Eur J Cardiothorac Surg 2002;22:482-484
© 2002 Elsevier Science NL
Unusual presentation of mucoepidermoid carcinoma with recurrent pulmonary embolism
Mohan Devbhandari*,
Sasha Stamenkovic,
William Walker,
Evan Cameron
Department of Cardiothoracic Surgery, The Royal Infirmary of Edinburgh, Edinburgh, UK
Received 4 April 2002;
accepted 15 May 2002.
* Corresponding author. Department of Cardiothoracic Surgery, Blackpool Victoria Hospital, Whinney Heys Road, Blackpool FY3 8NR, UK. Tel.: +44-1253-300-000, ext. 4056; fax: +44-1253-303-669
e-mail: sdevbhandari{at}aol.com
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Abstract
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Mucoepidermoid carcinoma is a rare type of tumor of bronchial glands. We describe an unusual presentation of mucoepidermoid carcinoma in a 19-year-old man with atypical pneumonia, deep vein thromboses and recurrent pulmonary embolism, which, to the best of our knowledge has not previously been reported.
Key Words: Lung cancer • Pulmonary embolism • Mucoepidermoid carcinoma
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1. Case report
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A 19-year-old, previously healthy and fit man, presented to a district hospital with 5 weeks history of purulent sputum, hemoptysis, night sweats and weight loss following a holiday abroad. Chest X-ray showed opacification of the left lower and mid-zones with a pleural effusion (Fig. 1)
. Sputum, pleural aspiration and blood cultures were negative. A computerized tomography (CT) scan showed consolidation of the left lung and bronchoscopy was normal. He was treated for atypical pneumonia with a course of antibiotics and discharged home.
He was readmitted as an emergency, 2 weeks later with bilateral calf pain. On examination deep vein thromboses were detected in both calves for which he was commenced on warfarin and discharged home after a week after the symptoms subsided. He required emergency readmission again after a week due to acute dyspnea. A CT pulmonary angiogram (Fig. 2)
revealed presence of thrombus in the lobar and segmental arteries of the right lower lobe and the first branch artery to upper lobe. The left main pulmonary artery was markedly attenuated with few small vessels extending into the left lung. The only bronchial tree that was visible on the left was the left main bronchus, which appeared to contain fluid distally. The left lung was of mixed attenuation throughout with multiple rounded areas of Hounsfield attenuation approximating water. The appearances were consistent with multiple cavities or masses within the lung. There were subcarinal and right hilar nodes measuring up to 2 cm diameter, which was thought to be reactive due to disease on the left side. Echocardiogram showed normal cardiac function. A search for tumor markers was negative.
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Fig. 2. CT scan showing absence of airway and vascular markings in the left lung with complete consolidation and accompanying pleural effusion.
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He was transferred to chest department in our hospital. A Geenfield caval filter was placed in the inferior vena cava through the right internal jugular vein under ultrasound guide and thrombolysis was commenced with tissue plasminogen activator followed by intravenous heparin. His condition failed to improve. A repeat CT scan showed persistent occlusion of the left main pulmonary artery 1.5 cm from the origin and residual thrombus in the right main pulmonary artery as well as the right lower lobe artery. There were fluid attenuations in the left lung suggestive of necrotic changes along with a large left sided pleural effusion. His general condition worsened and became more symptomatic with fever, dyspnea and hemoptysis. A radio-nucleide perfusion scan confirmed the complete absence of perfusion to the left lung with patchy perfusion defects in the right lung. Intravenous heparin was continued and he was referred to us for a thoracic surgical opinion. In view of his young age a pneumonectomy was planned to remove the presumably non-viable infarcted and necrotic lung, in spite his critical illness.
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2. Operation
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A left posterolateral thoracotomy was performed. There were dense adhesions between lung and the chest wall. The entire lung was found to be solid and it was very difficult to dissect out the hilar structures. Pulmonary artery (PA) and pulmonary veins (PV) were identified by intrapericardial approach and the PV was stapled and divided. The PA was mobilised and a sling was passed around the hilum and tightened as a tourniquet. The lung had to be removed in three fragments to complete the pneumonectomy. Following this, the sling was removed to tidy up all the hilar structures meticulously one at a time. The bronchus was closed with a continuous prolene (Ethicon Ltd, Edinburgh, UK) stitch.
On second post-operative day he developed a bronchopleural fistula and underwent a re-thoracotomy and closure of the bronchial stump with interrupted prolene stitches. He became septic requiring ventilatory support and inotropic support though we failed to isolate the organism. He developed multiorgan failure and died on the 9th post-operative day. The relatives declined an autopsy.
The gross pathologic examination of pneumonectomy specimen showed that almost all the fragments were consolidated with tan-colored firm tissue and focal haemorrhages. Only one of the fragments contained a small area of collapsed lung tissue, which appeared less affected. The majority of blocks processed were diffusely infiltrated by a malignant epithelial tumour, mostly with features of mucoepidermoid carcinoma, focally consistent with adenocarcinoma of no special type. There was no evidence of a high-grade squamous component. The tumour was present at the bronchial resection margin and was also infiltrating the pleural margin.
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3. Discussion
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Mucoepidermoid carcinoma is a rare primary tumour of the tracheo bronchial tree [1]. It tends to affect children and young adults and has been reported to have a more favourable prognosis compared to the commoner bronchogenic carcinomas [2]. However later reports have suggested that the prognosis with this tumour is not as good as once thought, as it can be highly malignant with a poor prognosis [3–5] as evident in our case as well. Because of central location and airway origin it can be visualised and diagnosed by bronchoscopy in the majority of cases.
This case has many unusual features. He presented with deep vein thrombosis (DVT) and recurrent pulmonary embolism (PE). Bronchoscopy was normal despite replacement of entire lung by tumour. The whole lung had turned into a solid mass and had to be removed piecemeal at surgery. Repeated searches for malignancy were negative. Lung biopsy was avoided due to full anticoagulation, negative radiology, young age and more obvious source of embolism in the DVT. A positive biopsy would not have changed our aggressive approach due to his young age. This case has highlighted the diagnostic dilemmas faced by clinicians in thoracic surgery. Wolf has also reported a case where the tumour eluded the diagnosis and was subsequently found on autopsy after patient died of an intracranial metastasis [6].
Recurrent PE can be a manifestation of mucoepidermoid carcinoma (MEC). A possibility of malignancy should be suspected in young patients with this symptom. This tumor can be very aggressive and prove to be rapidly fatal.
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References
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- Miller D.L., Allen M.S. Rare pulmonary neoplasms. Mayo Clin Proc 1993;68:492-498.[Medline]
- Welsh J.H., Maxson T., Jaksic T., Shahab I., Hicks J. Tracheobronchial mucoepidermoid carcinoma in childhood and adolescence: case report and review of literature. Int J Pediatr Otorhinolaryngol 1998;45(3):265-273.[Medline]
- Barsky S.H., Martin S.E., Matthews M., Gazdar A., Costa J.C. Low grade Mucoepidermoid carcinoma of the bronchus with high-grade biological behavior. Cancer 1983;51:1505-1509.[Medline]
- Shimizu J., Watanabe Y., Oda M., Morita K., Tshunezuka Y., Nonomura A. Clinicopathological study of mucoepidermoid carcinoma of the lung. Int Surg 1998;83:1-3.[Medline]
- Metcalf J.S., Maize J.C., Shaw E.B. Bronchial mucoepidermoid carcinoma metastatic to skin. Cancer 1986;58:2556-2559.[Medline]
- Wolf K.M., Mehta D., Clapool W.D. Mucoepicermoid carcinoma of the lung with intracranial metastases. Chest 1988;94:435-438.[Abstract/Free Full Text]
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Abstract
1. Case report
