[Primary salivary gland tumors from a pathology perspective : Morphomolecular peculiarities and diagnostic and therapeutic challenges]. / Primäre Speicheldrüsentumoren aus Sicht der Pathologie : Morphomolekulare Besonderheiten und diagnostisch-therapeutische Herausforderungen.

Similar to tumors of other organs, salivary gland neoplasms were historically viewed as a single neoplastic entity and mostly treated as such. Accordingly, only the clinical tumor stage, and not the histological subtype, was considered to be of significant prognostic impact. However, over the years, several distinct sub-entities have been characterized based on morphological features, such as adenoid cystic carcinoma, mucoepidermoid carcinoma, acinic cell carcinoma, and salivary duct carcinoma. Most importantly, the nosology of salivary gland carcinomas has undergone a dynamic "splitting" on the basis of morphological, immunophenotypic, and molecular characteristics, so that 21 independent carcinomas are now listed in the current World Health Organization (WHO) classification. Moreover, it has become evident that splitting of these carcinoma subtypes no longer represents a "pathologist's hobby," but carries significant prognostic and therapeutic relevance for optimized cancer surgery and potentially systemic therapy. The current review summarizes the major features of salivary gland tumors, both benign and malignant, and gives an account of their classification systems and genetic profiles.

Prognostic factors and overall survival in a 15-year followup of patients with malignant salivary gland tumors: a retrospective analysis of 193 patients.

INTRODUCTION: Malignant tumors of the salivary glands are uncommon pathological entities, representing less than 5% of head and neck neoplasms. The prognosis of patients with malignant tumors of the salivary glands is highly variable and certain clinical factors can significantly influence overall survival. OBJECTIVE: To analyze the clinicopathologic and sociodemographic characteristics that influence survival in patients with malignant tumors of the salivary glands METHODS: This retrospective study analyzed sex, age, race, education level, tumor location, tumor size, lymph node involvement, distant metastasis, margin status, treatment type, marital status, method of health care access and 15-year overall survival in 193 patients with malignant tumors of the salivary glands. The X², log-rank Mantel-Cox, multinomial regression and Cox logistic regression tests were used (SPSS 20.0,p < 0.05). RESULTS: The most common histological types were adenocarcinoma (32.1%), adenoid cystic carcinoma (31.1%) and mucoepidermoid carcinoma (18.7%). The 15-year overall survival rate was 67.4%, with a mean of 116±6 months. The univariate analysis revealed that male sex (p = 0.026), age > 50 years (p=0.001), referral origin from the public health system (p=0.011), T stage (p= 0.007), M stage (p< 0.001), clinical stage (p< 0.001), compromised surgical margins (p= 0.013), and chemotherapy (p< 0.001) were associated with a poor prognosis. Multivariate analyses also showed that age > 50 years was independently associated with a poor prognosis (p= 0.016). The level of education was the only factor more prevalent in older patients (p= 0.011). CONCLUSION: Patients with malignant tumors of the salivary glands older than 50 years have a worse prognosis and an independent association with a low education level.

Mucoepidermoid carcinoma of the salivary glands revisited with special reference to histologic grading and CRTC1/3-MAML2 genotyping.

Mucoepidermoid carcinoma (MEC) is the most common carcinoma of the salivary glands. Here, we have used two large patient cohorts with MECs comprising 551 tumors to study clinical, histological, and molecular predictors of survival. One cohort (n = 167), with known CRCT1/3-MAML2 fusion status, was derived from the Hamburg Reference Centre (HRC; graded with the AFIP and Brandwein systems) and the other (n = 384) was derived from the population-based Cancer Registry of North Rhine-Westphalia (LKR-NRW; graded with the AFIP system). The reliability of both the AFIP and Brandwein grading systems was excellent (n = 155). The weighted kappa for inter-rater agreement was 0.81 (95% CI 0.65-0.97) and 0.83 (95% CI 0.71-0.96) for the AFIP and Brandwein systems, respectively. The 5-year relative survival was 79.7% (95% CI 73.2-86.2%). Although the Brandwein system resulted in a higher rate of G3-MECs, survival in G3-tumors (AFIP or Brandwein grading) was markedly worse than in G1/G2-tumors. Survival in > T2 tumors was markedly worse than in those with lower T-stage. Also, fusion-negative MECs had a worse 5-year progression-free survival. The frequency of fusion-positive MECs in the HRC cohort was 78.4%, of which the majority (86.7%) was G1/G2-tumors. In conclusion, the AFIP and Brandwein systems are useful in estimating prognosis and to guide therapy for G3-MECs. However, their significance regarding young age (≤ 30 years) and location-dependent heterogeneity of in particular G2-tumors is more questionable. We conclude that CRTC1/3-MAML2 testing is a useful adjunct to histologic scoring of MECs and for pinpointing tumors with poor prognosis with higher precision, thus avoiding overtreatment.

Clinical outcomes of bulky parotid gland cancers: need for self-examination and screening program for early diagnosis of parotid tumors.

BACKGROUND: Early detection and diagnosis of parotid gland cancer (PGC) are essential to improve clinical outcomes, because Tumor-Node-Metastasis stage at diagnosis is a very strong indicator of prognosis in PGC. Nevertheless, some patients still present with large parotid mass, maybe due to the unawareness or ignorance of their disease. In this study, we aimed to present the clinical outcomes of bulky PGC (defined by a 4 cm cutoff point for T3-4 versus T1-2 tumors), to emphasize the necessity of a self-examination tool for parotid gland tumor. METHODS: We retrospectively reviewed 60 consecutive cases with bulky (equal to and greater than 4 cm in the longest diameter, determined radiologically) malignant tumors arising from the parotid gland from 1995 to 2016. The clinical and pathological factors were analyzed to identify risk factors for poor outcomes using Cox proportional hazard models. In addition, we designed a self-examination tool for parotid gland tumors, similar to breast self-examination for breast cancer detection. RESULTS: Patients with bulky parotid cancer showed 48.9% 5-year and 24.5% 10-year overall survival rates and a 47.9% risk of high-grade malignancy. The common pathological diagnoses were carcinoma ex pleomorphic adenoma (18.3%), adenocarcinoma (16.7%), mucoepidermoid carcinoma (16.7%), salivary duct carcinoma (16.7%), and adenoid cystic carcinoma (11.7%). Survival analyses revealed that tumor size (hazard ratio, HR = 1.262 upon increase of 1 cm, 95% confidence interval, 95%CI 1.059-1.502), lymph node metastasis (HR = 2.999, 95%CI 1.048-8.583), and high tumor grade (HR = 4.148, 95%CI 1.215-14.154) were independent prognostic factors in multivariable analysis. Functional preservation of the facial nerve was possible only in less than half of patients. CONCLUSION: In bulky PGC, lymph node metastasis at diagnosis and high tumor grade indicated poor survival outcomes, and functional outcomes of the facial nerve were suboptimal. Thus, a public effort seems to be necessary to decrease these patients with bulky PGC, and to increase patients' self-awareness of their disease. As a way of early detection, we proposed a parotid self-examination tool to detect parotid gland tumors at an early stage, which is similar to breast self-examination.

A Population-Based Analysis of Mucoepidermoid Carcinoma of the Oral Cavity.

OBJECTIVES: To identify survival outcomes for patients with mucoepidermoid carcinoma (MEC) of the oral cavity and the effects of different prognostic factors on survival. STUDY DESIGN: Retrospective cohort study using a national database. METHODS: Retrospective, population-based cohort study of patients in the Surveillance, Epidemiology and End Results (SEER) database who were diagnosed with MEC of the oral cavity from 1973 to 2016. Overall survival (OS) and disease-specific survival (DSS) were calculated. RESULTS: A total of 1693 patients with MEC of the oral cavity were included. Of those, there are 696 males and 997 females, the average age at diagnosis being 52.4 years. The vast majority of cases (86.4%) presented with stage I and stage II disease. A total of 206 patients had received both surgical and radiation therapy (RT), while 1338 patients just had undergone only surgery and 149 with no treatment. On multivariate analysis, advanced age, stage, and histologic grade were associated with worse OS and DSS. Surgical therapy was an independent favorable predictor of OS and DSS. For radiotherapy, multivariate analysis showed that it was associated with worse DSS, whereas there was no significant difference in OS. CONCLUSION: MEC of the oral cavity is associated with a generally favorable prognosis. Advanced age, stage, and histologic grade were independent negative prognostic factors for survival, and surgery was the main treatment to improve survival. LEVEL OF EVIDENCE: 4 Laryngoscope, 131:E857-E863, 2021.

Mucoepidermoid carcinoma of salivary glands: A French Network of Rare Head and Neck Tumors (REFCOR) prospective study of 292 cases.

BACKGROUND: To describe the characteristics of the largest European study of MEC of salivary glands and to determine the prognostic factors for overall and disease free survival. PATIENTS AND METHODS: Patients with MEC were prospectively included in the Réseau d'Expertise Français sur les Cancers ORL Rares (REFCOR, French Network of Rare Head and Neck Tumors) database between 2009 and 2015. RESULTS: A total of 292 patients were included. Tumors were classified as low grade in 175 cases (60%), intermediate in 39 (13%) and high grade in 78 (27%). Median follow-up was 26 months. The 5-year OS and DFS rates were respectively 83% and 69%. In multivariate analysis, age (p = 0.004), diabetes (p = 0.02) and advanced stage (p = 0.03) were found to have a significant negative impact on OS. Diabetes (p = 0.001), alcohol consumption (p = 0.003) and advanced stage (p = 0.001) were found to have a significant negative impact on DFS. Compare to low grade, high grade tended to have a negative impact on OS (p = 0.05) and had a significant effect on DFS (0.002) while intermediate grade had no significant influence on survival. The surgical treatment had a positive impact on both OS (p = 0.00005) and DFS (p = 0.0005). Postoperative radiotherapy had no impact in multivariate analysis. CONCLUSION: Advanced clinical stage, high grade tumor, high age, the impossibility of carrying out a complete surgical resection, and diabetes are the main prognostic factors in this prospective series of patients with MEC. Such findings open new research perspectives on the influence of these components on initial patient care.

Prognostic factors in patients with salivary gland malignancy: a retrospective cohort study.

Our objective was to determine the factors affecting the prognosis in patients with major salivary gland malignancy presenting to Aga Khan University Hospital in Karachi. Retrospective cohort study was carried out at our center on patients diagnosed and treated for salivary gland cancers. Presentation and treatment offered was reviewed from medical charts. Telephonic interviews were conducted to assess the survival of patients who were lost to follow-up. Log rank test was used to compare the mean survival times. A total of 36 patients were included in the study. The mean age was 45.1 +/- 14.6 years. Majority were male 21 (58.3%). The most common malignancy was mucoepidermoid carcinoma (36.1%) followed by adenoid cystic carcinoma (22.2%). Node positivity, grade of tumor, radiotherapy and chemotherapy were a significant indication of survival times on log rank test.

Long-term outcomes of patients in different histological subtypes of primary nasopharyngeal adenocarcinoma: A single-center experience with 71 cases.

OBJECTIVE: This study is aimed to evaluate the long-term outcomes and management approaches in different histological subtypes of primary nasopharyngeal adenocarcinoma (NPAC). MATERIALS AND METHODS: 71 patients with NPAC at our institution between 1984 and 2016 were reviewed, including adenoid cystic carcinoma (ACC) in 43 patients, mucoepidermoid carcinoma (MEC) in 17 patients, and primary traditional adenocarcinoma (AC) in 11 patients. 37 patients received primary radiotherapy and 34 patients underwent primary surgery. RESULTS: The median time of follow-up was 77 months. The 5-year overall survival rate (OS), locoregional failure-free survival rate (LRFFS) and distant metastasis failure-free survival rate (DFFS) were 69.9%, 67.1% and 77.9%, respectively. Patients who received combined modality therapy had better 5-year OS (73.7% vs 66.2%, p = 0.065) and LRFFS (73.1% vs 64.5%, p = 0.047) than patients receiving single modality therapy. Regarding the different histological subtypes, the survival rates of patients with ACC undergoing primary radiotherapy and primary surgery were similar (5-year OS 82.3% vs 68.8%, LRFFS 70.0% vs 70.8%, p＞0.05). As to patients with MEC and AC, those who underwent primary surgery achieved better 5-year OS (75.6% vs 45.5%, p = 0.001) and LRFFS (70.6%% vs 57.1%, p = 0.014) than those who received primary radiotherapy. Multivariate analyses indicated that histological subtypes and radiotherapy technique were independent factors for OS. CONCLUSIONS: The optimal treatment policy for NPAC remained the combination of radiotherapy and surgery. For patients with ACC, radiotherapy could be considered as the primary treatment. Surgery was suggested to be the primary treatment in patients with MEC and AC.

Treatment outcomes of patients with tracheobronchial mucoepidermoid carcinoma compared with those with adenoid cystic carcinoma.

OBJECTIVE: Tracheobronchial mucoepidermoid carcinoma (TMEC) is an extremely rare salivary gland-type neoplasm. We aimed to explore the clinical characteristics and prognosis of TMEC and to compare them with those of another rare salivary gland-type neoplasm, tracheobronchial adenoid cystic carcinoma (TACC). METHOD: We performed a retrospective review of all patients pathologically diagnosed with TMEC between 1965 and 2017 at our institution. We reviewed the patients' clinical characteristics, treatment methods and outcomes and compared the results of TMEC and TACC patients. RESULTS: A total of 115 consecutive patients, including 107 who underwent surgery and 8 who received nonoperative therapy, were included in our study. The 1-, 2-, and 5-year survival rates were 97.89%, 94.17%, and 90.50%, respectively, in the surgical group and 83.33%, 41.67% and 0.00%, respectively, in the nonoperative group. The multivariate analysis showed that N stage was an independent prognostic factor for overall survival (OS). TMEC patients were younger, had a shorter complaint duration, had fewer symptoms, had more bronchial tumors, and were more likely to undergo surgical treatment and achieve an R0 resection (surgically treated patients) than TACC patients; furthermore, TMEC patients had a significantly better OS than TACC patients (P < 0.050). CONCLUSIONS: TMEC has different characteristics and a better prognosis than TACC, which may reflect the different biological behaviors of these two salivary gland neoplasms. Radical treatment and close follow-up are critical for surgically treated TMEC patients with lymph node metastasis.

A retrospective multicenter study of sublingual gland carcinoma in Japan.

OBJECTIVE: Salivary gland carcinoma is rare among head and neck cancers. Sublingual gland carcinoma, a type of salivary gland carcinoma, is even rarer; therefore, the number of cases at a single institute is too small for sufficient evaluation of tumor characteristics. We conducted a multicenter, retrospective analysis of sublingual gland carcinomas in patients who visited 12 institutions associated with the Kyoto Hospital and Affiliated Facilities Head and Neck Clinical Oncology Group. METHODS: Thirteen previously untreated patients who visited the institutions between 2006 and 2015 were enrolled. The overall survival (OS) and disease-free survival (DFS) rates for all patients and by disease stage were analyzed. Statistical analyses were performed for all patients with respect to disease stage. RESULTS: Eight of thirteen patients were diagnosed with adenoid cystic carcinoma on pathological study. A significant difference in OS rate was observed between patients with Stage I-III and Stage IV disease; however, the difference in DFS rate by disease stage was not significant. CONCLUSION: Stage IV disease was identified as a poor prognostic factor in patients with sublingual gland carcinoma. However, even patients with Stage I-III disease experienced relatively short DFS. Distant metastasis is a serious problem among patients with sublingual gland carcinoma.

Warthin-like mucoepidermoid carcinoma of the parotid gland: a diagnostic and therapeutic dilemma

Mucoepidermoid carcinoma (MEC) is the most common malignant salivary gland tumor. Although the parotid gland is the most common site of involvement, other major salivary glands and the minor salivary glands­most commonly of the palate­also can be involved. The management of mucoepidermoid carcinoma depends on the grade of the tumor and the adequacy of resection. We present the case of a 56-year-old female presenting a painless progressive cheek mass over 2 months. Imaging and fine-needle aspiration cytology provided the diagnosis of Mucoepidermoid carcinoma. A superficial parotidectomy was done, and the histopathology revealed a predominantly cystic tumor with a bilayered epithelium of oncocytic and basal cells. Moderate nuclear pleomorphism with infiltration of atypical squamous cells in few glandular cysts was seen. Special staining revealed the presence of intracellular mucin. A diagnosis of Warthin-like variant of MEC was made, based on these findings. After the surgical procedures, the patient is disease-free at 8 months of follow-up. The Warthin-like variant is a rare variant of MEC with fewer than 10 cases described in the English literature. Various differential diagnoses include the malignant transformation of Wartin tumor (WT), squamous metaplasia of WT, and metastasis from a distant primary. We emphasize the role of routine microscopy in identifying rare variants of common malignancies. Even though translocation studies are helpful in diagnosis, the typical histopathological findings should confirm it.

Intermediate-grade carcinoma of the parotid and the impact of adjuvant radiation.

PURPOSE: To determine the influence of adjuvant radiotherapy on survival in surgically-managed early stage intermediate-grade mucoepidermoid and acinic cell carcinoma of the parotid. MATERIALS AND METHODS: The National Cancer Database was reviewed between 2004 and 2015 to identify patients with intermediate-grade, early T-stage, node-negative parotid carcinoma who underwent parotidectomy ± radiotherapy. RESULTS: There were 744 patients identified of which 81% had mucoepidermoid carcinoma and 19% had acinic cell carcinoma. Positive surgical margins were identified in 21% and adjuvant radiotherapy was administered in 38% of cases. Of the 159 patients with positive margins, 113 (71%) received adjuvant radiotherapy. Of the 585 patients with negative margins, 173 (30%) underwent adjuvant radiotherapy. In multivariable analysis, age (over 52 years: HR 5.19, 95%CI 2.33-11.57), insurance status (private insurance: HR 0.24 95%CI 0.13-0.43), and extent of parotidectomy (total parotidectomy: HR 2.02 95%CI 1.23-3.31) were significantly associated with overall survival, while adjuvant radiotherapy was not a significant predictive factor (HR 0.81, 95%CI 0.49-1.36). In patients with positive margin resections, however, adjuvant radiation was an independent predictor of improved survival when adjusted for age, insurance status, and extent of parotidectomy (HR 0.34, 95%CI 0.13-0.88). Conversely, in patients with negative margin resections, adjuvant radiation did not influence survival outcomes when adjusted for these covariates (HR 1.02, 95%CI 0.53-1.93). CONCLUSIONS AND RELEVANCE: In patients with early stage intermediate-grade parotid carcinoma, adjuvant radiotherapy significantly and independently improves survival in those with post-operative positive margins. Adjuvant therapy, however, does not appear to improve survival outcomes in those with negative margin resections.

Primary mucoepidermoid carcinoma of the bulbar conjunctiva.

A 58-year-old male presented with redness for 1 year and dimness of vision for 2 years in his left eye. Excision biopsy from an inferotemporal conjunctival mass revealed high-grade mucoepidermoid carcinoma (MEC) with lymphovascular invasion and positive margins. The tumor bed was irradiated to 80 Gy using strontium 90 ß-applicator. After 13 months, fine-needle aspiration cytology from a suspicious preauricular lymphadenopathy found metastatic cells. Ipsilateral supra-omohyoid neck dissection showed three positive nodes out of 15, and the left neck was irradiated. He is disease free at present, 12 months from external beam radiation therapy completion. With 48 cases reported in the literature, conjunctival MEC remains a rare condition, possibly in part due to clinicopathological underdiagnosis. This is unfortunate, given that this cancer is more aggressive, has a poorer prognosis, and warrants more vigorous treatment than squamous cell carcinoma, which it may be misdiagnosed as in the absence of appropriate staining and pathological review.

Mucoepidermoid carcinoma of the head and neck in children.

INTRODUCTION: Mucoepidermoid carcinoma is a rare malignant salivary gland neoplasm in the pediatric population. Few studies have discussed best practice with respect to diagnosis and treatment in children. OBJECTIVE: To present our institution's experience with the evaluation and management of pediatric mucoepidermoid carcinoma of the head and neck. METHODS: Retrospective chart review of patients under 20 years of age diagnosed with mucoepidermoid carcinoma of the head and neck between 1998 and 2017. Data assessed includes demographics, clinical presentation, imaging examinations, histopathology, treatment, complications, local recurrence, distant metastasis, and follow-up. RESULTS: Sixteen patients (10 female, 6 male) were identified with a median age of 12.9 (IQR: 10.9-15.0) years. Tumors were located within the parotid gland (n = 11, 68.8%), accessory lobe of the parotid gland (n = 2, 12.5%), palate (n = 2, 12.5%), and submandibular region (n = 1, 6.3%). In 9 patients (56.3%) a neoplastic etiology was suspected based on the clinical and/or radiographic findings and confirmed pathologically on biopsy or excision. All patients were treated surgically and five patients required adjuvant radiotherapy. One patient had recurrence requiring re-excision. Seven patients (43.8%) had transient facial paresis post-operatively and one had Frey syndrome. Median follow-up time was 59.7 months (IQR: 18.9-99.3). CONCLUSION: The malignant nature of mucoepidermoid carcinoma requires comprehensive, multidisciplinary management. Imaging and tissue sampling by fine needle aspiration give clinicians the best insight into location and nature of the mass. Complete surgical excision with attention to preservation of facial nerve and achieving negative margins is desired.

Rare cause of lung atelectasis in a young woman.

Pulmonary mucoepidermoid carcinoma is an extremely rare intrathoracic malignancy, comprising less than 1% of all lung tumours. These are very slow growing and are classified into low grade and high grade based on histological features. Surgical resection is the primary treatment with excellent outcomes, while chemotherapy or radiotherapy effectiveness is not known. Preoperative fluorine-18 fluorodeoxyglucose positron emission tomography/CT (18F-FDG PET/CT) is useful for predicting tumour grade and postsurgical prognosis.A clinical case of a 31-year-old woman who presented with dyspnoea on exertion, cough and wheezing is reported. Imaging studies revealed a mass involving the left lower lobe bronchus and atelectasis. 18F-FDG PET/CT showed uptake in the described mass with a maximum standardised uptake value of 9.7. Complete surgical resection was performed, and pathological examination revealed a high-grade mucoepidermoid carcinoma with tumour-free margins. Adjuvant chemotherapy was given and there is no evidence of tumour recurrence.

Salivary gland tumors: Molecular characterization and therapeutic advances for metastatic disease.

Salivary gland cancers represent a rare group of tumors composed by over 20 histological subtypes. Initially treated as one single disease, its diagnosis, prognosis, and treatment are currently being stratified based on morphology. More recently, insight has been provided on the molecular characterization of each subtype, further improving diagnostic accuracy and paving the way for personalized therapy. In this article, we provide a comprehensive review of recent breakthroughs, preliminary results of novel therapy, and future directions on the treatment of these complex malignancies.

The number and ratio of positive lymph nodes are independent prognostic factors for patients with major salivary gland cancer: Results from the surveillance, epidemiology, and End Results dataset.

INTRODUCTION: To investigate whether the positive lymph node number (PLNN) and positive lymph node ratio (PLNR) could predict the prognosis of patients with major salivary gland cancer (MSGC) and to identify the optimal cutoff points for these variables that stratify patients according to their risk of survival. METHODS: We used the Surveillance, Epidemiology, and End Results (SEER) database to identify all patients with MSGC between 1988 and 2014. A logistic regression analysis was carried out to evaluate the risk factors for lymph node metastasis (LNM) in MSGC. The X-tile program was used to identify the cutoff values for the PLNN and PLNR in MSGC patients with LNM. Cox proportional hazards regression models were performed to identify the predictors of cancer-specific survival (CSS). RESULTS: In the SEER database, 8668 eligible patients were identified and 3046 of them had LNM. The logistic regression analysis indicated that older age, male sex, larger tumor size, higher grade, tumor extension and high-risk pathology were associated with LNM. The X-tile program showed that a PLNN>4 and a PLNR>0.15 were prognostic indicators of CSS. A multivariable analysis indicated that, after the factors that might potentially affect the prognosis were adjusted for, the PLNN and PLNR were still associated with CSS. CONCLUSIONS: Our Results demonstrated that the PLNN and PLNR were independent prognostic indicators for MSGC patients with lymph node metastasis.

[Clinical analysis of malignant salivary gland tumors of the larynx].

Objective: To evaluate the clinical features, treatments and prognoses of malignant salivary gland tumors of the larynx. Methods: A total of 14 patients with malignant salivary gland tumors of the larynx diagnosed and treated in our hospital between 2003 and 2014 were analyzed retrospectively, and were compared with 62 patients from the Surveillance, Epidemiology, and End Results: (SEER) database of American for survival rates. Results Among 14 patients from our hospital, 9 males and 5 females, with a median of 66.5 years old; 8 patients with adenoid cystic carcinoma, 4 with mucoepidermoid carcinoma and 2 with adenocarcinoma; supraglottic, glottic and 4 subglottic malignancies respectively accounting for 5, 5 and 4 cases; 8 cases treated with surgery, 5 with surgery and postoperative radiotherapy and/or chemotherapy, and 1 with radiotherapy alone. The 14 patients showed a three-year overall survival rate of 50.0% and a five-year overall survival rate of 42.9%, respectively in contrast with the rates of 67.1% and 59.8% in SEER database. Conclusions: The malignant salivary gland tumor of the larynx is rare and it is difficult to be diagnosed early. It is more likely to occour local recurrence and distant metastasis. Extensive surgical resection with postoperative radiotherapy is advocated.