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Ann Thorac Surg 1998;66:928-929
© 1998 The Society of Thoracic Surgeons

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Case Reports

Tracheal mucoepidermoid carcinoma in a 7-year-old child

^a Division of Cardiothoracic Surgery, Department of Surgery, Washington University School of Medicine, St. Louis, Missouri, USA

Accepted for publication March 1, 1998.

Address reprint requests to Dr Mendeloff, Division of Cardiothoracic Surgery, Washington University School of Medicine, One Barnes Hospital Plaza, Suite 3108 Queeny Tower, St. Louis, MO 63110

	Abstract

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Mucoepidermoid cancers are rare tumors that arise from the serous and mucous glands of the upper airway and salivary glands. Patients, especially children, with tumors that arise in the trachea and upper airways are often misdiagnosed as asthmatic and are treated with bronchodilators without resolution. A 7-year-old girl who had been diagnosed as asthmatic was subsequently found to have a mucoepidermoid tumor of the trachea. She underwent a successful tracheal resection and remains tumor free on follow-up bronchoscopy at 16 months.

	Introduction

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Mucoepidermoid tumors represent approximately 0.2% of all lung tumors. They can occur in all age groups and have been reported in patients ranging in age from 9 years to 62 years [1]. The clinical course of these tumors varies and has been correlated with the pathologic grading. Complete surgical resection remains the treatment of choice for mucoepidermoid carcinomas.

The patient is a 7-year-old girl with no significant past medical history who over the past 2 years had been undergoing treatment for worsening asthma. She had shortness of breath with obstructive airway symptoms and pulmonary function tests that demonstrated worsening obstructive disease with forced expiratory volume in 1 second of 28%.

A chest radiography that was initially interpreted as normal in retrospect demonstrated the mass in the trachea (Fig 1). A computed tomographic scan was obtained, which demonstrated a large, circular mass approximately 2 cm above the carina, which virtually obstructed the lumen of the trachea (Fig 2). There was no mediastinal lymphadenopathy or evidence of tracheal invasion. She also had no evidence of any metastatic disease on abdominal and pelvic computed tomographic scans.

View larger version (126K): [in this window] [in a new window]   Fig 1. Chest radiograph demonstrating mass in the trachea (arrow).

View larger version (117K): [in this window] [in a new window]   Fig 2. Chest computed tomographic scan demonstrating mass in the lumen of the trachea.

The patient subsequently underwent a tracheal resection with primary anastomosis through a right thoracotomy. An infrahilar pericardial incision was created to mobilize the trachea and allow for a tension-free anastomosis. A total of six tracheal rings were resected and sent for pathologic examination.

The remainder of her postoperative course was uncomplicated, and she was discharged to home on postoperative day 7. She remains tumor free on surveillance bronchoscopy at 16 months.

	Comment

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Mucoepidermoid cancers of the trachea are extremely rare tumors that arise from the serous and mucous glands of the upper airway. These tumors represent approximately 0.2% of the tumors of the lung. Heitmiller and associates [1] described the minimum features required for the diagnosis of a mucoepidermoid cancer as (1) origin within a conducting airway that contains cartilage, (2) presence of squamous or intermediate elements that are manifested as either intercellular bridges or cytoplasmic membranes, and (3) presence of glandular elements that are manifested as either individual mucous cells, glandular cells, or signet ring cells.

These tumors generally produce symptoms of upper airway irritation such as coughing, hemoptysis, or, as in the current case, airway obstruction. Younger patients are often mistakenly diagnosed as having chronic obstructive airway disease. After symptoms persist despite medical therapy, these patients often undergo further workup and are found to have tumors obstructing the upper airways. In this age group one must always consider the possibility of a foreign body. The most common histologic tracheal tumor is an epidermoid carcinoma. Bronchial adenomas and adenoid cystic carcinomas are also seen.

In the largest reported series to date from the Massachusetts General Hospital [1], 13 of the 18 patients had an abnormal chest radiograph on admission. The most common finding on radiologic evaluation was the tumor mass itself followed by postoperative pneumonia and atelectasis. Bronchoscopy with biopsy remains the main diagnostic modality. Biopsies and removal of the tumors can be safely performed without significant risk of hemorrhage.

The clinical course of these tumors correlates with the histologic grade of the tumor. Low-grade tumors generally grow locally and are amenable to complete surgical resection. Bueno and coworkers [3] described parenchyma-sparing bronchoplastic techniques for bronchial tumors. Even in the pediatric population, bronchoplastic techniques can be used with successful outcomes, as described by Gaissert and associates [4]. Patients who undergo complete resection of mucoepidermoid cancers can expect to have normal life expectancies, whereas patients with high-grade lesions have been reported to have an extremely poor prognosis. Breyer and colleagues [2] reviewed 12 patients who were found to have high-grade tumors and all 12 died within 18 months. The tumor spreads by local invasion as well as through lymphatics, and hilar lymph node involvement has been reported to be the worst prognostic indicator in these patients. Patients who have not undergone complete resection are generally treated with radiation therapy, although the efficacy of this modality has yet to be determined.

We report a case of a tracheal mucoepidermoid carcinoma successfully treated with tracheal resection in a 7-year-old girl. The lesion was endoscopically removed for pathologic and histologic grading before definitive resection. Using bronchoplastic techniques, we reconstructed the trachea without complication. The patient remains free of recurrence at 16 months.

	References

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1. Heitmiller R.F., Mathisen D.J., Ferry J.A., Mark E.J., Grillo H.C. Mucoepidermoid lung tumors. Ann Thorac Surg 1989;47:394-399.[Abstract/Free Full Text]
2. Breyer R.H., Dainauskas J.R., Jensik R.J., Faber L.P. Mucoepidermoid carcinoma of the trachea and bronchus: the case for conservative resection. Ann Thorac Surg 1979;29:197-204.
3. Bueno R., Wain J.C., Wright C.D., Moncure A.C., Grillo H.C., Mathisen D.J. Bronchoplasty in the management of low-grade airway neoplasms and benign bronchial stenosis. Ann Thorac Surg 1996;62:824-829.[Abstract/Free Full Text]
4. Gaissert H.A., Mathisen D.J., Grillo H.C., Vacanti J.P., Wain J.C. Tracheobronchial sleeve resection in children and adolescents. J Pediatr Surg 1994;29:192-198.[Medline]

This article has been cited by other articles:

				M. Papiashvilli, D. Ater, A. Mandelberg, and L. Sasson Primary mucoepidermoid carcinoma of the trachea in a child Interact CardioVasc Thorac Surg, August 1, 2012; 15(2): 311 - 312. [Abstract] [Full Text] [PDF]

				B. Niggemann, B. Gerstner, M. Guschmann, K. Paul, J. Wit, H. Mau, and U. Wahn An 11-yr-old male with pneumonia and persistent airway obstruction Eur. Respir. J., March 1, 2002; 19(3): 582 - 584. [Full Text] [PDF]

This Article Abstract Full Text (PDF) Alert me when this article is cited Alert me if a correction is posted Citation Map Services Email this article to a friend Similar articles in this journal Similar articles in PubMed Alert me to new issues of the journal Add to Personal Folders Download to citation manager Author home page(s): Seiichi Noda Sudhir Sundaresan Eric N. Mendeloff Permission Requests Citing Articles Citing Articles via HighWire Citing Articles via Google Scholar Google Scholar Articles by Noda, S. Articles by Mendeloff, E. N. Search for Related Content PubMed PubMed Citation Articles by Noda, S. Articles by Mendeloff, E. N.