Immunohistochemical expression of the cation channel TRPC6 in the submandibular and lacrimal gland and in salivary gland tumors.

BACKGROUND: Canonical transient receptor potential channels play a crucial role in cancer cell proliferation. While TRPC6 subtype detection in submandibular glands and the relevance of some TRPC channels in this gland have been shown in animal models, its histological detection in human lacrimal and submandibular glands, as well as related tumors, lacks systematic study. Studying TRPC6 in humans could lead to new therapeutic options. This research aimed to immunohistochemically detect TRPC6 in human samples of physiological lacrimal and submandibular glands and of adenoid cystic carcinoma and mucoepidermoid carcinoma. METHODS: Seven fixed body donors and samples of six cancer patients were examined. The ten tissue samples collected from the submandibular and lacrimal glands were then processed into histological slides and stained with hematoxylin-eosin. Tumor samples were provided as sections. TRPC6 presence was determined by immunohistochemistry, which was performed by indirect detection with a primary TRPC6 antibody, a secondary HRP-conjugated antibody and the chromogen diaminobenzidine. RESULTS: Results confirm TRPC6 expression in all ten physiological gland samples: all samples showed a immunohistochemical signal with varying intensity. No significant gender-specific differences could be observed. TRPC6 was detected in four of six submandibular adenoid cystic carcinoma and the mucoepidermoid carcinoma samples, especially in tumor cells' cytoplasma and nuclei. Excretory ducts consistently showed TRPC6. Mucous tubules, their nuclei and the nuclei of adipocytes generally showed no signal while serous acini and their nuclei showed a weak TRPC6 signal. CONCLUSION: The discovery of TRPC6 in glandular tissue indicates a role in salivary gland function and calcium homeostasis is a basis for further research into its significance for tumor development in adenoid cystic carcinoma and mucoepidermoid carcinoma of salivary glands. TRPC6 could be used as a target for treatment of these tumors. However, the correlation between TRPC6 and submandibular and lacrimal gland diseases requires further exploration.

Recurrent lacrimal gland adenoid cystic carcinoma: A case report and literature review.

Introduction: Lacrimal gland adenoid cystic carcinoma (LGACC) is characterized by a high rate of recurrence, perineural invasion, and propensity for distant metastasis, resulting in poor prognosis. This case report aimed to highlight the diagnostic and therapeutic challenges of LGACC, underscore the importance of resectioning the tumor as completely as possible for the first time, adhere to postoperative adjuvant therapy, and provide detailed insights into its surgical and diagnostic management that may not be extensively covered in large case series and meta-analyses. Case presentation: A 34-year-old man presented with progressive left eye proptosis for 4 months. Initial evaluation and imaging led to a high suspicion of LGACC, which was confirmed after an eye-sparing excision of the left orbital tumor. The patient declined to undergo postoperative radiotherapy, which was recommended after the surgery. Thus, despite surgical intervention, the patient experienced tumor recurrence 3 months post-surgery, leading to orbital exenteration. Pathological examination confirmed the presence of poorly differentiated LGACC.This time the patient underwent postoperative radiotherapy, as recommended. However, despite local control, the patient developed an intracranial metastasis within a year. Conclusion: LGACC presents significant diagnostic and therapeutic challenges owing to its insidious onset, lack of specific symptoms, and high potential for recurrence and metastasis. Thus, this case emphasizes the need for early diagnosis, aggressive treatment, and adherence to postoperative adjuvant therapy to improve patient outcomes. Future research should focus on understanding the pathogenesis of LGACC and on developing standardized diagnostic and treatment protocols to enhance patient prognosis and survival.

The role of histology on the outcome of sinonasal carcinomas treated with radiotherapy: a single institution experience.

PURPOSE: Sinonasal malignancies are a rare group of head and neck cancers. We aimed to report the oncological outcomes based on histological types in patients who underwent radiotherapy. MATERIALS AND METHODS: In this single-institution study, we retrospectively retrieved and analyzed data of patients with sinonasal carcinomas who underwent radiotherapy during 2011-2016 as part of their treatment. The 3-year rate of local, regional, and distant recurrences, and overall survival were evaluated according to the histological type. RESULTS: A total of 28 patients were evaluated in this study, the majority of whom were male (60%). Squamous cell carcinoma (SCC), adenoid cystic carcinoma (ACC), and adenocarcinoma (ADC) were found in 15 patients (53.5%), 8 (28.5%), and 5 (18%), respectively. The highest rates of local and regional recurrences were observed in ACC and SCC, respectively. Distant recurrences were numerically more common in ADC. The 3-year OS was 48%, 50%, and 73% in SCC, ADC, and ACC, respectively. CONCLUSION: Different histopathologies of sinonasal cancer seem to have different patterns of failure, and this may be considered in the treatment approach.

Clinical Outcome in Patients with Large Sinonasal Tumors with Intracranial Extension.

Objectives Malignant tumors of the sinonasal cavities with extension to the frontal skull base are rare and challenging pathologies. Combined-approach surgery using a frontobasal craniotomy and endoscopic sinus surgery with reconstruction of the anterior skull base followed by adjuvant radiotherapy is a preferred treatment strategy in selected cases. Morbidity and mortality rates are high in this population. We aim to add our experience to the current literature. Design We performed a retrospective cross-sectional single center study of the long-term clinical outcome in a tertiary university referral hospital in the Netherlands between 2010 and 2021. Descriptive statistics and frequency distributions were performed Participants Patient, tumor, treatment, complications and survival characteristics of eighteen consecutive patients were extracted from the electronic health records. Main Outcome Measures The primary outcome measures are progression free survival, overall survival and complication rate. Results Eighteen consecutive patients were included with a mean age of 61 (SD ± 10) years (range 38-80); ten males and eight females. Gross total resection was achieved in 14 (77%) patients. Eleven (61%) patients underwent local radiotherapy, one (5%) chemotherapy and three (17%) a combination of both. Mean follow-up duration was 49 months (range 3 - 138). Three (17%) patients died in hospital due to post-operative complications. Six (33%) patients died during follow-up due to disease progression. Mean progression-free survival was 47 months (range 0 - 113). Conclusion In conclusion, the overall survival was 50% for this group of patients with large sinonasal tumors. Progressive disease affects survival rate severely. Surgical complications were seen in five (28%) patients. Radiotherapy is associated with high complication rates. Radiation necrosis was a serious complication in two patients and could be treated with high dose steroids.

Genetic Rearrangements in Different Salivary Gland Tumors: A Systematic Review.

Salivary gland tumors (SGT) encompass a wide range of neoplasms, each with its own unique histological type and clinical presentation. This review hones in on prevalent subtypes of SGTs, including adenoid cystic carcinoma (ACC), salivary duct carcinoma (SDC), and polymorphous adenocarcinoma (PAC). The articles, identified through specific keywords, were meticulously screened in databases like PubMed, Scopus, Google Scholar, and Web of Science from 2018 to 2023. Eight articles delved into genetic modifications among the selected SGT types. A fusion protein known as MYB-NF1B is typically associated with ACC, promoting cell proliferation while inhibiting apoptosis. The presence of MYB modifications in ACCs is a beacon of hope, as it is linked to a more favorable prognosis. In contrast, SDCs often exhibit HER2 expression. The invasive nature of SGTs contributes to their resistance to treatment. In the case of PAC, the role of PRKD1 is particularly noteworthy. PRKD1, integrated with other genes from the PRKD1/2/3 cluster, helps to differentiate PAC from other diseases. Furthermore, the genetic profiles of KTN1-PRKD1) and PPP2R2A:PRKD1 are distinct. The significant genetic variability among SGTs necessitates meticulous examination. This field is in a constant state of evolution, with new discoveries reshaping our understanding. Genetics is a key player in deciphering SGTs and tailoring treatments. This complex neoplasm demands ongoing research to uncover all genetic influences, thereby enhancing diagnostic methodologies, therapeutic strategies, and patient outcomes.

How predominant cell and stroma types harmonize to predict head and neck adenoid cystic carcinoma outcomes?

Adenoid cystic carcinoma (ACC) is an uncommon tumor that usually appears in the major salivary glands of the head and neck region, including the minor glands in the oral cavity, sinonasal tract, and other sites. ACC of the head and neck may have a low-grade histological appearance. This malignant tumor has unusual clinical characteristics such as occasional regional lymph node metastases and a prolonged yet continuously advancing clinical course. Additionally, it is an invasive tumor with perineural invasion, difficult-to-clear margins, metastasis, and localized recurrence. The cribriform and tubular proliferation of basaloid cells, which mostly display a myoepithelial cellular phenotype, are ACC's distinct histologic characteristics. The degree of genetic alterations and aneuploidy observed in tumor genomes are linked to the severity of histologic grade, which correlates with clinical prognosis. The three predominant cell types (PCTs) i.e., conventional ACC (C-ACC), myoepithelial-predominant ACC (M-ACC), and epithelial-predominant ACC (E-ACC)-and their respective applications will be reviewed. The function of extracellular matrix (ECM) components such as laminin, type IV collagen, fibronectin, and tenascin are also emphasized. An attempt has been made to explore the recent molecular diversity, regulatory pathways prevalent in PCT, ECM with its genetic changes, and translational utility with targeted therapies for ACC.

The Potential Role of PSMA-Targeted PET in Salivary Gland Malignancies: An Updated Systematic Review.

BACKGROUND: Recent studies have suggested using positron emission tomography/computed tomography (PET/CT) with prostate-specific membrane antigen (PSMA)-targeting radiopharmaceuticals for the detection of salivary gland malignancies (SGM), particularly adenoid-cystic carcinoma (ACC). METHODS: The authors conducted an extensive review of the scientific literature to examine the potential diagnostic role of PET/CT using PSMA-targeting radiopharmaceuticals in salivary gland malignancies (SGMs) and adenoid cystic carcinoma (ACC). This study included newly diagnosed SGM patients and those with disease recurrence in their imaging evaluation. RESULTS: This updated systematic review included a total of six studies that examined the diagnostic performance of PSMA-targeted PET/CT in ACC. The articles provided evidence of a high detection rate of PSMA-targeting PET/CT in ACC across all clinical contexts examined. SGMs other than ACC exhibited poorer diagnostic performance. CONCLUSIONS: PSMA-targeted PET/CT seems promising in detecting ACC lesions; moreover, PSMA appears to be a suitable potential target for radioligand therapy. Prospective multicentric studies are warranted to strengthen the role of PSMA-targeting radiopharmaceuticals in ACC, as both diagnostic and theragnostic agents.

Salvage endoscopic nasopharyngectomy in locally recurrent carcinoma adjacent to the internal carotid artery-A case report.

Key Clinical Message: In cases adjacent to critical structures, such as the internal carotid artery, surgeons should meticulously explore the feasibility of surgery before declaring the neoplasm unresectable. Abstract: Salvage treatment for locally recurrent carcinoma of the nasopharynx constitutes a unique challenge. Surgery remains the gold standard treatment modality. Endoscopic nasopharyngectomy is considered a safe and feasible procedure overcoming the morbidities of an open surgery. Tumor adjacency to the internal carotid artery (ICA) is not an absolute contradiction for the endoscopic approach. Even in cases adjacent to critical structures, surgeons should meticulously explore the feasibility of surgery before declaring the neoplasm unresectable. We present the case of a 56-year-old male with locally recurrent adenoid cystic carcinoma (AdCC) of the nasopharynx adjacent to the ICA treated with endoscopic nasopharyngectomy.

Primary tracheal adenoid cystic carcinoma: A case report and analysis of the tumor immune microenvironment using single cell RNA sequencing.

BACKGROUND: Tracheal adenoid cystic carcinoma (ACC) is a slow growing yet aggressive malignancy with high rates of local recurrence as well as distant metastasis. Tracheal ACC exhibit a low mutation burden along with high mutational diversity, and generally do not respond well to chemotherapeutics. METHODS: We present a rare case of primary tracheal ACC initially presenting with nonspecific cervicalgia and globus sensation that was ultimately treated with tracheal resection followed by chemoradiation. Immune profiling of intratumoral T-cell receptor (TCR) repertoire was subsequently performed using single cell RNA sequencing (scRNAseq). RESULTS: We describe a rare case of primary tracheal adenoid cystic carcinoma highlighting several management principles as well as providing new insights into intratumor T cell populations. CONCLUSIONS: Primary tracheal ACC is most commonly treated with surgical resection followed by adjuvant therapy. Further characterization of the tumor immune microenvironment is necessary to better understand ACC disease biology and to identify potential therapeutic targets.

Letter to editor: Comment on "Clinical disease course and survival outcomes following disease recurrence in adenoid cystic carcinoma with and without NOTCH signaling pathway activation".

The study by Feeney et al. provides critical insights into the prognostic implications of NOTCH pathway activation in adenoid cystic carcinoma (ACC), particularly after disease recurrence. Utilizing both next-generation sequencing and immunohistochemistry, the research delineates the survival outcomes between NOTCH-activated and non-activated ACC groups, highlighting poorer outcomes in the former. The findings advocate for the targeted therapeutic approach and suggest a potential for personalized treatment strategies, emphasizing the need for further research into NOTCH pathway inhibitors and their clinical applications.

Response of FGFR-2 Positive Adenoid Cystic Carcinoma to Futibatinib: A Case Report.

Adenoid cystic carcinoma (ACC) is an uncommon and aggressive head and neck cancer mainly affecting minor salivary glands. It affects more women than men in their 60s and 70s. The tumor is typically locally aggressive and has a high rate of distant metastatic disease. This report unveils a potential avenue for targeted therapy for the management of metastatic disease: a patient with ACC who harbored a specific fibroblast growth factor receptor 2 (FGFR-2) mutation and responded significantly to a novel FGFR-2 inhibitor. This finding could pave the way for personalized treatment options for ACC patients with similar genetic alterations. Nevertheless, the use of futibatinib requires further investigation to optimize treatment protocols, including exploring combination therapies, identifying predictive biomarkers for treatment response, and developing strategies to overcome potential resistance.

Anal metastasis in esophageal cancer: A case report.

BACKGROUND: Esophageal cancer is the sixth leading cause of cancer-related death and eighth most common cancer, affecting > 450000 people worldwide. Esophageal squamous cell carcinoma is the most common histological type, whereas esophageal adenoid cystic carcinoma (EACC) is rare. The liver is the most common distant metastatic site in esophageal cancer. Anal metastasis is rare and has not been reported in clinical practice before. Here, we report anal metastases in a patient with EACC after regular chemotherapy and surgical resection. CASE SUMMARY: A 61-year-old esophageal cancer patient was found to have lung and brain metastases during standardized treatment. The patient's treatment plan was continuously adjusted according to the latest treatment guidelines. However, the patient subsequently noticed rectal bleeding and itching, and after obtaining pathology results at the local hospital, anal metastasis of esophageal cancer was diagnosed. CONCLUSION: Postoperative pathology and immunohistochemistry confirmed EACC with rare anal metastasis. More exploration of EACC diagnosis and treatment is needed.

Submandibular and sublingual salivary gland involvement in adenoid cystic carcinoma.

Adenoid cystic carcinoma (AdCC) is a malignant salivary gland neoplasm that presents as an indolent but aggressive neoplasm. AdCC histogenesis is linked to the intercalated ducts of the salivary glands, equally affecting the major and minor glands. AdCC is associated with distant metastasis, most commonly to the lungs, and a high recurrence rate. AdCC accounts for 4.2% of all tumors. About 55% of all reported cases affect the submandibular gland, and around 50% of AdCC cases occur in the minor salivary glands. The present review describes a case of AdCC which presented a single nodular swelling on the right side involving the floor of the mouth. It also consolidates the histopathological profile of a case of AdCC with all the relevant histopathological features.

Locoregional Recurrence in Adenoid Cystic Carcinoma of the Breast: A Retrospective, Multicenter Study (KROG 22-14).

Purpose: This study aims to evaluate the treatment approaches and locoregional patterns for Adenoid cystic carcinoma (ACC) in the breast, which is an uncommon malignant tumor with limited clinical data. Materials and Methods: A total of 93 patients diagnosed with primary ACC in the breast between 1992 and 2022 were collected from multi-institutions. All patients underwent surgical resection, including breast-conserving surgery (BCS) or total mastectomy (TM). The recurrence patterns and locoregional recurrence-free survival (LRFS) were assessed. Results: Seventy-five patients (80.7%) underwent BCS, and 71 of them (94.7%) received post-operative radiation therapy (PORT). Eighteen patients (19.3%) underwent TM, with 5 of them (27.8%) also receiving PORT. With a median follow-up of 50 months, the LRFS rate was 84.2% at 5 years. Local recurrence (LR) was observed in 5 patients (5.4%) and 4 cases (80%) of the LR occurred in the tumor bed. Three of LR (3/75, 4.0%) had a history of BCS and PORT, meanwhile, two of LR (2/18, 11.1%) had a history of mastectomy. Regional recurrence occurred in 2 patients (2.2%), and both cases had a history of PORT with (n=1) and without (n=1) irradiation of the regional lymph nodes. Partial breast irradiation (p=0.35), BCS (p=0.96) and PORT in BCS group (p=0.33) had no significant association with LRFS. Conclusion: BCS followed by PORT was the predominant treatment approach for ACC of the breast and local recurrence mostly occurred in the tumor bed. The findings of this study suggest that partial breast irradiation might be considered for PORT in primary breast ACC.

Correlation of the Size of Adenoids With Impedance Audiometry Findings.

Introduction Adenoid tissue is part of the first line of immunity of the upper aero-digestive tract. It is located in the postero-superior wall of the nasopharynx behind the choana. Adenoid hypertrophy, a common childhood disorder, significantly contributes to the pathogenesis of otitis media with effusion (OME), which is the leading cause of hearing impairment in young children. This condition can result in delayed speech, poor academic performance, and language development issues. Assessing the size of the adenoids and their correlation with OME is crucial, as undiagnosed cases can lead to complications such as atelectasis of the tympanic membrane and cholesteatoma. Clinical examination of the nose alone is often insufficient, and children do not cooperate for nasal endoscopy. Therefore, a lateral radiograph of the skull is considered the most reliable method for assessing the adenoid size. The size of the adenoids can affect Eustachian tube patency, which is reflected in the results of impedance audiometry. This study aimed to correlate the size of adenoids with impedance audiometry findings. Methods This cross-sectional observational study was conducted in the Department of Otorhinolaryngology of a tertiary care hospital from October 1, 2022, to March 31, 2024. A sample size of 50 patients was taken for the study. The inclusion criterion of selection of the patients included patients aged 3 to 15 years, who suffered from recurrent attacks of upper respiratory tract infections, particularly those with adenoid facies confirmed by X-ray with a non-perforated tympanic membrane. Exclusion criteria encompassed patients below 3 or above 15 years, and those with acute or chronic suppurative otitis media, craniofacial anomalies, or nasal pathologies like polyps. Adenoids were graded using X-ray imaging of the nasopharynx, and correlations between the adenoid size and impedance audiometry findings, such as middle ear pressure and compliance, were analyzed.  Results The study assessed the relationship between the adenoid size and impedance audiometry findings, focusing on middle ear pressure and compliance, as well as the occurrence of OME. The results indicated a significant decline in middle ear pressure with increasing adenoid grades. Specifically, adenoid grade 1 had an average pressure of -3.50 daPa, while grade 4 had the lowest average pressure at -119.72 daPa. This trend was statistically significant with a p-value of 0.00042. Similarly, compliance values also decreased with higher adenoid grades. Grade 1 had an average compliance of 0.64 ml, whereas grade 4 had the lowest average compliance at 0.28 ml. This relationship was statistically significant, as indicated by a p-value of 0.0048. Additionally, the analysis showed that a significant majority of patients with enlarged adenoids also presented with OME, highlighting a strong association between adenoid hypertrophy and this condition. Conclusion The study concluded that larger adenoids were associated with lower middle ear pressure and reduced compliance. Additionally, a significant majority of patients with enlarged adenoids also had OME. This underscores the importance of evaluating adenoid hypertrophy in the context of OME due to its potential impact on childhood hearing and development.

MYB expression by immunohistochemistry is highly specific and sensitive for detection of solid variant of adenoid cystic carcinoma of the breast among all triple-negative breast cancers.

BACKGROUND: Adenoid cystic carcinoma is a rare subtype of triple-negative breast carcinoma. These low-grade tumours, which are treated by simple mastectomy and have an excellent prognosis compared to other triple-negative breast carcinomas. Solid-variant adenoid cystic carcinomas have basaloid features and are difficult to distinguish morphologically from other triple-negative breast cancers. Breast adenoid cystic carcinoma exhibits MYB protein overexpression, which can be detected by immunohistochemistry (IHC). AIM: We compared the IHC expression of MYB in solid-variant adenoid cystic carcinoma with that in other triple-negative breast cancers. METHODS: We conducted IHC staining of 210 samples of triple-negative breast cancers, including solid-variant adenoid cystic carcinoma (n = 17), metaplastic breast carcinoma (n = 44), basaloid triple-negative breast cancer (n = 21), and other triple-negative invasive ductal carcinoma (n = 128). We classified nuclear staining of MYB as diffuse/strong (3+), focal moderate (2+), focal weak (1+), or none (0). RESULTS: All 17 solid/basaloid adenoid cystic carcinoma cases exhibited 3+ MYB expression. Of the 21 solid/basaloid triple-negative breast cancers, one (5%) had 2+ expression, seven (33%) 1+ expression, and 13 (62%) 0 expression. Of the 44 metaplastic carcinoma cases, 39 cases (89%) had no (0) staining, and the other five cases had focal weak (1+) or moderate (2+) staining. Among the 128 triple-negative invasive ductal carcinoma cases, 92 cases (72%) had no (0) staining, 36 cases (28%) exhibited focal weak (1+) or moderate (2+) staining. CONCLUSIONS: Our study revealed diffuse/strong MYB staining (3+) only in solid/basaloid adenoid cystic carcinomas. Thus, we recommend routine MYB IHC staining in triple-negative breast carcinoma with solid/basaloid morphology to improve diagnostic accuracy.

The Diagnostic Value of CT Scan in Identifying Adenoid Hypertrophy in Adults: A Case Report.

The utility of computed tomography (CT) scan in diagnosing adenoid hyperplasia in adults. A 22-year-old woman presented with persistent bilateral nasal obstruction, anterior nasal discharge, mouth breathing, and snoring over the past three months. Despite attempts with both local and systemic decongestants, there was no improvement, and flexible nasopharyngoscopy could not be conducted. CT scans revealed a heterogeneously enhancing space-occupying mass in the nasopharynx, and a rare diagnosis of adult adenoid hypertrophy was reported. The patient responded to a combination of painkillers, antibiotics, and nasal decongestants. Adenoid hyperplasia in adults is quite rare and inadequate examination by indirect posterior rhinoscopy may lead to misdiagnosis and mismanagement. A CT scan not only provides a clearer view of the nasopharyngeal space and adenoids but also reveals details about the nature of lesions, including their extension and potential bone destruction, suggesting the presence of a malignant tumor. Additionally, a CT scan proves valuable in diagnosing chronic sinusitis.

Molecular characterization of the salivary adenoid cystic carcinoma immune landscape by anatomic subsites.

Adenoid cystic carcinoma (AdCC) is a slow-growing salivary gland malignancy that relapses frequently. AdCCs of the submandibular gland exhibit unique differences in prognosis and treatment response to adjuvant radiotherapy compared to other sites, yet the role of tumor anatomic subsite on gene expression and tumor immune microenvironment (TIME) composition remains unclear. We used 87 samples, including 48 samples (27 AdCC and 21 normal salivary gland tissue samples) from 4 publicly available AdCC RNA sequencing datasets, a validation set of 33 minor gland AdCCs, and 39 samples from an in-house cohort (30 AdCC and 9 normal salivary gland samples). RNA sequencing data were used for single sample gene set enrichment analysis and TIME deconvolution. Quantitative PCR and multiplex immunofluorescence were performed on the in-house cohort. Wilcoxon rank-sum, nonparametric equality-of-medians tests and linear regression models were used to evaluate tumor subsite differences. AdCCs of different anatomic subsites including parotid, submandibular, sublingual, and minor salivary glands differed with respect to expression of several key tumorigenic pathways. Among the three major salivary glands, the reactive oxygen species (ROS)/nuclear factor erythroid 2-related factor 2 (NRF2) pathway signature was significantly underexpressed in AdCC of submandibular compared to parotid and sublingual glands while this association was not observed among normal glands. Additionally, the NRF2 pathway, whose expression was associated with favorable overall survival, was overexpressed in AdCCs of parotid gland compared to minor and submandibular glands. The TIME deconvolution identified differences in CD4+ T cell populations between AdCC of major and minor glands and natural killer (NK) cells among AdCC of minor, submandibular, and parotid glands while plasma cells were enriched in normal submandibular glands compared to other normal gland controls. Our data reveal key molecular differences in AdCC of different anatomic subsites. The ROS and NRF2 pathways are underexpressed in submandibular and minor AdCCs compared to parotid gland AdCCs, and NRF2 pathway expression is associated with favorable overall survival. The CD4+ T, NK, and plasma cell populations also vary by tumor subsites, suggesting that the observed submandibular AdCC tumor-intrinsic pathway differences may be responsible for influencing the TIME composition and survival differences.

Adenoid Cystic Carcinoma of the Nasopharynx: A Retrospective Study of 12 Cases.

Objective: This study aimed to describe the experience of a single institution in China in treating adenoid cystic carcinoma of the nasopharynx. Methods: We reviewed the previous literature and conducted a retrospective analysis of 12 patients who diagnosed with nasopharyngeal adenoid cystic carcinoma (NACC) in clinical data, treatment, and follow-up data during 2019 to 2021. Results: Patients ranged in age from 32 to 68 years (mean 40.7 years, median 48.5 years), with a male to female ratio of 5:7. Most of our patients have T4a and T4b diseases (50% and 25%, respectively). A quarter of patients develop distant metastases. Among the 12 patients, 7 of them have positive margins under the microscope (7/12, 58.3%). The chief clinical manifestations were epistaxis, facial swelling, facial pain, headache ear stuffy, and hearing loss. If the tumors involved with cavernous sinus, brain stem infiltrated, and internal carotid artery circumvented, patients will undertake routine enhanced magnetic resonance imaging with Magnetic Resonance Angiography/Magnetic Resonance Venogram (MRA/MRV) to clearly show the lesion region. All patients underwent endoscopic endonasal approach. Fifty percent of patients received radiotherapy and 25% of patients received chemotherapy. None of the patients was lost and the follow-up time ranged from 16 to 45 months. The mean and median follow-up were 2.08 and 1.58 years. Two patients were dead of distant metastasis within 18 and 20 months after the surgery, and another patient with recurrent NACC died of hemorrhage. Conclusion: NACC is a rare malignant tumor that occurs in the nasopharynx, which can grow along the nerve, destroy the bone of the skull base, and metastasize to other organs. Up to now, there is no standard treatment. Our results show that endoscopic sinus surgery is a better choice for advanced or recurrent NACC.