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J Thorac Cardiovasc Surg 2006;132:1460-1461
© 2006 The American Association for Thoracic Surgery

Brief Communication

Extra-adrenal pheochromocytoma involving the left atrium: An infrequent pathologic occurrence

M. Ibrahim Nassar, MDa,*, Ramiro de la Llana, MDa, Eduardo de Bonis, MDc, Pilar Garrido, MDa, Antonio Alarcó, MDb, Rafael Méndez, MDd, Rafael Martínez-Sanz, MDa

a Department of Cardiovascular Surgery, University Hospital of the Canary Islands, University of La Laguna, Tenerife, Spain
b Department of General Surgery, University Hospital of the Canary Islands, University of La Laguna, Tenerife, Spain
c Department of Nephrology, University Hospital of the Canary Islands, University of La Laguna, Tenerife, Spain
d Department of Pathology, University Hospital of the Canary Islands, University of La Laguna, Tenerife, Spain.

Received for publication June 16, 2006; accepted for publication August 16, 2006.

* Address for reprints: M. Ibrahim Nassar, MD, Pedro Modesto Campos no. 4 5°-C, 38005 Sta Cruz de Tenerife, Spain. (Email: kardionassar{at}gmail.com).

Cardiac paraganglioma is extremely infrequent. About 20 cases have been reported, some of them more than once. Our patient presented with the clinical signs, symptoms, and biochemical evidence suggestive of pheochromocytoma. Computed tomography showed a tumor situated above the left atrium that was successfully removed by means of cardiac surgery.

Clinical Summary

A 37-year-old woman experienced hypertensive crisis while being anaesthetized for cholecystectomy. An anterior mediastinal mass, compatible with pheochromocytoma, was revealed by means of computed tomographic scanning and vanillylmandelic acid determinations.

Mediastinal sternotomy was performed to reach a theoretic anterior mediastinal mass. However, during the operation the mass was found to be embedded in the left atrium, extending into the wall of the right upper lobar segmental arteries. The only way to remove the tumor was by means of rear total left atrial exeresis followed by substitution with a large polytetrafluoroethylene* patch (10 x 12 cm) and resection of the pulmonary affected arteries (Figure 1). The technique was performed with cardiopulmonary bypass (CPB).


Figure 1
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  		Figure 1. Resection of total rear atrium and substitution with a polytetrafluoroethylene patch.

 
The postoperative course was uneventful, and the patient was discharged 11 days later after echocardiographic confirmation of normalized left atrial function. At 6 months, echocardiography showed complete patch endothelialization, and oral anticoagulation was suspended.

Histologic findings included tumor node formation with complete delimitation by a connective capsule. Microscopic findings showed organoid clustering of neoplastic cells forming a "zellballen." A sustentacular cell population can be demonstrated by staining for S-100 protein at the periphery of the nets. Tumor cells were oval or polygonal with granular cytoplasm. This cytoplasm presented strongly positive immunoreactivity for chromogranin A. We observed tumor cells connecting with the external muscular wall of the left atrium (Figure 2).


Figure 2
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  		Figure 2. Histologic findings typical of paraganglioma.

 
Discussion

Since the beginning of this century, there have been descriptions of paraganglia in various sites near the base of the heart and great vessels.1Go This subset of paraganglioma might require special surgical management techniques, such as CPB with cardioplegia2Go and even human cardiac explantation and autotransplantation when the paraganglioma is large.3Go In our case the aortopulmonary paraganglioma arises from the paraganglia, which might be intracardial, usually at the level of the atria, or extracardial. These tumors account for about 1% of all primary mediastinal neoplasias.4Go

Occasionally cardiac paragangliomas are associated with paragangliomas in other sites. The incidence of malignancy with metastasis has been estimated at 13%.5Go

Surgical resection might necessitate CPB with removal of portions of the heart affected by the tumor, as in our case, and intraoperative deaths have occurred because of excessive bleeding.

This case was exceptional for the complexity of the surgical technique, the semispherical patch modeling, and the normalization of left atrial function without loss of sinusal rhythm.

Footnotes

* Gore-Tex patch, registered trademark of W. L. Gore & Associates, Inc, Newark, Del. Back

References

  1. Becker AE. The glomera in the region of the heart and great vessels. A microscopic-anatomical and histochemical study [MD thesis]. Amsterdam: University of Amsterdam; 1966.
  2. Orringer MB, Sisson JC, Glazer G, et al. Surgical treatment of cardiac pheochromocytomas. J Thorac Cardiovasc Surg 1984;89:753-757.
  3. Cooly DA, Reardon MJ, Frazier OH, Angelini P. Human cardiac explantation and auto-transplantation: application in a patient with large cardiac pheochromocytoma. Tex Heart Inst J 1985;12:171-176.[Medline]
  4. Benjamin SP, McCormack LJ, Effler DB, Groves LK. Primary tumors of the mediastinum. Chest. 1972;62297-303.
  5. Lack EE, Stillinger RA, Colvin DB, Groves RM, Burnette DG. Aortico-pulmonary paraganglioma: report of a case with ultrastructural study and review of the literature. Cancer 1979;43:269-278.[Medline]




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