Interactive Cardiovascular and Thoracic Surgery 3:578-580(2004)
© 2004 European Association of Cardio-Thoracic Surgery
Pulmonary metastases in a bilateral carotid body paraganglioma
Carlos García Francoa,*,
Sarah Heilib,
Luis Jiménez Hiscocka and
José Zapatero Gaviriaa
a Department of Thoracic Surgery, Hospital Fundación Jiménez Díaz, Avda. Reyes Católicos no. 2, 28040 Madrid, Spain
b Department of Pulmonology, Hospital Fundación Jiménez Díaz, Avda. Reyes Católicos no. 2, 28040 Madrid, Spain
* Corresponding author. Tel.: +34-696-464905; fax: +34-915-725617. (E-mail: cgarciafranco{at}terra.es).
Received February 24, 2004;
received in revised form May 15, 2004;
accepted June 7, 2004
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Abstract
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We report the case of a bilateral carotid body paraganglioma which gave metastases to the lung parenchyma; the rarity of diffuse visceral spread is emphasised in this article. The patient was a 69-year-old woman operated for a right carotid body paraganglioma in 1997. In August 2002, a recurrent left carotid body paraganglioma was resected. Three months later she started presenting minimum hemoptysis and pulmonary tumours were detected during a routine chest roentgenographic examination. A right upper lobectomy and a wedge resection in the lower lobe were performed to remove them. Grossly, the greatest tumour measured 3.3x2.5x2.3cm and was well circumscribed and subpleural. It was histologically confirmed that these pulmonary tumours were metastases from the primary paraganglioma.
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1. Introduction
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Paraganglia are structures which belong to the autonomous nervous system and are located in multiple organs. They are characterized by the presence of morphologically and cytochemically similar neuroendocrine cells derived from the neural crest (we exclude from this group the suprarenal gland) [1]. Tumors originated in paraganglia are called paraganglioma, these are normally highly vascularized. Paraganglioma of the carotid body is one of the most common paraganglionic neoplasms distributed in the head and neck area [2]; the overall incidence of bilaterality in the sporadic form of the disease is estimated to be 5%, but this rises to 33% in cases with previous family history. The number of cases of histologically proved distant metastases, however, is extremely low and very few cases with pulmonary metastases have been described.
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2. Case report
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We present the case of a 69-year-old woman operated for a right carotid body paraganglioma in 1997. In August 2002, she was reoperated for local appearance of a new left carotid body paraganglioma. In November 2002, during her check-up, she commented on the appearance of red blood clots when cleaning the tracheotomy cannula, a weight loss of about 5kg over the previous five months and pain in the right side of her chest. The physical exploration was normal although the global situation of the patient was bad, with progressive weakness and difficulties in her daily life. Blood differential count showed 11,330leukocytes/mm3 with a normal number of white and red blood cells and normal coagulation times. The tumour markers SCC and CEA were not high. An arterial blood gas analysis revealed the following parameters: pH of 7; PaCO2 of 38 mmHg and PaO2 of 73 mmHg. Spirometry revealed a forced vital capacity of 2040ml (95% of predicted value), FEV1 of 1610ml (91%), FEV1/VC IN of 102%. A chest roentgenogram revealed a well circumscribed, solitary 4-cm right upper lobe mass. An axial computed tomography (TC) revealed two vascular density masses in the anterior and posterior segments of the right upper lobe (Fig. 1), and a nodule with the same density in the lower right lobe. There was also a small mediastinal lymphatic node of about 1cm in diameter in the inferior right pretracheal region. Bronchoscopy showed a red, polypoid mass in the right main stem bronchus. Minimum friction by the bronchoscope on the surface of the mass was enough to make it bleed. The needle bronchoscopic aspiration of the pretracheal lymph node showed normal lymphatical cytology. An arteriography showed a conglomerate of vessels in the apical segment of the right upper lobe and two nodules, with the same vascular characteristics, in the regions described previously (Fig. 2). Embolization of these vessels was attempted to control a probable massive haemopthysis and, as a security measure, to control a possible bleeding event before the surgical procedure to determine the origin of these tumours. Finally, a right upper lobectomy was performed, a 3.3x2.5x2.3cm circumscribed mass was identified in the apical segment of the right upper lobe, arising within the main right upper bronchi; other nodules were detected in the anterior and posterior segments of the right upper lobe. The border of resection of the main right upper bronchi was studied in a frozen section and it was concluded that this had been affected by the tumor. A wedge bronchotomy was performed to eradicate the tumoral tissue left in the main bronchi. A gray-white subpleural nodule was identified in the inferior right lobe and a wedge resection was applied. The nodule measured 1.5x1cm and focally abutted on the pleural surface without gross puckering. The intraoperatory pathologic evaluation of this nodule and of the mass in the apical segment established that it could be compatible with a paraganglioma. The other nodules were compatible with parenchymal blood clots. Five hiliar lymphatical nodes were excised, and no malignant cells were found. The patient was discharged 7 days after surgical resection. Abdominal recurrence appeared at the one-year check-up and chemotherapy was administrated, obtaining a poor response. The current situation of the patient is bad with nodules spreading through lungs and liver.
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Fig. 1 TC revealed two vascular density masses in the anterior and posterior segments of the right upper lobe.
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Fig. 2 An arteriography showed a conglomerate of vessels in the apical segment of the right upper lobe.
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3. Discussion
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The majority of authors agree that the only proof of malignancy in paraganglioma is the presence of metastases. In our case, metastases were confirmed by conventional histological examination, where the characteristic Zellballen pattern was found. It is important to stress that the histological appearance of a carotid body paraganglioma is not a reliable guide to its propensity for malignant behaviour, which demonstrates a poor correlation between histology and tumour behaviour [3–5].
Carotid body paraganglioma will grow relentlessly if not resected, causing neurovascular problems; in some series a mortality rate of about 8% has been noted in untreated cases. In other cases, even if resected, it can produce metastases. Treatment for head and neck paragangliomas is surgical resection, radiotherapy or both. The use of chemotherapy in metastasic paraganglioma has not been yet clearly established and although some results are optimistic, specially concerning the treatment of symptoms, an increase in survival rates has not been clearly proved [6–8]. There is practically no documentation on how to treat pulmonary metastases as they are really most rare [9,10] (In our opinion, if isolated lung metastases appear they should be resected if feasible).
doi:10.1016/j.icvts.2004.06.010
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References
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Abstract
1. Introduction