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Asian Cardiovasc Thorac Ann 2001;9:212-214
© 2001 Asia Publishing EXchange Pte Ltd

CASE STUDY

Cardiac Paraganglioma

Anil Z Apaydin, MD, Derina Wu, MD1, Randall B Griepp, MD

Department of Cardiothoracic Surgery
1 Department of Pathology Mount Sinai Medical Center New York, New York, USA
For reprint information contact: Randall B Griepp, MD Tel: 1 212 241 8181 Fax: 1 212 534 3357 Department of Cardiothoracic Surgery, Mount Sinai Medical Center, Annenberg 7-54, Box 1028, One Gustave Levy Place, New York NY 10029, USA.

   Abstract
TOP
 Abstract
INTRODUCTION
 CASE REPORT
 DISCUSSION
 REFERENCES
 
A rare case of cardiac paraganglioma in a 69-year-old man with coexisting coronary artery disease is reported. Diagnostic, operative, and pathological findings are presented. Although difficult, excision is the treatment of choice. Follow-up is warranted because of potential recurrence, especially in cases of incomplete resection.


   INTRODUCTION
TOP
 Abstract
 INTRODUCTION
CASE REPORT
 DISCUSSION
 REFERENCES
 
Paragangliomas are rare neoplasms of neural crest origin, arising in the chromaffin and chemoreceptor tissues. Chromaffin paragangliomas (pheochromocytomas) most commonly occur in the adrenal medulla; they are characterized by a positive chromaffin reaction, and they produce catecholamines.1 Nonchromaffin paraganglia normally do not secrete pressor substances but have chemoreceptor function.1 The most common sites for paragangliomas of the chemoreceptor tissue (chemo-dectomas) are the carotid body in the neck and the glomus jugulare in the region of the middle ear. A nonchromaffin paraganglioma arising from the heart is extremely rare. This is one of the few cases discovered in a living person.2


   CASE REPORT
TOP
 Abstract
 INTRODUCTION
 CASE REPORT
DISCUSSION
 REFERENCES
 
A 69-year-old asymptomatic white male with a history of borderline hypertension, had a positive exercise tolerance test. A coronary cineangiogram showed significant 3-vessel coronary artery disease and a well-vascularized mass feeding off the right coronary artery (Figure 1Go). Transesophageal echocardiography defined a 4 x 4-cm homogenous mass in the right ventricular (RV) free wall adjacent to the aorta (Figure 2Go), and a patent foramen ovale. Chest radiography, computed tomography, and magnetic resonance imaging of the chest were unremark-able. The patient underwent resection of the tumor, 4-vessel coronary bypass grafting, endarterectomy to the right coronary artery, and closure of the patent foramen ovale under cardiopulmonary bypass. The tumor arose from the RV anterior wall and was covered with epi-cardium and subepicardial fat tissue. It was surrounded by the ascending aorta posteriorly, the right atrial appendage laterally, and the right coronary and pulmonary arteries medially. Inferomedially, the tumor, which was very vascular, invaded the RV free wall by displacing the right coronary artery medially. Although it was difficult to develop a plane between the mass and the surrounding tissues, the tumor was resected down to the muscle layer. The RV cavity was not entered. More than 95% of the tumor was removed in one piece. It was a soft lobulated tan-colored mass that appeared to be encapsulated and measured 5 x 3 x 3 cm. Pathology of frozen sections established the diagnosis of paraganglioma. Severe bleeding from the cavity where the tumor had been located was controlled by packing it with gel foam and by suturing the epicardial flaps to each other and to the aortic adventitia. The postoperative course was uneventful and the patient was discharged on the 5th postoperative day. He remained asymptomatic 24 months after the operation.



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  		Figure 1. Coronary angiogram showing a well-vascularized tumor feeding off the right coronary artery.

 


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  		Figure 2. Transesophageal echocardiogram showing the location of the tumor. AO = aorta, PA = pulmonary artery, RA = right atrium.

 
After the frozen section study, the resected specimen was placed in 10% buffered formalin for paraffin section studies. The cut surface of the tumor in the fresh state was smooth, tan-gray, and fleshy. After formalin fixation, the mass darkened to a mahogany brown and the formalin also turned brown. Tissue sections were stained with hematoxylin and eosin. Microscopically, the tumor had a pseudocapsule of compressed fibrous connective tissue. There was a solid area where the tumor had a nesting or zellballen pattern. Other areas were edematous with tumor cells forming trabeculae and cords. The tumor was composed of a heterogeneous population of cells. The main cells were large with abundant clear to eosinophilic cytoplasm and indistinct cell borders. The nuclei were round to oval with a small but distinct nucleolus. Marked nuclear pleomorphism was observed in focal areas (Figure 3Go). Rare mitoses were seen. Immunohistochemical staining was performed by the avidin-biotin complex technique with antibodies against S100 and chromogranin. S100 highlighted the sustentacular cells and the chromo-granin demonstrated the neuroendocrine nature of the tumor cells, confirming the diagnosis of paraganglioma.



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  		Figure 3. Photomicrograph of the tumor, depicting nuclear pleomorphism (hematoxylin and eosin stain, original magnification x400).

 

   DISCUSSION
TOP
 Abstract
 INTRODUCTION
 CASE REPORT
 DISCUSSION
REFERENCES
 
Cardiac paragangliomas are extremely rare and limited information on their characteristics has been derived from tumors arising from sites other than the heart. Cardiac paragangliomas originate from paraganglion cells of the arteries or the visceral autonomic paraganglia of the atria.3 Reports of functioning cardiac paragangliomas (cardiac pheochromocytomas) have increased in the past 10 years, mainly due to the introduction of iodine-131 metaiodo-benzylguanidine in 1980.3 Most patients with a functioning cardiac paraganglioma are symptomatic (palpitations, hypertension). Nonfunctioning cardiac paragangliomas (chemodectomas) cause no symptoms that would indicate the use of iodine-131 metaiodobenzylguanidine, and are discovered only if cardiac symptoms develop. In the English-language literature, 8 nonfunctioning cardiac paragangliomas have been reported.2–6 Malignant behavior is found in 10% of all paragangliomas and there are no definite microscopic criteria to distinguish the benign from the malignant form. Gopalakrishnan and colleagues2 reported a case of nonfunctioning paraganglioma that was thought to be benign by histological examination and patient history, but a metastatic tumor developed within 2 years and the patient died of multiple metastases 4 years postoperatively. Interestingly, the autopsy report showed that the tumor had the same histologic features in the heart and metastases as in the original surgical specimen taken from the heart.7 Abad and colleagues6 reported a cardiac paraganglioma that was diagnosed by open biopsy but considered non-resectable. The case was followed up but there was no increase in tumor size echocardio-graphically after 16 months.

Nonchromaffin paragangliomas are generally seen in adults between the ages of 30 and 70 years, the average age being around the mid-40s, and they are more common in women. A definite familial incidence has been noted. It is generally accepted that excision when possible, should be the first line of treatment. Cardiac paragangliomas present a difficult surgical challenge. Unlike paragangliomas found in the abdomen, cardiac lesions typically do not form a well-defined plane between the tumor and adjacent tissue. The infiltrative intramural component that is usually encountered, prevents these highly vascular tumors from being easily excised.3 Patients should be followed up closely because of the possibility of recurrence, especially in cases of incomplete resection. There is no consensus regarding screening for recurrence. Scintigraphic imaging with a somatostatin analog such as indium-111 pentetreotide has been suggested.5 Echo-cardiography and gadolinium-enhanced magnetic re-sonance imaging may be helpful in detecting cardiac recurrence.4 We did not excise and replace the RV free wall to ensure complete resection because the risk of RV dysfunction outweighed the risk of recurrence in this particular case. More information about the behavior of these rare tumors is required to define the operative strategy.


   REFERENCES
TOP
 Abstract
 INTRODUCTION
 CASE REPORT
 DISCUSSION
 REFERENCES
 

  1. Abell MR, Hart WR, Olson JR. Tumors of the peripheral nervous system. Hum Pathol 1970;1:503–51.[Medline]

  2. Gopalakrishnan R, Ticzon AR, Cruz PA, Kennedy FB, Duffy FC, Barmada B, et al. Cardiac paraganglioma (chemodectoma): a case report and review of the literature. J Thorac Cardiovasc Surg 1978;76:183–9.[Abstract]

  3. Orringer MB, Sisson JC, Glazer G, Shapiro B, Francis I, Behrendt DM, et al. Surgical treatment of cardiac pheochromocytomas. J Thorac Cardiovasc Surg 1985; 89:753–7.[Abstract]

  4. Orr LA, Pettigrew RI, Churchwell AL, Jennings HS III, Petracek MR, Vansant JP. Gadolinium utilization in the MR evaluation of cardiac paraganglioma. Clin Imaging 1997;21:404–6.[Medline]

  5. Cha R, Rainear K, Paczolt E, Mahapatro D. Cardiac paraganglioma in New Jersey. N J Med 1997;94:35–7.

  6. Abad C, Jimenez P, Santana C, Coello I, Acosta A, Hernandez E, et al. Primary cardiac paraganglioma. Case report and review of surgically treated cases. J Cardiovasc Surg (Torino) 1992;33:768–72.[Medline]

  7. Cruz PA, Mahidhara S, Ticzon A, Tobon H. Malignant cardiac paraganglioma: follow-up of a case. J Thorac Cardiovasc Surg 1984;87:942–4.[Medline]





This Article
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Randall B Griepp
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Right arrow Articles by Apaydin, A. Z
Right arrow Articles by Griepp, R. B
Related Collections
Right arrow Cardiac - other

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