International Journal of Pediatric Otorhinolaryngology Extra
Volume 7, Issue 1 , Pages 6-8, January 2012

Mucoepidermoid carcinoma of the tongue in a child

  • Mosaad Abdel-Aziz

      Affiliations

    • Study was carried out at Kasr El-Aini Hospital of Cairo University.
    • Corresponding Author InformationCorrespondence address: 2 El-Salam St., King Faisal, Above El-Baraka Bank, Giza, Cairo, Egypt. Tel.: +20 105140161; fax: +20 225329113.

Department of Otolaryngology, Faculty of Medicine, Cairo University, Egypt

Received 9 June 2011; received in revised form 22 July 2011; accepted 24 July 2011. published online 18 August 2011.

Article Outline

Abstract 

Tongue base tumors are not common, they are mostly malignant and although the rarity of mucoepidermoid carcinoma of tongue base, it constitutes more than 50% of malignant lesions of salivary glands in this region. In this report, we present a 15-year old girl with mucoepidermoid carcinoma of tongue base with discussion of histopathological types of the tumor and its management.

Keywords: Tongue base, Mucoepidermoid carcinoma, Minor salivary gland tumors, Pediatric malignancy

 

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1. Introduction 

Mucoepidermoid carcinoma (MEC) is a malignant, locally invasive neoplasm of the salivary glands. Its incidence rate has been reported to be 0.44 per 100,000. It is the most common malignant neoplasm of the salivary glands, especially in the parotid gland, but MEC also can occur in submandibular and minor salivary glands [1]. Minor salivary glands are scattered in different areas of the oral cavity; their tumors constitute a small proportion of all head and neck neoplasms, but are more frequently malignant [2].

Although tongue base tumors are not common, they are mostly malignant and although the rarity of MEC of tongue base, it constitutes more than 50% of malignant lesions of salivary glands in this region [3]. In this report, we present a 15-year old girl with MEC of tongue base with discussion of histopathological types of the tumor and its management.

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2. Case presentation 

A 15-year-old girl referred to the Department of Otolaryngology of Kasr El-Aini Hospital (Cairo University) with tongue mass. The patient had complained of dysphagia, throat pain, and pain in her right ear for three months. Examination of the patient revealed an oval mass on the dorsal surface of the right side of tongue base that was seen easily when the patient protruded her tongue; the mass was 5cm×3cm crossing the midline, it was reddish in color with irregular surface (Fig. 1). Neck examination showed no palpable lymph nodes. Computerized tomography (CT) was requested and it showed a soft tissue mass on the right side of tongue base and crossing the midline, it was infiltrating the muscles but was not involving the whole thickness of the tongue (Fig. 2).

Biopsy was taken under local anesthesia, histopathological examination revealed the diagnosis of mucoepidermoid carcinoma of low grade type. As the morphological appearance of MEC share similarities to metastatic clear cell renal adenocarcinoma and clear cell bronchogenic squamous carcinoma [4], ultrasonography of the kidneys and chest radiograph were performed to rule out metastatic carcinoma from these areas and they proved free kidneys and chest.

Complete surgical excision was done under general anesthesia with nasal endotracheal intubation; a trans-oral excision was performed (Fig. 3). Post-operative antibiotic, analgesics and steroids were given to the patient for three days, the patient was informed to eat cold semisolid foods for one week and he had been discharged from hospital in the 3rd post-operative day. Histopathological examination of the specimen revealed a low grade mucoepidermoid carcinoma with negative margins (Fig. 4).

Follow-up of the patient for 3years showed no recurrence. Informed consent was obtained from the parents to publish the case, and the work has been approved by the Institutional Ethical Committee, also the principles outlined in the Declaration of Helsinki were followed.

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3. Discussion 

Salivary gland tissues are distributed widely in the upper aerodigestive tract; the major salivary glands are the parotid, submandibular and sublingual glands, while minor salivary glands are distributed in many sites, such as the lips, cheek, palate, tongue, oropharynx, paranasal sinuses, parapharyngeal space and supraglottic larynx. Neoplasms originating from salivary glands comprise about 3–6% of all head and neck tumors, with an estimated global incidence of 0.4–13.5 per 100,000 persons annually [5], [6]. Neoplasms of minor salivary gland origin occur much less commonly than those arising from major salivary glands; however, MEC is the commonest malignant tumor of major and minor salivary glands [1], [2], [3].

Malignant tumors of the intra-oral minor salivary glands constitute 2–3% of all malignant neoplasms of the upper aerodigestive tract [7]. MEC is composed histologically of epidermoid cells and mucin-producing cells, which take origin from the duct epithelial lining. The epidermoid cells proliferate in sheets or islands, and keratinizing may occur. When the epidermoid element predominates, the histological appearance of the tumor may closely resemble that of squamous cell carcinoma, and it is thus classified as a high-grade MEC tumor. Conversely, the presence of mucin-producing cells within a predominately cystic architecture is regarded as low-grade MEC tumors [2], [4]. However, metastasis and tumor-related death have been noted with low-grade rather than high-grade tumors [8], in the Armed Forces Institute of Pathology series of 227 cases of MEC, 17 patients (7%) with low-grade tumors had local metastases (n=7) or died of disease (n=10) [9]. Dillard et al. [8] have suggested a role for transforming growth factor β1 (TGF-β1) in tumor behavior as elevated levels of TGF-β1 increase desmoplasia, angiogenesis, and tumor progression, with loss of expression of TGF-β RII (which is a receptor complex for TGF-β1) may define a transition from low- to high-grade MEC. Other factors that may affects tumor progression may be the proliferating cell nuclear antigen which may increases with the grade of malignancy [10], also, mucin expression patterns can be used as a prognostic index; membrane-bound mucins (MUC) are expressed on the cell surfaces of MEC. Studies have shown that the presence of MUC-1 is related to aggressive tumor, while MUC-4 represented greater cellular differentiation and better prognosis [11], [12].

The treatment of the low grade MEC of the minor salivary glands involves wide local excision with adequate tumor-free margins, while high grade tumors require more aggressive surgery with or without postoperative radiotherapy and chemotherapy [13], [14].

Leong et al. [2] reported MEC in a 27year-old man that presented with severe bleeding per mouth necessitating emergency management to control bleeding and tracheostomy was performed to protect the airway. Surgical excision of the tumor was done trans-orally using CO2 laser, although there was no lymph node metastasis, the authors performed selective neck dissection on the side of the tumor as it was of high-grade type, also the patient received post-operative radiotherapy and chemotherapy. In our case, we did not need to perform neck dissection as the tumor was of low-grade type with no metastatic neck nodes. Conley and Tinsley [15] have reported that lymph node metastases occur in nearly three-quarter of patients at presentation with high grade-cancer, Jones [16] advised performance of selective neck dissection for patients with high-grade or large lesions N0 neck and radical neck dissection for patients with positive neck nodes. Although MEC is a radio-resistant tumor, post-operative irradiation is suggested by many authors in high-grade type and if the surgical margin was not free [13], [14], [17].

Follow up of our case for 3years showed no recurrence. King and Fletcher [17] reported that the five-year survival rate is about 70% for low-grade tumor and a 47% survival for high-grade tumor, while other authors reported a higher rate of survival for low-grade type (96%) with death rate ten times higher in high-grade lesion [18]. However, some authors recommended follow up for life as recurrence may be delayed for years [2], [19], [20].

In summary, we report mucoepidermoid carcinoma of the tongue in a 15-year old girl; the tumor was of low-grade type. Less aggressive treatment in the form of local trans-oral excision was sufficient with no recurrence for 3years follow up. Since metastasis is uncommon but not unheard of, long-term surveillance is recommended.

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Financial support 

There are no financial disclosures.

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Conflicts of interest 

There are no conflicts of interest.

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References 

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PII: S1871-4048(11)00059-1

doi:10.1016/j.pedex.2011.07.004

International Journal of Pediatric Otorhinolaryngology Extra
Volume 7, Issue 1 , Pages 6-8, January 2012

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