Ann Thorac Surg 2004;78:e43-e44
© 2004 The Society of Thoracic Surgeons
Case report
Primary cardiac paraganglioma
Ryszard W. Lupinski, MD, PhDa,*,
Sriram Shankar, FRCS, FAMSa,
Thirugnanam Agasthian, MMed, FRCSa,
Chong Hee Lim, FRCS, FAMSa,
Kent Mancer, MD, FRCPCb
a Department of Cardiothoracic Surgery, National Heart Centre, Singapore
b Department of Pathology, Singapore General Hospital, Singapore
Accepted for publication January 14, 2004.
* Address reprint requests to Dr Lupinski, Department of Cardiothoracic Surgery, National Heart Centre, Mistri Wing, 17 Hospital Ave, Singapore 168752
rilup2{at}singnet.com.sg
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Abstract
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We report a case of a 29-year-old male, who during workup of hypertension was found to have a malignant primary paraganglioma of the heart. The tumor arose from the site of the aortopulmonary window and right ventricular outflow tract (RVOT) and was removed with the aid of cardiopulmonary bypass. Reconstruction of the RVOT and pulmonary valve was necessary because of involvement by the tumor. The surgical course was uncomplicated, with normalization of catecholamine secretion and blood pressure.
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Introduction
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Primary cardiac tumors are rare. Primary cardiac paragangliomas account for fewer than 50 cases in the medical literature. They are typically located adjacent to or involving the left atrium. Other rare reported locations are right atrium, inferior vena cava, and right ventricular outflow tract (RVOT). This is a case of primary cardiac paraganglioma, which arose from the aortopulmonary window and RVOT. This localization of cardiac paraganglioma is extremely rare, having been reported in only two previous cases.
This is the case of a 29-year-old male, who was referred by a general practitioner for treatment of uncontrolled hypertension and was admitted for workup. At age 23 he had been admitted to the hospital with pallor, cough, chest pain, breathlessness for 2 weeks, a fever of 38.4°C, and raised jugular venous pressure. In hospital his blood pressure measurements were between 97/56 and 167/85 mm Hg. At that stage, he was not on any treatment for his elevated blood pressure. Chest roentgenogram indicated cardiomegaly and globular heart. Two-dimensional echocardiography revealed pericardial effusion. At surgery, 200 mL of hemoserous fluid was drained, adhesions released, and a piece of thickened pericardium was sent for histopathology evaluation. It revealed chronic granulomatous inflammation and antituberculosis treatment was given for 9 months. At that admission he also had esophageo-gastroendoscopy as the occult blood was positive. It revealed gastric erosions and duodenal ulcer.
Between admissions to the hospital, his blood pressure was as high as 196/120 mm Hg. Nifedipine 60 mg, atenolol 50 mg, and phenoxybenzamine 10 mg bd was given to treat the hypertension. On his second admission a workup for hypertension included renal ultrasound and Doppler study, which revealed normal kidneys and vessels. The urine showed raised levels of norepinephrine 16,994 nmol/d (N 89 to 473), dopamine 11,007 nmol/d (N 424 to 2,612), normetanephrine 31,766 nmol/d (N 600 to 1900), and vanillylmandelic acid 99.1 nmol/d (N 0 to 34.3). Abdominal computed tomographic scan excluded an adrenal pheochromocytoma. Subsequently, he underwent iodine-131-meta-iodobenzylguanidine scan and the findings were highly suggestive of the presence of an ectopic pheochromocytoma in the anterior-middle mediastinum as well as an abnormal focus in the left lung (Fig 1). For further evaluation of the tumor the patient had chest magnetic resonance imaging. It revealed a large lobulated mass in the anterior mediastinum (Fig 2). No obvious lesions were found within the left lung. Computed tomographic thorax also showed no evidence of any focal lesion within the left lung parenchyma (Fig 3). Surgical removal of the anterior mediastinal mass was performed. The chest was opened by median sternotomy. A hypervascularized tumor invaded the thymus and pericardium and grew into the RVOT (Fig 4). As the heart was invaded by the tumor, it was decided to put the patient on cardiopulmonary bypass. The tumor was removed en bloc with thymus, pericardium, part of pulmonary trunk (including anterior leaflet of pulmonary valve), and RVOT myocardium. The anterior leaflet of the pulmonary valve was reconstructed with autologous pericardium and RVOT was repaired using equine pericardium. The tumor, which measured 6 x 5 x 3 cm, was sent for histopathology evaluation and the result confirmed the diagnosis of mediastinal paraganglioma. The thymus was adjacent to, but not directly invaded by, the tumor. After surgery, the blood pressure normalized and the patient was discharged home on the fifth postoperative day. Two-dimensional echocardiography revealed no significant pulmonary regurgitation.
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Fig 1. Meta-iodobenzylguanidine scan. Arrows indicate increased uptake of the tracer in the mediastinum and in the left lung.
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Comment
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Primary cardiac neoplasms are rare, occurring in only 0.001% to 0.03% of several reported autopsy series. About 75% of primary cardiac tumors are benign. The malignant tumors include various sarcomas, mesothelioma, malignant fibrous histiocytoma, and malignant paraganglioma.
Paraganglioma is a tumor that arises from paraganglia, the clusters of neuroendocrine cells widely distributed throughout the body, including the adrenal medulla, carotid, vagal, and paraaortic bodies and groups of cells associated with sympathetic ganglia. Benign and malignant paragangliomas are histologically indistinguishable [1]. Primary adrenal paraganglioma is called pheochromocytoma, while extraadrenal are called paraganglioma. Cardiac paraganglioma is very rare. In the recent review of the surgical literature only 37 cases have been reported [2]. Patients with cardiac paragangliomas may present with hypertension, or with various obstructive or compressive symptoms, depending on the location of the tumor [3]. Cardiac paragangliomas are typically located adjacent to or involving the left atrium [4]. Other rare locations include the right atrium, inferior vena cava, and RVOT. Removal of the tumor requires reconstruction of resected sites [5]. Heart transplantation is the modality of choice when total removal of the tumor is not feasible. Successful management depends on total resectability of the tumor. Incomplete resection leads to recurrence and, in the case of malignant paraganglioma, to metastases and death of the patient [6]. As our patient presented with gastric and duodenal ulcer, he is under close follow-up for possible gastric gastrointestinal stromal tumor, which together with malignant pheochromocytoma and pulmonary chondroma is a part of Carney's triad [7]. It remains unclear whether the pericardial effusion and adhesions found during the first thoracotomy and limited pericardiectomy 6 years before curative surgery was a manifestation of the tumor that was not yet detected.
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References
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- Lack EE. Aorticopulmonary paraganglioma. In: Atlas of tumor pathology. Tumor of the adrenal and extra-adrenal paraganglia system. Third series. Washington, DC: Armed Forces Institute of Pathology; 1997:375-386
- Lin JC, Palafox BA, Jackson HA, Cohen AJ, Gazzaniga AB. Cardiac pheochromocytoma: resection after diagnosis by 111-indium octreotide scan. Ann Thorac Surg. 1999;67:555–558[Abstract/Free Full Text]
- Tekin UN, Khan IA, Singh N, Nair VM, Vasavada BG, Sacchi TJ. A left atrial paraganglioma presenting with compressive dysphagia. Can J Cardiol. 2000;16:383–385[Medline]
- Hamilton BH, Francis IR, Gross BH, et al. Intrapericardial paragangliomas (pheochromocytomas): imaging features. Am J Roentgenol. 1997;168:109–113[Abstract/Free Full Text]
- Rotker J, Oberpenning F, Scheld HH, Hertle L, Knichwitz G, Hamm D. Pheochromocytomas with extension into central vascular structures. Ann Thorac Surg. 1996;61:222–224[Abstract/Free Full Text]
- Jeevandam V, Oz MC, Shapiro B, Barr ML, Marboe C, Rose EA. Surgical management of cardiac pheochromocytoma. Resection versus transplantation. Ann Surg. 1995;221:415–419[Medline]
- Carney JA. The triad of gastric epithelioid leiomyosarcoma, pulmonary chondroma and functioning extra-adrenal paraganglioma. A five-year review. Medicine (Baltimore). 1983;62:159–169[Medline]
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Abstract
Introduction
