes MM.
Department of Thoracic Surgery, S. Joao Hospital, Oporto, Portugal.

We describe the case of an intrapericardial pheochromocytoma located in the anterior surface of the heart and spreading over the pulmonary trunk. Under cardiopulmonary bypass (CPB) the tumor was removed. "En bloc" resection of the anterior wall of the rig ht ventricular infundibulum and the pulmonary trunk was performed, with implantation of a fresh aortic homograft in the pulmonary position to avoid free pulmonary regurgitation.

Rev Med Liege. 2001 Nov;56(11):748-52.
Acute cardiac failure revealing a paraganglioma
Martin y Porras M, Hamoir E, Defechereux T, Delhougne V, Kulbertus H.
Service de Chirurgie, Universite de Liege.

Therapy of cardiomyopathy is usually symptomatic. So, the etiology is often only superficially investigated. However, because of their curability, rare causes must be sought for. We report a case of acute cardiac failure in a young female patient with sev ere hypertension of recent onset. Urinary catecholamines analysis and medical imaging demonstrated a paraaortic paraganglioma. After coelioscopic resection, cardiac function recovered.

Arch Pathol Lab Med. 2001 May;125(5):680-2.
Pigmented cardiac paraganglioma.
Mikolaenko I, Galliani CA, Davis GG.
Department of Pathology, University of Alabama at Birmingham, Birmingham, AL, USA.

A pigmented left atrial paraganglioma was found at autopsy in a 40-year-old black man who died unexpectedly. The cause of death was ascribed to coronary artery disease. The atrial mass was sharply demarcated and polypoid, measured 4 cm in greatest dimensi on, and had a cut surface that revealed dark red-brown soft tumor tissue. Histopathologically, the neoplasm exhibited a classic organoid clustering of cells (zellballen) with a prominent capillary network. The chief cells contained a brown-black pigment w ith histochemical characteristics of melanin. We report a case of pigmented cardiac paraganglioma because of its rarity. To our knowledge, no mention has been made of the presence of pigment in previously reported cases of cardiac paragangliomas.

Ann Thorac Surg. 1996 Jun;61(6):1845-7.
Paraganglioma of the interatrial septum.
Cane ME, Berrizbeitia LD, Yang SS, Mahapatro D, McGrath LB.
Department of Thoracic and Cardiovascular Surgery, Deborah Heart and Lung Center, Browns Mills, NJ 08015, USA.

The case of a patient undergoing successful resection of an interatrial septal paraganglioma is presented. The diagnosis of an interatrial mass was established preoperatively by echocardiography, ultrafast cine computed tomographic scan, and cardiac cathe terization. The tumor was excised in total, and the interatrial septum and the roof of the left atrium were reconstructed using a bovine pericardial patch.

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