A rare case of mucoepidermoid carcinoma of the nasal cavity. (Original Article).
Mucoepidermoid carcinoma of the nasal cavity is rare. We report the case of a 57-year-old man who was evaluated for a rapidly enlarging subcutaneous mass on the nasal bridge. The tumor was diagnosed as a mucoepidermoid carcinoma. The patient underwent extensive surgical resection and postoperative radiotherapy, but 5 months later he required orbital exenteration for persistent disease. Despite these radical measures, the patient died with persistent disease 9 months following the initial evaluation. The aggressive behavior of this tumor highlights the need for physicians to be aware of the differential diagnosis, symptoms, and signs of neoplasms that originate in the nasal cavity so that prompt treatment can be instituted.
Mucoepidermoid carcinoma occurs most often in the major salivary glands, the minor salivary glands of the oral cavity and pharynx, and the lacrimal glands. Only rarely does it arise in the respiratory tract (i.e., the larynx, trachea, and bronchi) or the sinonasal cavities. (1) According to combined reports of four large series of major and minor salivary gland tumors, sinonasal mucoepidermoid carcinoma accounts for 0.6% of all salivary tumors and 4.8% of all mucoepidermoid carcinomas. (2) To our knowledge, only 38 cases of sinonasal mucoepidermoid carcinoma have been previously reported in the English-language literature. (2)
When these tumors do occur in the sinonasal tract, the most common site is the maxillary antrum; other sites, in order of decreasing incidence, include the nasal cavity, nasopharynx, and ethmoid sinuses. This pattern most likely reflects the relative distribution of minor salivary glands in this area of the upper aerodigestive tract. In this article, we describe a new case of mucoepidermoid carcinoma of the nasal cavity, and we review the diagnosis, histopathologic features, and treatment options regarding this aggressive disease.
An otherwise healthy 57-year-old white man was referred to the Miami Veterans Affairs Medical Center for evaluation of a rapidly enlarging subcutaneous mass over his upper nasal dorsum. He complained that he had experienced severe facial pain and intermittent epistaxis during the preceding 9 months. His history was significant for tobacco, marijuana, and cocaine use.
Facial examination revealed that the nasal bridge had been widened by a subcutaneous mass that extended from the glabellar area to the rhinion (figure 1). At the area of the left medial canthus, the mass had eroded the bone and extruded through the skin. Intranasal examination detected a large septal perforation with friable borders and the presence of soft tissue that filled the upper nasal cavity. The patient's extraocular muscle movements and visual acuity were intact bilaterally. Neck examination identified no adenopathy or masses. Computed tomography (CT) and magnetic resonance imaging (MRI) were performed to evaluate the extent of local disease and possible intracranial extension. Although there was no evidence of intracranial extension on these images, the mass extended to the cribriform plate.
A biopsy specimen of the mass was consistent with high-grade mucoepidermoid carcinoma. The patient underwent anterior craniofacial resection, total rhinectomy, medial maxillectomies, and resections of the ethmoid and sphenoid sinuses. All tissue margins obtained intraoperatively were negative for tumor on frozen-section analysis.
The patient's recovery was uneventful, and he was started on postoperative radiotherapy. Five months after the completion of radiotherapy, he complained of increasing facial and eye pain. Persistent disease was found in the right infraorbital region and right eye. After providing adequate counseling and obtaining informed consent, we performed a right orbital exenteration with a complete maxillectomy. Intraoperatively, microscopic mucoepidermoid carcinoma was identified in the pericranial flap that had been used to reconstruct the cribriform plate during the first resection. After extensive discussions with the patient, his family, and the multidisciplinary treatment team, no further therapeutic intervention was planned. The patient died from meningitis 9 months following his initial evaluation.
Malignant tumors of the nasal cavity are rare. The differential diagnosis of cancer in this area includes squamous cell carcinoma, adenocarcinoma, undifferentiated carcinoma, melanoma, lymphoma, and, rarely, mucoepidermoid carcinoma. Muir and Nectoux studied tumor registry data on 1,130 male and 691 female patients who had cancer of the nose, nasal cavity, middle ear, or accessory sinuses and found a predominance of squamous cell carcinomas (60% of cases). (3) Other common findings were undifferentiated carcinomas (10.2%) and adenocarcinomas (9.4%). Melanomas and lymphomas accounted for 4.9 and 3% of cases, respectively. However, in more recent studies, lower rates of squamous cell carcinoma have been reported. For example, Haraguchi et al reviewed 60 cases of cancer of the nasal cavity and found that squamous cell carcinoma was still the most common type, but it had accounted for only 25% of cases; melanoma and lymphoma accounted for 23% each, and mucoepidermoid carcinoma accounted for 1.7%. (4)
Sinonasal tract adenomatous tumors have been presumed to arise from submucosal mucoserous glands. However, a study of the mucosal origin of 107 sinonasal tract adenomatous tumors by Gnepp and Heffner found that most (including 83% of mucoepidermoid carcinomas) originated in the surface mucosa. (5) The mucoepidermoid carcinomas in their series were frequently accompanied by abnormal (dysplastic) surface mucosa adjacent to the tumor. These findings suggest that mucoepidermoid carcinoma of the sinonasal tract, like squamous cell carcinoma, can be preceded by mucosal dysplasia or carcinoma in situ.
Although no definitive risk factors for mucoepidermoid carcinoma have been identified, minor trauma and chronic irritation have been implicated in the etiology of sinonasal tract cancers in general. Reports of other causes have been sporadic; these causes include vitamin A deficiency, occupational factors (e.g., working with wood dust, industrial toxins, nickel, chrome, leather, textiles, and clothing), and radiation exposure. There is no persuasive evidence that cigarette smoking is an etiologic factor in sinonasal carcinoma.
Symptoms and physical findings in patients with mucoepidermoid carcinoma of the sinonasal tract can include nasal obstruction, epistaxis, facial pain, diplopia, and/or a subcutaneous mass if the tumor has eroded bone and invaded the subcutis. Imaging studies, including CT, should be part of the early clinical management to determine if a neoplasm is present. When a mass is identified, MRI is essential for assessing possible intracranial extension.
The histopathologic picture of mucoepidermoid carcinoma includes varying proportions of cystic and solid epithelial elements made up of different proportions of mucous, epidermoid, intermediate, and clear cells (figure 2). Intermediate cells usually predominate; they are round to ovoid and smaller than epidermoid (squamous) cells. Mucous cells can be sparse or inconspicuous, but their presence is required for a diagnosis; special stains for mucin--such as mucicarmine or alcian blue (figure 3)--might be needed to identify them. Clear cells often contain glycogen and are probably altered epidermoid cells.
Tumor grading is based on morphologic and cytologic features. The five main grading parameters are (1) the proportions of cystic and solid elements, (2) the presence or absence of neural invasion, (3) the presence or absence of necrosis, (4) the mitotic rate, and (5) the presence or absence of cellular anaplasia. A quantitative system assigns a point value to each parameter, and the total score determines the tumor grade (low, intermediate, or high). (6)
We found no reported series of cases of mucoepidermoid carcinoma of the nasal cavity in which the treatment strategy and clinical outcomes could be assessed. However, this information can be extracted from reports that described mucoepidermoid carcinoma in all sites in the head and neck. One of the largest such series (367 patients), reported by Spiro et al, included 18 cases of mucoepidermoid carcinoma of the sinonasal tract. (7) Spiro et al reported that the overall 10-year determinate cure rate for mucoepidermoid carcinoma in all head and neck sites was 90% for low-grade tumors and 42% for high-grade tumors. In addition to high tumor grade, other predictive factors that have been associated with poor 5-year survival include bone invasion, age beyond 60 years, pain, positive cervical nodes, and facial nerve palsy. In a series of 60 patients with mucoepidermoid carcinoma of the head and neck, Healey et al described five patients who had high-grade mucoepidermoid carcinoma of the maxillary antrum; four of the five died of local disease despite radical surgery and radiotherapy. (8)
The findings described in these reports imply that treatment of mucoepidermoid carcinoma of the nasal cavity should be based on the tumor grade, the extent of tumor invasion, and the medical condition of the patient. Surgical resection alone has been advocated for low-grade mucoepidermoid carcinoma. For intermediate and high-grade tumors, however, complete resection and postoperative radiation are recommended. Clinical nodal disease should be treated with appropriate neck dissection and postoperative radiotherapy if indicated. Because of the small number of reported cases of mucoepidermoid carcinoma of the nasal cavity and the paucity of data on the use of chemotherapy in these patients, the role of chemotherapy as an adjunct to local treatment cannot currently be assessed.
In summary, mucoepidermoid carcinoma of the nasal cavity is rare. Affected patients can manifest the signs and symptoms typical of chronic sinusitis, such as nasal obstruction and epistaxis. CT should be part of the early management of these patients to look for a possible tumor. Although the pathologic diagnosis of low- and intermediate-grade mucoepidermoid carcinoma is relatively straightforward, high-grade mucoepidermoid carcinoma can be difficult to differentiate from squamous cell carcinoma and therefore might require special histologic stains to demonstrate the mucous component. Low-grade mucoepidermoid carcinoma can often be treated with surgical resection alone, depending on the extent of the disease. Combined surgery and postoperative radiotherapy has been advocated for intermediate- and high-grade tumors. However, even with aggressive measures, high-grade mucoepidermoid carcinoma of the nasal cavity is associated with poor survival. Therefore, early treatment is of the utmost importance.
(1.) Kaznelson DJ, Schindel J. Mucoepidermoid carcinoma of the air passages: Report of three cases. Laryngoscope 1979;89:115-21.
(2.) Simpson RJ, Hoang KG, Hyams VJ, Jarchow RC. Mucoepidermoid carcinoma of the maxillary sinus. Otolaryngol Head Neck Surg 1988;99:419-23.
(3.) Muir CS, Nectoux J. Descriptive epidemiology of malignant neoplasms of nose, nasal cavities, middle ear and accessory sinuses. Clin Otolaryngol 1980;5:195-211.
(4.) Haraguchi H, Ebihara S, Saikawa M, et al. Malignant tumors of the nasal cavity: Review of a 60-case series. Jpn J Clin Oncol 1995;25: 188-94.
(5.) Gnepp DR, Heffner DK. Mucosal origin of sinonasal tract adenomatous neoplasms. Mod Pathol 1989;2:365-71.
(6.) Ellis GL, Auclair PL. Tumors of the Salivary Glands. 3rd series, fascicle 17. Washington, D.C.: Armed Forces Institute of Pathology, 1996.
(7.) Spiro RH, Huvos AG, Berk R, Strong EW. Mucoepidermoid carcinoma of salivary gland origin. A clinicopathologic study of 367 cases. Am J Surg 1978:136:461-8.
(8.) Healey WV, Perzin KH, Smith L. Mucoepidermoid carcinoma of salivary gland origin. Classification, clinical-pathologic correlation, and results of treatment. Cancer 1970;26:368-88.
From thc Department of Otolaryngology--Head and Neck Surgery (Dr. Thomas and Dr. McClinton) and the Department of Pathology (Dr. Regalado), University of Miami School of Medicine.
Reprint requests: Dr. Giovana R. Thomas, Department of Otolaryngology--Head and Neck Surgery, University of Miami School of Medicine, 1550 NW 10th Ave., Papanicolaou Bldg., Room 214, Miami, FL 33136-1013. Phone: (305) 243-3362; fax (305) 324-3254; e-mail: firstname.lastname@example.org
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|Publication:||Ear, Nose and Throat Journal|
|Date:||Aug 1, 2002|
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