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Mucoepidermoid carcinoma is the most common malignant tumor of salivary glands. However, it is a rare entity in larynx. Laryngeal cases are frequently misdiagnosed with other malignancies and they are under-reported. So, recognizing the clinical and histological features of this tumor is essential. Laryngeal mucoepidermoid carcinoma can arise in su...
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Citations
... Hellquist et al. highlighted that, in the presence of a high transformation of head and neck AdCC, the risk of nodal metastasis is 5-10 times higher than conventional AdCC and therefore elective neck dissection is strongly advised in patients with this highly aggressive variant [106]. Even in laryngeal MEC, there is general consensus about the need for elective treatment of the neck in the presence of high-grade lesions [107,108]. ...
Malignant tumors of minor salivary glands (MiSGMTs) are rare, the majority of them being located in the oral cavity and oropharynx. Adenoid cystic carcinoma (AdCC) is the most frequently encountered histologic type followed by mucoepidermoid carcinoma (MEC); however, many other malignant salivary tumor types have also been described. Presenting complaints of MiSGMT depend on the anatomic site of origin. A painless submucosal swelling is the most frequent finding, possibly associated with obstructive symptoms when the tumor is located in the sinonasal cavities, pharynx, larynx, or trachea; pain or nerve impairment may also be reported.
... Nearly half of the cases develop cervical lymph node and visceral metastases, particularly in the lung [61,62]. Lymph node metastases of MEC may be observed with non-enhancening nodes on cross-sectional imaging, which correspond to necrosis, tumor keratinization or a cystic nature [63]. ...
Salivary gland carcinomas of the larynx are uncommon. Adenoid cystic carcinoma is the most prevalent type of salivary gland carcinoma in this region, although other histologies such as mucoepidermoid carcinoma and adenocarcinomas have been reported. These tumors may present with advanced-stage due to nonspecific symptoms and their relatively slow-growing nature. The index of suspicion for a non-squamous cell carcinoma entity should be high when a submucosal mass is present. An accurate diagnosis is mandatory due to the impact each biologic entity has on treatment and outcome. Data concerning treatment and outcome are scarce, but primary surgery with utmost focus on free surgical margins is the treatment of choice. The role of adjuvant radiotherapy has not been well defined, although there is an agreement that it should be considered in advanced-stage or high-grade disease. This review considers only the most common malignant salivary neoplasms of the larynx with a focus on clinical management of these tumors.
Objective
Mucoepidermoid carcinomas (MEC) of the larynx account for less than 1% of all laryngeal tumors. The unique features and clinical behavior of these rare entities remain unknown. To fill this knowledge gap, we performed a scoping review of every reported case of laryngeal MEC to study the clinical behavior and the treatment modalities in this rare entity.
Review Methods
We followed the guidelines outlined in the Preferred Reporting Items for Systematic Reviews and Meta‐Analyses extension for scoping reviews (PRISMA‐ScR) statement.
Data Sources
MedLine, Google Scholar, and Science Direct databases were searched to identify eligible studies.
Results
We concluded with 25 studies and overall 66 cases. Our study revealed that the average age of cases was 55.7 years, and the range was from 12 to 81 years, with younger onset in females. Supraglottic (60%) is the most common subsite of laryngeal MEC s followed by glottis (27.3%). Supraglottic carcinomas frequently present with metastatic neck disease at the time of the initial diagnosis and require more extensive surgical approaches. Surgery with negative surgical margins seems to be the cornerstone in the treatment of MEC. Radiation therapy has not been tested widely as monotherapy but is considered a useful adjuvant modality.
Conclusion
This study suggests that negative surgical margins seem to be associated to higher disease control rate and that high‐grade supraglottic cases likely benefit from addressing neck disease simultaneously.
Level of Evidence
NA Laryngoscope , 2023
The pathology of non-squamous carcinoma of the larynx is broad and there is a wide differential diagnosis. The most common presenting symptoms for laryngeal malignancies, both squamous and non-squamous, are hoarseness and dyspnea. Presentation with persistent or worsening symptoms and a submucosal lesion should raise suspicion for a non-squamous malignancy of the larynx. Accurate histology determines the most appropriate treatment and has an impact on prognosis.
Mucoepidermoid carcinoma is a common malignant neoplasm of the salivary glands. While quite common in the oral cavity, it is rare in the larynx. A middle-aged male patient presented to the otolaryngology clinic of our institution with the chief complaint of hoarseness. A supraglottic subepithelial mass was detected on
the left laryngeal ventricle after a comprehensive clinical examination. Eventually, the diagnosis was established with a biopsy after a direct laryngoscopy. The multidisciplinary team of our institution suggested total laryngectomy without adjuvant modalities. An uneventful procedure followed and the patient remains free of disease and up to date. Mucoepidermoid tumors of the larynx are rare and surgical treatment is strongly indicated as the treatment of choice.
Background
The objective of this manuscript is to report a rare case of laryngeal cancer: mucoepidermoid carcinoma of the larynx and its treatment with radiotherapy alone.
Case presentation
We report a case of a 46-year-old male Lebanese patient who presented to the ENT clinic for chronic dysphonia. He is a heavy smoker and an occasional drinker, with no history of voice overuse. A laryngeal endoscopy has shown leukoplakia all along the mobile right vocal cord. The biopsy findings of the lesion were consistent with intermediate-grade mucoepidermoid carcinoma of the larynx. A review of the pathology has yielded the same results. No distant metastases were found on PET-scan. After discussing the treatment options with the patient, we proceeded with radiotherapy. The patient remains disease-free 5 years after completion of treatment.
Conclusions
Mucoepidermoid carcinoma of the larynx is very rare. It is mainly known to affect the parotid gland. Moreover, when surgery is considered as first-line therapy, we treated the patient with radiotherapy alone.
Objectives
Discussions regarding the specific management and outcomes for laryngeal MEC are limited to very small, single-institution case series. To look further into the diagnosis and management of these uncommon non-squamous cell carcinomas of the larynx, we present 3 recent cases of laryngeal MEC treated at our institution.
Methods
Patients at a tertiary hospital treated for MEC between October 2019 and December 2020 were retrospectively identified. Chart review, imaging analysis, and histologic slide creation were completed for all patients.
Results
We identified and treated 2 patients with high-grade supraglottic and 1 patient with intermediate-grade glottic MEC. These patients presented to our clinic with a primary complaint of either gradual, worsening dysphonia, dysphagia, or both. All patients underwent laryngovideostroboscopy as well as panendoscopy with directed submucosal biopsy, which was consistent with MEC. MRI was performed in 2 of the cases further elucidating the extent of submucosal spread. PET-CT was performed in all 3 cases, and none demonstrated evidence of regional or distal metastases. Surgically, high-grade MEC lesions were treated with a total laryngectomy. The intermediate MEC lesion was managed with a supracricoid partial laryngectomy (SCPL). Surgical margins were free of tumor in all cases with no nodal metastases by modified radical neck dissection. Radiation therapy was offered to both high-grade MEC patients and declined by one. Radiation was not recommended to the patient with intermediate-grade MEC as we believed that the risk of additional treatment outweighed the benefit.
Conclusion
We believe that MEC of the larynx should be considered in patients with atypical submucosal laryngeal masses. Laryngovideostroboscopy, MRI, and PET imaging may be valuable in determining the extent of the lesions and planning appropriate surgery. Postoperative radiation therapy should be considered a per tumor grade in other more studied sites, as there is no data on efficacy in laryngeal MEC.
The recently updated WHO classification of head and neck tumors has listed more than 20 (sub-)types of salivary gland cancers. Although there was consensus of the Board that diagnosis on histological criteria alone may be inaccurate, the editors finally reasoned that the necessary set up for molecular analyses is not globally available, as yet, or the data are either not convincing or robust enough to supplement the histological and immunohistochemical diagnostic tools. Nevertheless, the increasing knowledge about tumor-type specific translocations, point mutations, and amplifications in salivary gland cancers needs more explanatory comments than the new WHO fascicle could afford, particularly taking into account the already established molecular support of diagnostic, and predictive pathology in specialized clinical centers. In the German Salivary Gland Expert Network (www.hansepathnet.de), we advocate the application of molecular analyses for clinicopathological purposes in mucoepidermoid, adenoid cystic, and secretory carcinomas, while more translational research is necessary before molecular tools can be applied in other neoplasias covered in this chapter, in routine clinical practice.
