Ann Thorac Surg 2003;75:1950-1951
© 2003 The Society of Thoracic Surgeons
Case report
Nonfunctioning paraganglioma of the aortopulmonary window
Cristiano F. Andrade, MDa,
Spencer M. Camargo, MDa,
Marcelo Zanchet, MDa,
José C. Felicetti, MDb,
Paulo F. G. Cardoso, MD, PhD*b
a Department ofDepartments of Pavilhao Pereira Filho Hospital, Porto Alegre, RS,, Brazil
b Department ofSurgery, Division of Thoracic Surgery, Pavilhao Pereira Filho-Santa Casade Porto Alegre Hospital, Faculdade Federal de Ciencias Medicas Porto Alegre-Rio Grande do Sul, Brazil
Accepted for publication November 14, 2002.
* Address reprint requests to Dr Cardoso, Pavilhao Pereira Filho-Santa Casa de Porto Alegre, Rua Professor Annes Dias 285, 1 Andar, Porto Alegre, RS 90020-090, Brazil
e-mail: cardoso{at}via-rs.net
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Abstract
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Aortopulmonary paraganglioma is a rare tumor of the mediastinum. The only effective treatment is complete resection, which may pose a surgical challenge because of its proximity to the heart, great vessels, and trachea, often rendering a complete resection difficult to achieve. We report a case in which the tumor was excised under cardiopulmonary bypass and resulted in massive bleeding only controlled by means of packing the pleural cavity during 48 hours, known as damage control strategy. The patient survived and has been disease-free for 2 years.
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Introduction
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Paragangliomas are slow-growing tumors originating from ganglionic cells of the autonomic nervous system [1]. Aortopulmonary paraganglioma is a rare tumor of the mediastinum, arising in close proximity with the major vessels and deriving from the fourth and fifth branchial arches [2]. In such a location, these tumors may pose a surgical challenge because of their proximity to the heart, great vessels, and trachea, thus rendering complete excision difficult to achieve [3].
A 50-year-old woman was admitted to the hospital because of dyspnea, dry cough, and chest pain for 3 weeks. Physical examination revealed normal blood pressure, heart, and breath sounds were decreased in the left hemithorax. Chest roentgenogram revealed an enlarged heart and a left-sided mediastinal mass (Fig 1A).
A chest computed tomographic scan revealed an 11-cm diameter mass that was densely vascularized and was located within the aortopulmonary window with signs of vascular invasion (Fig 1B). Echocardiogram showed a large pericardial effusion with cardiac tamponade. A pericardiocentesis improved her symptoms. Citopathology of the fluid was negative for malignancy. She was submitted to a left anterior mediastinotomy with biopsy of the mass, which caused massive bleeding at the site (2,000 mL) controlled with meticulous hemostasis. The histopathologic diagnosis was a paraganglioma. There were no signs and symptoms suggesting increased catecholamine secretion and urine vanillylmandelic acid levels were not elevated. Three days after the biopsy, the patient underwent resection through a median sternotomy. The mass was completely excised under cardiopulmonary bypass, including the left phrenic nerve and part of the pericardium. There were no cardiac implants. Removal of the surgical specimen (Fig 2)
resulted in massive bleeding in the site requiring transfusion (6,000 mL of blood) and an extension of the incision through an anterolateral left thoracotomy on the fifth interspace in order to control the hemorrhage. Despite reversal of anticoagulation and withdrawal of cardiopulmonary bypass, the bleeding continued. After 9 hours of surgery and several futile attempts to control the bleeding, the pleural cavity was packed with gauze pads and the sternothoracotomy was closed. The output through the chest tubes reduced subsequently within the next 48 hours, allowing removal of the packing through the left thoracotomy on the second postoperative day. The patient recovered slowly despite a wound infection on postoperative day 15, which was treated with drainage and antibiotics without sternal osteomyelitis or empyema. The patient was discharged on postoperative day 22 and remained asymptomatic without recurrence 2 years after surgery.
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Fig 1. (A) Roentgenogram showing an enlarged heart, a left mediastinal mass and ipsilateral pleural effusion. (B) Computed tomographic scan revealing an 11-cm mass within the aortopulmonary window with signs of vascular invasion.
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Comment
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Paragangliomas are found in all mediastinal compartments. When located in the posterior mediastinum, such tumors are somewhat easier to resect as opposed to those in the anterior or middle mediastinum, which may pose a surgical challenge because of the proximity to central airways, heart, and great vessels, making a complete excision often difficult to achieve [3, 4]. A functioning paraganglioma secretes catecholamines and its clinical diagnosis is suggested by refractory arterial hypertension. The diagnosis is confirmed by detection of increased urinary vanillylmandelic acid and other catecholamine metabolites [5]. Conversely, nonfunctioning paragangliomas of the mediastinum can be asymptomatic and detected during routine chest roentgenograms [2]. The symptoms are usually related to compression causing chest pain, dyspnea, dry cough, hoarseness, dysphagia, hemoptysis, and eventually superior vena cava syndrome [2]. Sometimes the identification of functioning paragangliomas is difficult and the scintigraphy with 131I-metaiodobenzylguanidine is used as a screening test in order to orient the computed tomographic scan. There is a slight female predominance and average age at diagnosis is 49 years [2]. There are no reliable morphologic criteria to microscopically separate benign from malignant paragangliomas. Despite recent studies in DNA ploidy, the presence of distant metastasis is thus far the criterion for malignancy found in 26% of patients [3, 5]. Paragangliomas are not sensitive to radiotherapy or chemotherapy, therefore the only chance for cure is a complete surgical resection allowing long-term survival (10 years) in 84%, as opposed to 50% when a partial resection is performed [3]. The operative mortality can be as high as 6%. Cardiopulmonary bypass was used in this patient because we anticipated that the major vessels and heart could be involved by the tumor. In such instances, adequate exposure is paramount and the addition of a left thoracotomy is useful in order to gain access to all structures of the anterior and middle mediastinum, mobilize the great vessels, and control bleeding. In this patient, the bleeding was controlled by packing the chest cavity, which is a salvage procedure often used in severe trauma patients. This treatment modality, known as damage control, is indicated whenever local hemostasis cannot be achieved because of coagulopathy, major venous injury, time-consuming procedures, and management of life-threatening injuries. The goal of damage control is to prevent hypothermia, acidosis and coagulopathy, known as the "lethal triad" [6]. In this case, such a maneuver was a valuable tool and played an important role in the successful early postoperative outcome. We did not consider the use of preoperative angiograms to map the vascular supply of the tumor because we judged that a primary resection was deemed feasible. In retrospect, based upon a review of the report by Rakovich and colleagues [7], we hypothesize that perhaps preoperative identification of the vascular supply of the tumor could allow tumor embolization, which ultimately could reduce intraoperative bleeding.
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References
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- Dunn G.D., Brown M.J., Sapsford R.N., et al. Functioning middle mediastinal paraganglioma (phaeochromocytoma) associated with intercarotid paragangliomas. Lancet 1986;1(8489):1061-1063.[Medline]
- Lack E.E., Stillinger R.A., Colvin D.B., et al. Aortico-pulmonary paraganglioma. Cancer 1979;43:269-278.[Medline]
- Lamy A.L., Fradet G.J., Luoma A., et al. Anterior and middle mediastinum paraganglioma: complete resection is the treatment of choice. Ann Thorac Surg 1994;57:249-252.[Abstract/Free Full Text]
- Ogawa J., Inoue H., Koide S., Kawada S., et al. Functioning paraganglioma in the posterior mediastinum. Ann Thorac Surg 1982;33:507-510.[Abstract/Free Full Text]
- Herrera M.F., Heerden J.A., Puga F.J., et al. Mediastinal paraganglioma: a surgical experience. Ann Thorac Surg 1993;56:1096-1100.[Abstract/Free Full Text]
- Shapiro M.B., Jenkins D.H., Schwab W., Rotondo M.F. Damage control: collective review. J Trauma 2000;49:969-978.[Medline]
- Rakowich G., Ferraro P., Therasse E., Duranceau A. Preoperative embolization in the management of a mediastinal paraganglioma. Ann Thorac Surg 2001;72:601-603.[Abstract/Free Full Text]
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Abstract
Introduction
