Malignant Salivary Gland Tumors Research Articles

Secretory carcinoma of minor salivary glands.

Secretory carcinoma is a malignant salivary gland tumor, which typically presents as an indolent painless mass within the parotid gland. Involvement of minor gland is reported but less common. Secretory carcinoma was often misclassified as other salivary gland mimics and in particular, acinic cell carcinoma prior to 2010. It was first recognized by Skalova et al. as a molecularly distinct salivary gland tumor harboring the same fusion gene as well as histological and cytogenetic features seen in juvenile breast cancer. Secretory carcinoma is generally managed as other low-grade salivary gland neoplasms and has a favorable prognosis; however, high-grade transformation requiring aggressive therapeutic interventions have been documented. Recent studies of biological agents targeting products of this fusion gene offer the promise of a novel therapeutic option for treatment of this malignancy Due to limited number of reported cases, the spectrum of clinical behavior, best practices for management and long-term treatment outcomes for secretory carcinoma remain unclear. We report a long-standing secretory carcinoma involving minor salivary glands of the mucobuccal fold, which was detected years after it was first noted by the patient. This case brings to light the importance of a thorough clinical exam during dental visits and reviews diagnostic differentiation of this malignancy from other mimics and discusses decision making for its management.

MRI-based radiomics for predicting histology in malignant salivary gland tumors: methodology and "proof of principle".

Defining the exact histological features of salivary gland malignancies before treatment remains an unsolved problem that compromises the ability to tailor further therapeutic steps individually. Radiomics, a new methodology to extract quantitative information from medical images, could contribute to characterizing the individual cancer phenotype already before treatment in a fast and non-invasive way. Consequently, the standardization and implementation of radiomic analysis in the clinical routine work to predict histology of salivary gland cancer (SGC) could also provide improvements in clinical decision-making. In this study, we aimed to investigate the potential of radiomic features as imaging biomarker to distinguish between high grade and low-grade salivary gland malignancies. We have also investigated the effect of image and feature level harmonization on the performance of radiomic models. For this study, our dual center cohort consisted of 126 patients, with histologically proven SGC, who underwent curative-intent treatment in two tertiary oncology centers. We extracted and analyzed the radiomics features of 120 pre-therapeutic MRI images with gadolinium (T1 sequences), and correlated those with the definitive post-operative histology. In our study the best radiomic model achieved average AUC of 0.66 and balanced accuracy of 0.63. According to the results, there is significant difference between the performance of models based on MRI intensity normalized images + harmonized features and other models (p value < 0.05) which indicates that in case of dealing with heterogeneous dataset, applying the harmonization methods is beneficial. Among radiomic features minimum intensity from first order, and gray level-variance from texture category were frequently selected during multivariate analysis which indicate the potential of these features as being used as imaging biomarker. The present bicentric study presents for the first time the feasibility of implementing MR-based, handcrafted radiomics, based on T1 contrast-enhanced sequences and the ComBat harmonization method in an effort to predict the formal grading of salivary gland carcinoma with satisfactory performance.

Molecular Aspects of Mucoepidermoid Carcinoma and Adenoid Cystic Carcinoma of the Salivary Gland.

Salivary gland tumors (SGTs) are rare and highly heterogeneous lesions, making diagnosis a challenging activity. In addition, the small number of studies and samples evaluated difficults the determination of prognosis and diagnosis. Despite the solid advances achieved by research, there is still an intense need to investigate biomarkers for diagnosis, prognosis and that explain the evolution and progression of SGTs. We performed a comprehensive literature review of the molecular alterations focusing on the most frequent malignant SGTs: mucoepidermoid carcinoma and adenoid cystic carcinoma. Due to the importance of biomarkers in the tumorigenenic process, this review aimed to address the mechanisms involved and to describe molecular and biomarker pathways to better understand some aspects of the pathophysiology of salivary gland tumorigenesis. Molecular analysis is essential not only to improve the diagnosis and prognosis of the tumors but also to identify novel driver pathways in the precision medicine scenario.

Oncologic outcomes of the most prevalent major salivary gland cancers: retrospective cohort study from single center.

The preoperative diagnosis of salivary gland cancer (SGC) is crucial for the application of appropriate treatment, particularly involving the extension of the resection. Retrospective search of medical database identified 116 patients treated surgically with malignant tumors of salivary gland between 2010 and 2020. Analysis included the demographical data, clinical course, type of surgical and adjuvant treatment, histology type and margin status, perivascular invasion (LVI), perineural invasion (PNI), metastatic lymph nodes (LN). Facial nerve function, recurrence-free and overall survival were evaluated. Adequate statistics were used for data analysis. The final cohort included 63 SGC patients, with adenoid cystic carcinoma the most common pathological type (27%, n = 17), followed by adenocarcinoma (17.4% n = 11). T1 and T2 patients accounted for majority cases (n = 46). The lymph node metastases were confirmed with the histopathology in 31.7% (n = 20). Distant metastases were observed in 4.8% of cases (n = 3). 38% (n = 24) of SGC were treated selectively with surgery, 49.2% (n = 31) had postoperative radiotherapy and 15.9% (n = 10)-radio-chemotherapy. The final facial nerve function was impaired in 38% of patients. Mean overall survival (OS) for all patients was 108.7 (± 132.1) months, and was the most favorable for acinar cell carcinoma (118.9 ± 45.4) and the poorest for squamous cell carcinoma (44 ± 32). Cox regression analysis of disease-free survival and OS identified significant association only with patients' age over 65years, the hazard ratio of 7.955 and 6.486, respectively. The efficacy of treatment modalities for SGC should be verified with regard to the histopathological type, but also the patients' age should be taken into account.

Differentiation of salivary gland tumors using diffusion-weighted image-based virtual MR elastography: A pilot study.

Differentiation among benign salivary gland tumors, Warthin tumors, and malignant salivary gland tumors is crucial to treatment planning and predicting patient prognosis. However, differentiation of those tumors using imaging findings remains difficult. This study evaluated the usefulness of elasticity determined from Diffusion-weighted image (DWI)-based virtual MR elastography (MRE) compared with conventional MRI findings in differentiating the tumors. This study included 17 benign salivary gland tumors, six Warthin tumors, and 11 malignant salivary gland tumors scanned on neck MRI. The long and short diameters, T1 and T2 signal intensities, tumor margins, ADC values, and elasticity from DWI-based virtual MRE of the tumors were evaluated. The interobserver agreement in measuring tumor elasticity and the ROC curves were also assessed. The long and short diameters and the T1 and T2 signal intensities showed no significant difference among the three tumor groups. Tumor margins and the mean ADC values showed significant differences among some tumor groups. The elasticity from virtual MRE showed significant differences among all three tumor groups and the interobserver agreement was excellent. The area under the ROC curves of the elasticity were higher than those of tumor margins and mean ADC values. Elasticity values based on DWI-based virtual MRE of benign salivary gland tumors, Warthin tumors, and malignant salivary gland tumors were significantly different. The elasticity of Warthin tumors was the highest and that of benign tumors was the lowest. The elasticity from DWI-based virtual MRE may aid in the differential diagnosis of salivary gland tumors.

The tumor microenvironment of benign and malignant salivary gland tumors.

Treatment of salivary gland tumors (SGTs) remains challenging. Little is known about the immune landscape of SGTs. We aimed to characterize the tumor microenvironment in benign and malignant SGTs. Eleven benign and nine malignant tumors were collected from patients undergoing curative intent surgery. Specimens were analyzed using mass cytometry by time-of-flight. Immune cell populations were manually gated, and T cells were clustered using the FlowSOM algorithm. Population frequencies were compared between high-grade and low-grade malignancies, corrected for multiple hypothesis testing. There were trends towards increased CD4+ and CD8+ T cells among malignant tumors. High-grade malignancies exhibited trends towards higher frequencies of CD8+ PD-1+ CD39+ CD103+ exhausted T cells, CD4+ FoxP3+ TCF-1+ CD127- Tregs, and CD69+ CD25- CD4+ T cells compared to low-grade malignancies. SGTs exhibit significant immunologic diversity. High-grade malignancies tended to have greater infiltration of exhausted CD8+ T cells and Tregs, which may guide future studies for immunotherapy strategies.

Pitfalls in Salivary Gland Cytology.

Salivary gland lesions possess diagnostic challenges on fine needle aspiration (FNA) material. They are relatively uncommon, yet present with a wide spectrum of cytomorphology. Herein, we review common salivary gland neoplasms, their cytomorphologic features, their diagnostic pitfalls, and ancillary studies helpful in achieving an accurate diagnosis. There are many cytomorphologic overlaps between benign and malignant salivary gland entities. Moreover, metaplasia, cystic changes, and degenerative changes are common findings adding to diagnostic dilemmas. These complicating factors contribute to a minute risk of malignancy in salivary gland lesions that are interpreted as benign on FNA. In rare cases, even malignant salivary gland neoplasms are misinterpreted as benign on aspirated material due to the many cytomorphologic overlaps. For example, benign and malignant neoplasms containing stroma such as myoepithelioma and adenoid cystic carcinoma may be misinterpreted as pleomorphic adenoma. Moreover, diagnosis of salivary gland neoplasms with basal cell features can be confusing on FNA materials; for example, basal cell adenoma can be misinterpreted as adenoid cystic carcinoma. Mucoepidermoid carcinomas have many different appearances on aspirated material due to variable amounts of mucin, degree of nuclear atypia, cellular content, and squamous metaplasia. Acinic cell carcinoma exhibits large cells with abundant cytoplasm on FNA, which can be mistaken for oncocytic cells in oncocytoma or Warthin tumor. Salivary duct carcinoma shows distinct features of malignancy and thus can be mistaken for secondary tumors involving the salivary glands or other malignant salivary gland tumors. The presence of tumor-associated lymphocytes is another underlying cause of misdiagnosis, especially when considering the differential diagnosis of an an intraparotid lymph node. Ancillary studies such as immunohistochemistry and molecular studies are gaining more attention to be utilized on FNA cases. PLAG1 immunostaining, CD117 , DOG1, mammaglobin, and androgen receptor (AR) are examples of commonly used immunostains in diagnosis of salivary gland lesions. MYB gene fusion , rearrangements of the MAML2 gene, ,and ERBB2/HER2 are examples of molecular alterations useful in diagnosis of salivary gland neoplasms. In conclusion, the aim of salivary gland cytology is to differentiate benign entities from the malignant ones and to prevent unnecessary aggressive treatments.

Radical Resection of Malignant Tumors of Major Salivary Glands: Is This Possible?

In primary therapy, a universally recognized surgical indication applies to all tumors of the salivary glands. According to the classic rule, radical resection of a head and neck tumor requires clean margins of at least 5 mm, although recent studies have shown that for certain locations, 1 mm may be sufficient. In the surgical resection of a tumor of the salivary glands, especially of the parotid gland, can these rules be respected? Owing to the complex branching and connections of the facial nerve within the parotid gland, even a medium-sized malignant tumor may be in contact with a branch of the nerve, thus raising the question of its preservation. The facial nerve is so important from a functional and aesthetic point of view that it is commonly believed that it should be preserved unless it is incorporated into the tumor. This is a compromise between an oncological resection, that is, the complete excision of the tumor with no residual cancer cells left behind, and quality of life. Almost all authors try to overcome this lack of radicality by indicating postoperative (chemo)radiotherapy. In this article, the pros and cons of nerve preservation will be analyzed by examining the published studies on this topic.

First Case Report of Bilateral Parotid Lymphoepithelial Carcinoma.

The parotid is the largest salivary gland and is located anteriorly to the sternocleidomastoid muscle and laterally to the ramus of the mandible. Neoplasms in this gland are relatively rare, with 80% being benign and 20% malignant, primarily represented by mucoepidermoid carcinoma. In the head and neck region, lymphoepithelial carcinoma (LEC) accounts for 0.4% of malignant salivary gland tumors. A 35-year-old man with no previous comorbidities was admitted to a Head and Neck Surgery Specialty Service for a painless right cervical mass of uncertain growth. Extensive diagnostic investigation revealed involvement of the contralateral parotid, associated with systemic lymph node enlargement. Thus, adjuvant radiotherapy was decided by the treating team. This case confirms the heterogeneous features and distinctive behavior that the disease can present, as seen with bilateral parotid LEC.

A Clinicopathological Study of Salivary Gland Lesions and Immunohistochemical Expression of Bcl2 in Benign and Malignant Salivary Gland Neoplasms

Introduction Salivary glands neoplasms (SGNs) are rare tumors and comprise approximately 3-10% of the neoplasms of the head and neck region and 1% of the neoplasms of the whole body(1) . Annual global incidence of SGNs is between 0.4 to 13.5 cases per 1, 00,000 individuals(2) . Among these, malignant salivary gland neoplasms (MSGNs) are 0.40 to 0.65 per 1, 00,000 cases(3) . MSGNs are 0.3% of all malignancies(4) . Early diagnosis of MSGNs play an important role in better management of these tumors(5) . Fine-needle aspiration cytology (FNAC) is used as a screening and early diagnostic tool in SGNs however its role is limited(6) . Accurate diagnosis depends upon the histological evaluation of SGNs

The immunohistochemical expression Ki-67 In Polymorphous Low Grade Adenocarcinoma of the Salivary Glands

Background: Polymorphous Low Grade Adenocarcinoma is a rare malignant tumor of minor salivary glands. It has an unique histopathological, clinical and behavioural features. Most of its incidence has been presented in the palate. It shows a variety of growth patterns within the same one lesion, including cribriform, tubular, and solid areas that may mimic adenoid cystic carcinoma. The tumor cells histologically are characterized by lacking of nuclear atypical and hyperchromatism with rare mitoses. However, the prognosis of this tumor is good because of its low grade, although it has an unpredictable potential to metastasize.&#x0D; Objectives: To evaluate the histopathological expression of Ki-67 proliferation antigen in Polymorphous low grade adenocarcinoma and compare the results with the clinicopathological parameters and grading of the tumors.&#x0D; Materials and Methods: This study includes 20 cases with Polymorphous Low Grade Adenocarcinoma of minor salivary glands diagnosed histopathologically and treated surgically, were investigated for the immunohistochemical expression of Ki-67 antigen. Immunohistochemical staining was performed using a Labeled Strept- Avidin Biotin method (LSAB) and 0.05 (two-sided) was the level of significance.&#x0D; Results: Twenty cases of Polymorphous Low Grade Adenocarcinoma of salivary glands were histologically diagnosed in this retrospective study to evaluate the expression of Ki-67. The mean age for patients with PLGA was nearly (50.6). (12) (60%) cases were males and (8) (40%) cases were females and also male to female ratio was (1.5:1).&#x0D; In regarding to site distribution, the most common site was the palate followed by the submandibular gland and floor of the mouth. &#x0D; Conclusions: it was observed that there is a higher rate of cellular proliferation in malignant tumors with high grade, indicating their role in the malignancy and aggressive behavior of these tumors.

Wnt/β-catenin-C-kit axis may play a role in adenoid cystic carcinoma prognostication

Adenoid cystic carcinoma (ACC) is one of the most common malignant salivary gland tumors. ACC is composed of myoepithelial and epithelial neoplastic cells which grow slowly and have a tendency for neural invasion. The long term prognosis is still relatively poor. Although several gene abnormalities, such as fusions involving MYB or MYBL1 oncogenes and the transcription factor gene NFIB, and overexpression of KIT have been reported in ACC, their precise functions in the pathogenesis of ACC remain unclear. We recently demonstrated that the elevated expression of Semaphorin 3A (SEMA3A), specifically expressed in myoepithelial neoplastic cells, might function as a novel oncogene-related molecule to enhance cell proliferation through activated AKT signaling in 9/10 (90%) ACC cases. In the current study, the patient with ACC whose tumor was negative for SEMA3A in the previous study, revisited our hospital with late metastasis of ACC to the cervical lymph node eight years after surgical resection of the primary tumor. We characterized this recurrent ACC, and compared it with the primary ACC using immunohistochemical methods. In the recurrent ACC, the duct lining epithelial cells, not myoepithelial neoplastic cells, showed an elevated Ki-67 index and increased cell membrane expression of C-kit, along with the expression of phosphorylated ERK. Late metastasis ACC specimens were not positive for β-catenin and lymphocyte enhancer binding factor 1 (LEF1), which were detected in the nuclei of perineural infiltrating cells in primary ACC cells. In addition, experiments with the GSK-3 inhibitor revealed that β-catenin pathway suppressed not only KIT expression but also proliferation of ACC cells. Moreover, stem cell factor (SCF; also known as KIT ligand, KITL) induced ERK activation in ACC cells. These results suggest that inactivation of Wnt/β-catenin signaling may promote C-kit-ERK signaling and cell proliferation of in metastatic ACC.

Polymorphous Adenocarcinoma: A Systematic Review of the Literature and Presentation of Two Cases in a Less-Considered Anatomical Site.

Polymorphous adenocarcinoma (PAC) is the second-most common malignant tumour of the minor salivary glands. Although PAC predominantly affects the palate, it can also involve the buccal mucosa. This systematic review aims to investigate the literature data about PAC. Furthermore, we report two cases of patients affected by PAC in an infrequently considered anatomical site. According to PRISMA guidelines, a systematic review search was conducted on PubMed, Scopus, and Web of Science. Observational studies conducted on patients with a histological diagnosis of PAC were selected and analysed. Furthermore, two cases of patients with PAC affecting the buccal mucosa were reported. Twenty-nine studies were included, and 143 patients affected by PAC were analysed (62 males, 75 females, and 6 undefined, with a mean age of 57.4 ± 14.5 years). The palate was the most affected site (99/143, 69.2%), followed by the buccal mucosa (12/143, 8.4%). Moreover, we report two cases of patients with PAC affecting the buccal mucosa (one male and one female, with a mean age of 70.5 ± 2.5 years). The present study underscores the importance of considering the buccal mucosa as a possible location of minor salivary gland tumours; although it is a less-considered affliction, it is not uncommon.

Malignant salivary gland tumors: agreement between fine needle aspiration biopsy, incisional biopsy and final histopathological diagnostic.

Incisional biopsy is indicated for intraoral tumors, but it is a contraindicated surgical procedure for major salivary glands. To avoid complications and facilitate diagnosis, fine needle aspiration biopsy (FNAB) is a type of biopsy widely used for preoperative diagnosis in these glands. The aim of this study was to analyze the agreement between the diagnosis by FNAB (major glands), incisional biopsy (minor glands) and histopathological analysis of the surgical specimen in salivary gland tumors from a database (medical records) of patients treated in a cancer treatment reference center in the Northeast region of Brazil. The sample consisted of 110 cases, being 86 of them malignant tumors in major salivary glands (parotid gland=73; submandibular gland=13) and 24 cases in minor salivary glands (palate). The female gender was the most affected (57.3%), especially in patients over 60 years (42.7%). In the TNM classification, 41.8% of the cases were in T2 at the time of diagnosis, with most of the regional lymph nodes in N0 (85.5%) and 87.3% of the cases in M0. FNAB was able to identify malignant neoplasms in 68.6% of the cases (n=59), while incisional biopsy in palatal tumors obtained agreement of 75% of the cases (n=18). The analysis revealed that tumors classified as T3-T4 (p=0.012) showed greater agreement between pre- and post-surgical diagnosis. The results obtained in this study indicate that FNAB has similar accuracy to incisional biopsy in providing satisfactory preoperative diagnoses of malignant tumors in salivary glands.

Impaired intratumoral dendritic cell function and potential predictive value of dendritic cell markers for metastasis in malignant salivary gland tumors.

The differentiation between primary and metastatic salivary gland neoplasms (SGNs) helps in determining appropriate management strategies, including the need for additional diagnostic tests, surveillance, or aggressive treatment. The purpose of this study was to identify and quantify the immature and mature dendritic cells (DCs) in metastatic and no metastatic SGNs and determine its association with clinicopathological findings. Cross-sectional, observational, and descriptive study that includes 33 malignant salivary gland neoplasms [MSGN (6, 18.1% metastatic)], and 22 pleomorphic adenomas (PA), as a control group. Clinical and histopathological characteristics were obtained. Immunohistochemistry for human leukocyte antigen D-related (HLA-DR), CD1a, CD83, and Ki-67 proteins was done. Positive intra- and peritumoral DCs were counted. Individuals with MSGN had a lower density of intratumoral HLA-DR+ cells than those with PA (p=0.001), Ki-67 immunostaining was significantly higher in MSGN than in PA (6% vs. 1.4%, p<0.001). Metastatic MSGN showed less intratumoral CD1a+ than non-metastatic (3.2 vs. 165.1, p=0.001). No differences in intra- and peritumoral CD83+ cells were found between benign and malignant SGN. These results suggest that the immune-protective function of intratumoral DCs is compromised in MSGNs. DCs markers may represent useful prediction tools for metastases in salivary gland malignancies, with crucial implications in the implementation of appropriate disease management strategies.

Evaluation of cell proliferation marker CDC-7 in salivary adenoid cystic carcinoma

BackgroundAdenoid cystic carcinoma (AdCC) is considered one of the most common destructive types of malignant salivary gland tumor that have high affinity to perineural invasion (PNI). This study was conducted to access different histological features of AdCC, and assessment of the immunohistochemical expression of CDC-7. MethodsThirty formalin-fixed paraffin incorporated tissue blocks of AdCC were classified according to the WHO histopathological types. The immune-expression of CDC-7 positive area was evaluated according to percentage area as following: Negative = 0 %, Weak = 1–10 %, Moderate = 11–49 %, and Strong = 50–100. The correlations between expression of the marker and different clinico-pathological variables were investigated using Chi-square (χ2) test. The P‐value ≤ 0.05 was considered statistically significant. ResultsThe expression of CDC-7 revealed statistical significant difference between the different tumor types (p ≤ 0.05). ConclusionThe biological behavior of AdCC can be predicated from the expression of CDC-7.

Epithelial malignant tumors of the parotid salivary glands in children and adolescents

The article provides data on epithelial malignant tumors of the parotid salivary glands in children and adolescents. We present a historical background and modern classification proposed by the World Health Organization in 2022. We present the evolution of surgical and non-surgical treatment methods used in pediatric oncology.

Impact of Sarcopenia on Survival of Patients With Malignant Salivary Glands Tumors.

Malignant salivary glands tumors (MSGTs) are a quite rare and heterogeneous group of tumors. Management of these lesions remains controversial and challenging. Thus, finding new prognostic factors that can help to guide the decision-making process, appears to be paramount. The aim of this study was to evaluate the prognostic performance of preoperative sarcopenia to stratify MSGTs patients at high risk of disease progression. Retrospective study. A single-institution analysis (Maxillo-facial Surgery Unit, University of Naples Federico II). The study consists of a retrospective analysis of 74 patients surgically treated for MSGTs. For all patients, the skeletal muscle index (SMI) was calculated and sarcopenia was defined as SMI < 41 in females and <43 in males. The correlation between sarcopenia and tumor variables was analyzed. The prognostic performance of sarcopenia was evaluated through survival Kaplan-Meier curves. Sarcopenia resulted statistically related to age (P < .001), tumor size (P < .001), lymph node metastases (P < .001), and American Joint Committee on Cancer tumor, node, metastasis stage (P < .001). Kaplan-Meier survival curves show that 47.3% of sarcopenic patients died before their final follow-up. Data obtained from our study seem to confirm the correlation between sarcopenia and other high-risk features. The early detection of sarcopenia in patients with negative prognostic factors could be used to implement the support therapeutic strategies aimed at restore the clinical conditions of the patients. Sarcopenia may be routinely investigated before surgery to suggest the implementation of precautionary therapeutic strategies to improve the standard treatment response, reducing possible complications.

Epidemiological Profile of Salivary Gland Tumors in Southern Iranian Population: A Retrospective Study of 405 Cases.

Salivary gland tumors (SGTs) are a rare and diverse group of tumors that account for 3 to 10% of all head and neck malignancies. We aimed to conduct a comprehensive epidemiological analysis of SGTs in the south of Iran and compare the findings with previous reports from Iran and other parts of the world. Using a retrospective study, 405 patients diagnosed with SGTs were observed over an eight-year period between April 2013 and October 2021 in Shiraz, Iran. Patients' demographic and clinicopathological features were obtained from patients' records. Quantitative and descriptive data analysis was performed using SPSS software. There were 302 benign (74.5%) and 103 (25.4%) malignant SGTs. Pleomorphic adenoma and Warthin's tumors were the most common benign SGTs (70.5% and 21.5%, respectively). The most common malignant SGTs were mucoepidermoid carcinoma and adenoid cystic carcinoma (26.2% and 22.3%, respectively). There was a statistically significant association between tumor origin and its malignancy status (p < 0.001). In addition, the results indicated that benign tumors were most commonly detected in the parotid gland (p < 0.05). The benign tumors were more frequently observed among the younger population (p = 0.006). In summary, the findings of the current study were mainly consistent with the previous reports from Iran and the rest of the world. Benign tumors were the most prevalent type of SGTs, and the parotid gland was the most common site. While the majority of cases that developed from the major salivary glands were benign, all the minor SGTs were malignant. Older patients were more likely to develop malignant tumors compared to younger ones. This study provides insights into the prevalence, age-related incidence, gender distribution, and geographic variation of salivary gland tumors. This can be instrumental to develop a guideline for screening, diagnosis, and determining an optimal treatment.

Salivary Gland Secretory Carcinoma; Review of 13 Years World-Wide Experience and Meta-Analysis.

Secretory Carcinoma is a malignant salivary gland tumor, initially described in 2010. This rare tumor is associated with the translocation t(12;15) (p13;q25), resulting in the fusion gene ETV6-NTRK3. Since this tumor is quite rare, most publications describe only small cohorts of patients. We aimed to investigate the clinical, pathological, and prognostic features of this newly defined malignant entity. Pubmed, Google Scholar, and Web of Science databases. All published articles between 2010 and 2023 were reviewed. Search terms included the terms "Mammary Analogue Secretory Carcinoma" and "Secretory Carcinoma". All articles published in English reporting on Secretory Carcinoma of salivary glands were retrieved. One-hundred and 12 retrospective articles reporting a total of 674 patients were included, with 52% males and a mean age of 44.9 ± 18.9. The event rate for patients with advanced-stage disease (Stage 3/4) at presentation was 24.1% (95% CI 17.6%-31.9%, I2  = 9.2%), 14.6% for regional metastases (95% CI 10.5%-20%, I2  = 12%), and the event rate of distant metastasis was 8.4% (95% CI 5.5%-12.7%, I2  = 4.2%). Adjuvant radiotherapy was administered for 30.3% of patients (95% CI 24.1%-37.2%, I2  = 21.5%). The recurrence rate was 19% (95% CI 15.1%-23.8%, I2  = 5%). Survival outcomes showed a 17.2% death of disease rate for Secretory Carcinoma patients (95% CI 13.5%-21.8%, I2  = 7.3%). Secretory Carcinoma is a rare and relatively newly defined entity arising in the parotid gland most commonly. Characterized as a low-grade tumor, the majority of patients are diagnosed at an early stage, without regional or distant disease, and the prognosis is relatively good. NA Laryngoscope, 134:1716-1724, 2024.