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Interact Cardiovasc Thorac Surg. 2012 August; 15(2): 311–312.
Published online 2012 May 18. doi:  10.1093/icvts/ivs199
PMCID: PMC3397757

Primary mucoepidermoid carcinoma of the trachea in a child

Abstract

Mucoepidermoid carcinoma of the trachea is a rare tumour, especially in the paediatric population. We report the case of a 9-year-old boy with mucoepidermoid carcinoma of the trachea that was preoperatively diagnosed as an intraluminal polypoid mass arising from the trachea and extending into the right main bronchus. A complete resection of the tumour with reconstruction and end-to-end anastomosis of the trachea was performed. The patient is now, 24 months after surgery, free of disease.

Keywords: Trachea, Mucoepidermoid carcinoma, Child, Tracheal resection, End-to-end anastomosis, Reconstruction

INTRODUCTION

Primary malignant tracheal neoplasms in children are rare. The presenting symptoms are generally dyspnoea, coughing, wheezing, haemoptysis or obstructive pneumonia. There have only been a few cases, predominantly of boys ranging in age from 4 to 9 years, described in the literature [15]. We present a 9-year-old previously healthy child with a tracheal tumour in whom the symptoms were considered to be caused by asthma. The child underwent emergency preoperative and intraoperative diagnoses of the tumour and successful resection of two tracheal rings together with the tumour followed by end-to-end anastomosis.

CASE REPORT

A 9-year old boy was referred to our hospital from the Gaza Strip in the Palestinian Authority. Six months prior to admission, the child was diagnosed with asthma because he had dyspnoea and was coughing, and treatment with bronchodilators was started without any improvement in his respiratory status. One week prior to admission at our hospital, the patient was hospitalized in the local hospital with progressing dyspnoea and was intubated for 24 h with the existence of a tracheal tumour not being known. After successful extubation, the child was still dyspnoeic, and due to suspected heart disease, was transferred to our centre for further evaluation.

On admission to the emergency room the child was dyspnoeic, with 36 breaths a minute, and diaphoretic. His head was sunken into his shoulders as in the case of heavy-smoking chronic obstructive pulmonary disease patients. A chest X-ray did not reveal any abnormality, and cardiac problems were excluded by echocardiography. A computed tomography (CT) scan of the chest and upper abdomen was then carried out, which surprisingly revealed a tracheal orifice completely blocked by a tumour that was above the level of the carina and also extended into the right main bronchial orifice (Fig. 1). CT three-dimensional reconstruction of the trachea revealed a large broad-based tracheal tumour originating from the lower tracheal rings.

Figure 1:
CT scan of the child with a tracheal tumour.

The patient was immediately transferred from the emergency room to the operating theatre where a flexible bronchoscopy, carried out under general anaesthesia, revealed a large red sessile tumour in the lower trachea, completely blocking the tracheal orifice. The option of endoscopic resection or biopsy of the tumour prior to surgery was discarded due to the immobility of the tumour and difficulty ventilating the child. Due to the existence of immediate cardiopulmonary bypass facilities at our institution, we decided that the optimal surgical approach was via a sternotomy rather than via a right thoracotomy. Following a sternotomy, the anterior and posterior pericardia were opened, the trachea with bifurcation mobilized and, under direct vision of the bronchoscope, the tumour was excised en-bloc with two rings of the trachea. Intraoperative frozen examination revealed a mucoepidermoid tumour of the trachea with tumour-free surgical margins. End-to-end anastomosis was carried out using absorbable 4.0 polydioxanone sutures. Continuous sutures were made on the posterior membrane and interrupted sutures on the cartilaginous part of the trachea. The mediastinum was drained by two 24 French chest tubes, the chest was closed and the child transferred to the paediatric intensive care unit.

The child was extubated 2 days after surgery. The postoperative course was uncomplicated, chest drains were removed on the fifth day following surgery and, on the seventh day after surgery, the child was discharged from the hospital in an excellent general state. The final pathologic examination confirmed the diagnosis of primary low-grade polypoid mucoepidermoid carcinoma originating broadly from the trachea, without the involvement of the surgical margins. No evidence of metastatic mucoepidermoid carcinoma was identified in the five paratracheal lymph nodes that were excised. Follow-up 2 years after surgery is normal and the child is today tumour free.

DISCUSSION

Mucoepidermoid tumours arise from the serous and mucous glands of the upper airways or salivary glands. These tumours are rare and generally discovered in the young population. Mucoepidermoid tumours represent ~0.2% of all lung tumours. Generally, these tumours produce symptoms of upper respiratory tract obstruction, such as dyspnoea, cough, and wheezing, and rarely haemoptysis or atelectasis. Asthma may be mistakenly diagnosed in young patients and in older patients with chronic pulmonary obstructive disease. When the symptoms are not resolved, a more scrupulous workup is usually performed and intratracheal tumours diagnosed. The most common malignant tracheal tumours are squamous cell and adenoid cystic carcinomas. Bronchoscopy with biopsy remains the main diagnostic modality in non-emergency cases.

The clinical course of these tumours correlates with the histological grade of the tumour. Low-grade tumours generally grow locally and are amenable to complete surgical resection with normal life expectancy, whereas patients with high-grade lesions have been reported to have a poor prognosis. Complete resection of the tumour en-bloc with the tracheal rings combined with reconstruction of the trachea is the mainstay of treatment [15]. The role of oncological treatment is unclear because of the small number of patients reported in this age group, who are usually treated successfully by surgery [15]. There are no reports in the literature of palliative treatment for these tumours in children.

We have discussed the case of a tracheal mucoepidermoid carcinoma in a 9-year old boy that was successfully treated with tracheal resection and reconstruction. At present, 24 months after surgery, the child is in good general health and free of disease.

Conflict of interest: none declared.

REFERENCES

1. Romão RL, de Barros F, Maksoud Filho JG, Gonçalves ME, Cardoso S, Tannuri AC, et al. Malignant tumor of the trachea in children: diagnostic pitfalls and surgical management. J Pediatr Surg. 2009;44:e1–4. 4. [PubMed]
2. Chan EY, MacCormick JA, Rubin S, Nizalik E. Mucoepidermoid carcinoma of the trachea in a 4-year-old boy. J Otolaryngol. 2005;34:235–8. [PubMed]
3. Desai DP, Mahoney EM, Miller RP, Holinger LD. Mucoepidermoid carcinoma of the trachea in a child. Int J Pediatr Otorhinolaryngol. 1998;45:259–63. [PubMed]
4. Noda S, Sundaresan S, Mendeloff EN. Tracheal mucoepidermoid carcinoma in a 7-year-old child. Ann Thorac Surg. 1998;66:928–9. [PubMed]
5. Kim J, Park C, Kim K, Shim YM, Yang MK, Han J, et al. Surgical resection of mucoepidermoid carcinoma at the carina in a 9-year-old boy. J Pediatr Surg. 1998;33:1561–2. [PubMed]

Articles from Interactive Cardiovascular and Thoracic Surgery are provided here courtesy of Oxford University Press