Adenoid Cystic Carcinoma Of Head Research Articles

The Efficacy of Immunotherapy and Clinical Utility of Comprehensive Genomic Profiling in Adenoid Cystic Carcinoma of Head and Neck.

Background and Objectives: Adenoid cystic carcinoma (ACC) of the head and neck is generally slow-growing but has a high potential for local recurrence and metastasis to distant organs. There is currently no standard pharmacological treatment for recurrent/metastatic (R/M) ACC, and there are cases in which immune checkpoint inhibitors (ICIs) are administered for ACC according to head and neck squamous cell carcinoma (HNSCC). However, the efficacy of ICIs for ACC remains unclear, and the predictive biomarkers need to be elucidated. Materials and Methods: The Center for Cancer Genomics and Advanced Therapeutics (C-CAT) database enabled the retrospective but nationwide analysis of 263 cases of ACC of the head and neck. Then, we examined and reported four cases of ACC that received ICIs and comprehensive genomic profiling (CGP) in our institution. Results: The C-CAT database revealed that 59 cases out of 263 received ICIs, and the best response was 8% of objective response rate (ORR) and 53% of disease control rate (DCR) (complete response, CR 3%, partial response, PR 5%, stable disease, SD 44%, progressive disease, PD 19%, not evaluated, NE 29%). The tumor mutational burden (TMB) in ACC was lower overall compared to HNSCC and could not be useful in predicting the efficacy of ICIs. Some cases with MYB structural variants showed the response to ICIs in the C-CAT database. A patient with MYB fusion/rearrangement variants in our institution showed long-term stable disease. Conclusions: ICI therapy is a potential treatment option, and the MYB structural variant might be a candidate for predictive biomarkers for immunotherapy in patients with R/M ACC.

Adenoid cystic carcinoma of head and neck: Summary and review of imaging findings

PurposeCurrent reports of adenoid cystic carcinoma of the head and neck (ACC) are all case reports, and there is no basilar summary of its imaging findings. This study aims to summarise ACC's computed tomography (CT) and magnetic resonance imaging (MRI) findings to improve radiologists' knowledge of this disease. MethodsWe collected clinical and imaging data of patients with ACC during the last decade, and two radiologists retrospectively analysed the imaging characteristics. ResultsOf the 16 patients included, six were able to self-perceive bulkiness, and 11 had regional pain. Tumour morphology was regular in six cases, with clear borders in 11 cases, invasion of the surrounding bony mass in 12 cases, and invasion of peripheral nerves in 15 cases. CT mostly shows an irregular soft-tissue density mass with mild-to-moderate enhancement after contrast medium administration. On MRI, the ACC showed isointense or hypointense signals on T1-weighted images (T1WI) and hyperintense or slightly hyperintense signals on T2-weighted images (T2WI). All signals were markedly enhanced after gadolinium enhancement. ConclusionsACC often has an irregular morphology, sometimes with a cystic component, enhancement on enhancement scans, easy destruction of adjacent bone, and invasion of peripheral nerves. The diagnosis should be considered when these features are encountered in clinical practice.

Prognostic Significance of Primary Tumor Surgery in Adenoid Cystic Carcinoma Patients With Distant Metastases at Diagnosis: A Population-Based Database Analysis in Head and Neck Region.

Purpose: Adenoid cystic carcinoma (ACC) is an aggressive cancer that often leads to poor prognosis, especially when it has metastasized. The prognostic significance of primary tumor surgery (PTS) for ACC with distant metastasis (DM) at the time of diagnosis has not been extensively studied. Methods: Using data from the surveillance, epidemiology, and end results (SEER) database from 2010 to 2019, we identified patients with ACC in head and neck region and synchronous DM. We evaluated the effect of PTS on different patterns of metastasis using Kaplan-Meier analysis, log-rank tests, and multivariate Cox regression analysis to assess the therapeutic benefit of PTS in the overall cohort and various subgroups. Results: Of the 192 identified patients with synchronous metastatic ACC of head and neck, 91 (47.4%) underwent PTS. Patients who received PTS had significantly better survival than nonsurgical patients (median overall survival: 19 vs 43 months, P = .006). Cox regression analysis also showed that PTS was associated with improved survival (HR = .46, 95% CI: 0.42-0.88; P = .028). In sub-analyses, except for patients with liver metastases [overall survival (OS), P = .107, cancer-specific survival (CSS), P = .153], PTS consistently conferred significant survival benefits in patients with bone metastases (OS, P = .041, CSS, P = .065) and lung metastases (OS, P = .016, CSS, P = .027). PTS also led to better survival in patients who did not receive radiotherapy (median OS: 13 vs 52 months, P = .007). Conclusion: Our study suggests that PTS in metastatic ACC patients of head and neck improved overall and CSS, particularly in those with bone or lung metastases and those who did not receive radiotherapy. However, further prospective studies are needed to confirm these findings, and the indications for PTS in metastatic ACC should be carefully evaluated by a multidisciplinary board.

Adenoid cystic carcinoma of head and neck. A 5-year retrospective study: Experience in a single third-level reference center

Adenoid cystic carcinoma of head and neck. A 5-year retrospective study: Experience in a single third-level reference center

Prognostic Model and Nomogram for Early-Onset Adenoid Cystic Carcinoma of Head and Neck: A Retrospective SEER-Based Analysis.

Adenoid cystic carcinoma of head and neck (ACCHN) is an uncommon head and neck cancers, whose predilection age is 40 to 60. Some studies have revealed that early-onset cancers, such as colorectal cancers and esophageal adenocarcinoma, might present some unique clinicopathological features and have different prognosis with late-onset ones. However, little is known about the early-onset ACCHN. This study aimed to develop a prognostic nomogram for overall survival (OS) of patients younger than 40 with ACCHN. Cases with ACCHN from 1975 to 2016 were retrieved from SEER-18 program. Demographic, clinical, and survival outcomes data of patients were identified for further analysis. The caret package was used to randomly divide early-onset patients into a training cohort and a validation cohort. A prognostic nomogram was constructed based on the univariate and multivariate Cox analysis. The discriminative ability and calibration power of the nomogram was evaluated by the concordance index (C-index), calibration curve, and receiver operating characteristic (ROC) curve. A total of 5858 cases with ACCHN were selectively retrieved from SEER program in this study. The number of patients younger than 40, which was defined as early-onset ACCHN in this study, was 825. Based on the outcomes of multivariate analysis, tumor size, chemotherapy, surgery, and stage were selected for the construction of nomogram to predict 10-year OS. The C-index was 0.792 (95%CI 0.760-0.823) and 0.776 (95%CI 0.720-0.832) in the training and validation set, respectively. The area under the ROC curve values were 0.875 (95%CI 0.810-0.940) and 0.833(95%CI 0.754-0.912). The calibration plot indicated that this nomogram had proper calibration in both the training and validation cohorts. A novel prognostic nomogram for early-onset ACCHN was constructed and validated in this study. This nomogram could be applied for assisting clinicians to more accurately assess the prognosis of young patients, which might facilitate clinical decision-making and subsequent follow-up.

Prognosis of adenoid cystic carcinoma of head and neck and risk factors for lung metastasis

Objective: To analyze the prognosis and risk factors of lung metastasis of patients with adenoid cystic carcinoma(ACC) of head and neck. Methods: A retrospective study was conducted. The data of 157 patients with ACC of head and neck treated in Beijing Tongren Hospital, Capital Medical University from January 2014 to October 2020 were collected, including 72 males and 85 females, with onset age between 14 and 72 years old. According to whether lung metastasis occurred, the patients were divided into lung metastasis group (88 cases) and non-pulmonary metastasis group (69 cases). Kaplan-Meier method was used to calculate the overall survival rate and progression-free survival rate using SPSS 26.0 software. Log-rank test was used to evaluate statistically relevant clinicopathological factors. Cox proportional risk model was used in multivariate analysis for the factors affecting the lung metastasis-free survival using R Studio 1.2.5042. Results: The 3-year and 5-year overall survival rates were 91.5% and 85.2%, respectively. The 3-year and 5-year progression-free survival rates were 57.7% and 34.3%, respectively. Univariate analysis showed that primary site, histological grade, high-grade transformation, Ki-67, T stage, and lymph node status were the risk factors for lung metastasis (χ2=11.78, 10.41, 4.06, 4.71, 5.37, 16.20, respectively, all P<0.05). Multivariate analysis showed independent risk factors for lung metastasis, including submandibular gland and sublingual gland (HR=3.53, 95%CI: 1.19-10.46, P<0.05), T3-4 stage (HR=3.09, 95%CI: 1.54-6.23, P<0.05), and Grade Ⅱ-Ⅲ grade (HR=2.47, 95%CI: 1.26-4.86,P<0.05). Conclusion: Distant metastasis, mainly pulmonary metastasis, affects the long-term prognosis of patients with ACC significantly. Primary site, T stage and histopathological grade can be used as the predictors for the risk of lung metastasis.

Prognosis of adenoid cystic carcinoma in head and neck region treated with different regimens-A single-centre study.

No study has evaluated the impact of regimen on recurrence, metastasis and survival in patients with adenoid cystic carcinoma (ACC). The present study aimed to compare the efficacy of radioactive seed implantation and other regimens in treating ACC, so as to investigate the clinical applicability of radioactive seed implantation and determine the indications for this regimen. A total of 188 patients with ACC in oromaxillofacial region were allocated to four groups according to the treatment regimen: group 1 was treated with a combination of surgery and 125 I seed therapy, group 2 with a combination of surgery and external radiotherapy, group 3 with surgery, whereas group 4 was untreated. The Kaplan-Meier method was used to assess the survival rates, and the Cox regression analyses were used to identify the associated prognostic factors. The overall survival rates of 188 patients and groups 1, 2, 3 and 4 were 85.7%, 75%, 68.2% and 37.5%, respectively. Cox regression analysis revealed that age, T stage, N stage and regimen were independent prognostic factors of survival. Amongst patients with primary ACC, the efficacy of radioactive seed implantation was higher in those with perineural invasion than in those without. Patient age, T stage, N stage and regimen are independent prognostic factors of survival in patients with ACC. Patients treated with surgery combined with postoperative 125 I seed radiotherapy have a higher overall survival rate, and those with perineural invasion are more suitable for radioactive seed implantation therapy.

Local Recurrence and Survival in Patients with Adenoid Cystic Carcinoma of Head and Neck

Local Recurrence and Survival in Patients with Adenoid Cystic Carcinoma of Head and Neck

Detection of novel fusion genes by next-generation sequencing-based targeted RNA sequencing analysis in adenoid cystic carcinoma of head and neck

Adenoid cystic carcinoma (AdCC) is a rare, indolent salivary gland tumor that is reported to be driven by fusion genes. However, MYB/MYBL1-NFIB fusions have been detected in <60% of all AdCC cases and the oncogenic driver mutations in approximately 40% of AdCC remain unknown. Our aim was to identify novel gene fusions in AdCC. We investigated 20 AdCC cases using a targeted RNA sequencing panel to identify gene fusions and performed quantitative real-time reverse transcription polymerase chain reaction to assess MYB, MYBL1, and NFIB expression levels. A total of 36 fusion transcripts in 15 cases were detected and validated by Sanger sequencing. The MYB-NFIB and MYBL1-NFIB fusion genes were detected in 9 and 3 cases, respectively, in a mutually exclusive manner. Furthermore, novel gene fusions, namely, NFIB-EPB41L2, MAP7-NFIB, NFIB-MCMDC2, MYBL1-C8orf34, C8orf34-NFIB, and NFIB-CASC20, were identified. Among them, NFIB-EPB41L2 and NFIB-MCMDC2 are thought to activate MYB and MYBL1 expression, respectively, through the insertion of a genomic segment in proximity to MYB and MYBL1 genes, respectively. Six novel gene fusions other than MYB/MYBL1-NFIB were identified. The detection of novel fusion genes and investigation of the molecular mechanism will contribute to the development of novel molecular targeted therapies for this disease.

Outcome of Adenoid Cystic Carcinoma of Head and Neck After Postoperative Intensity Modulation Radiotherapy: A Single Institution Study.

ObjectiveThis study was retrospectively evaluated the outcome of postoperative intensity modulation radiotherapy (IMRT) for patients with adenoid cystic carcinoma of head and neck (ACCHN), and identified the unfavorable prognostic factors.MethodsFifty-five ACCHN patients treated in Sichuan Cancer Hospital between January 2007 and December 2016 were retrospectively evaluated. Median age of patient was 47 years (range, 21–73 years). Thirty-five patients were male and 20 were female. In 30 patients, tumors were located in major salivary glands (54.5%), and 25 patients in minor salivary glands (45.5%). The numbers of R0, R1, and R2 surgical resection classification patients were 22 (40.0%), 20 (36.4%), and 13 (23.6%). The median total RT dose was 62 Gy (range, 46–72 Gy), and 54.5% of patients were treated with adjuvant chemotherapy. Statistical analyses were performed using the Log rank test for univariate analysis and the Cox proportional hazard model for multivariate analysis.ResultsMedian follow-up period was 68.5 months (12–132 months). The 5-year local-regional recurrence-free survival (LRRFS), distant metastasis-free survival (DMFS) and overall survival (OS) were 93.9%, 75.3% and 82.5%. In univariate analysis, T stages (p=0.025) and AJCC stages (p=0.036) were the prognostic factors for OS; Age (p=0.042), T stages (p=0.025), N stages (p=0.021), AJCC stages (p=0.021) and adjuvant chemotherapy (p=0.010) were the prognostic factors for DMFS; T stage (p=0.049) was the prognostic factor for LRRFS. In multivariate factors analyses, T stages (p=0.026), AJCC stages (p=0.028) and RT dose (p=0.025) were the significant prognosticators for OS. The most common acute toxicities over three degrees were myelosuppression (5.5%), mucositis (9.1%) and dermatitis (1.8%).ConclusionPostoperative IMRT seems to achieve reasonable local-regional control and OS in patients with adenoid cystic carcinoma of head and neck, with acceptable treatment relative toxicities.

Prognostic value of elective neck dissection in adenoid cystic carcinoma of head and neck: a meta-analysis: A call for randomized trials and international consensus

Adenoid cystic carcinoma of head and neck (AdCCHN) is an uncommon salivary gland cancer characterized for infrequent neck metastases, and high rate of local and distant recurrence. The aim of this meta-analysis was to analyse the significance of elective neck dissection (END) in terms of overall survival (OS) in patients with AdCCHN. A systematic literature search and meta-analysis was performed. Endpoint assessed by this meta-analysis included 5-year OS (death from any cause). Statistical heterogeneity was assessed using the Cochrane Q test and I2 statistic. A pooled odds ratio (OR) was reported with 95% confidence interval (CI). There were 1934 patients in the END arm and 3083 in the observation group. The pooled OR, calculated for END vs. observation, was 0.94. Patients receiving END had similar risk for death compared to observation cohort (P=0.76). No significant difference in final outcome after patient stratification based on T stage was identified (OR for T1/T2 1.27, P=0.39; OR for T3/T4 0.95, P=0.90). Observation for cN0 neck is a reasonable option in AdCCHN. These findings suggest the need for prospective trials on indications and extent of END in AdCCHN.

Concurrent chemoradiotherapy for locally advanced unresectable adenoid cystic carcinoma of head and neck: experience from a single institute.

To analyze the outcome of locally advanced unresectable adenoid cystic carcinoma (ACC) of head and neck treated with radical concurrent chemoradiotherapy (CRT) at a single tertiary care centre. Between 2011 and 2018, 23 patients with locally advanced unresectable ACC of head and neck treated with non-surgical radical treatment with concurrent chemoradiotherapy were evaluated for outcome and toxicity. All but one patient received cisplatin-based concurrent chemotherapy and 74% of patients were treated with intensity-modulated radiotherapy. Median follow-up was 53months (range 3-115months). Following treatment, 11 patients achieved complete response (47.8%) and of the 12 patients with residual disease, 7 patients additionally had disease stabilization without local progression. Overall 15 patients had disease progression. Median time to progression was 28months (range 6-67months). The 3-year and 5-year overall survival, local progression-free survival (LPFS) and distant progression-free survival (DPFS) were 78%, 79.7%, 67.4% and 63%, 50.9%, 48.6%, respectively. Acute grade 3 mucositis was observed in three patients, and one patient additionally developed grade 4 neutropenia with subsequent complete recovery. No grade 3 or higher late toxicity was observed. Radical concurrent chemoradiotherapy is a promising treatment option in locally advanced unresectable ACC with acceptable toxicity.

Carcinoma adenoide quístico de cabeza y cuello. Un estudio retrospectivo de 5 años. Experiencia en un centro de referencia de tercer nivel

Adenoid cystic carcinoma (ACC) is a rare tumor, with an annual incidence of 3-4.5 cases/million. We conducted a descriptive and retrospective study. We included patients diagnosed with ACC of head and neck of a single third-level referral center, from January 2008 to December 2013. A total of 23 patients were included in the study, 10 males (43.5%) and 13 females (56.5%) (F:M ratio 1.3:1). Median age was 52 years. Localization was predominantly observed in the oral cavity (26%) followed by the palate (17.4%). At presentation, 15 patients (65.2%) had clinical Stage II. The most frequent histology was the cribriform type in 10 patients (43.5%). Following resection, positive margins were observed in 15 (65.2%), neural invasion in 8 (34.8%), and lymphovascular invasion in 2 (8.7%) patients. The median time to recurrence in our population was 23 months. Recurrence at 5 years was observed in 15 patients (65%). At 2 years, recurrence was predominantly local in 7 patients (46%); whereas after 2 years, it was predominantly pulmonary 6 patients (40%). Overall, 5 year-survival was 78%. Cystic adenoid carcinoma is characterized by a high recurrence rate. Nevertheless, it has a high overall 5-year survival, which justifies an aggressive treatment.

Adenoid cystic carcinoma of head and neck

Adenoid cystic carcinoma is a relatively rare tumor of head and neck. Due to lack of high-quality evidence, optimal treatment still controversial and continues to evolve over time. We conducted a retrospective review of 26 patients with adenoid cystic carcinoma of head and neck treated between 1993 and 2015 in the radiation Oncology Center of Habib Bourguiba Hospital, Sfax, Tunisia. Demographic, clinical, treatment, and survival details of the patients were collected and analyzed. Disease-free survival (DFS) and overall survival (OS) were estimated by Kaplan-Meier method. The mean age of patients in the study was 51 years (range:16-73 years), with a male preponderance (54% vs. 46%). The most commonly primary disease sites were the parotid glands (34.6%), the oral cavity (19.66%) and the submandibular glands (11.5%). More than half the patients presented with earlier-stage disease (T1/2:73%); most had a negative nodal status (92.3%). Twenty-four patients (92.4%) received surgery with adjuvant RT; one (3.8%) RT only and another (3.8%) surgery only. Fourteen (53.8%) patients had positive margins and no additional patients had close margins. Perineural invasion (PNI) was documented in 8 (32%) patients. The median time to any recurrence was 21 months (range:3-204 months). Five-year overall survival and disease-free survival rates were 62% and 53%. Solid histological subtype, positive surgical margins, PNI and advanced stages were predictors of poor outcome in adenoid cystic carcinoma of head and neck. Surgery followed by adjuvant radiotherapy remains the treatment of choice that may provide local control and prolong patient survival.

Elective neck dissection in adenoid cystic carcinoma of head and neck: yes or no? A systematic review.

Adenoid cystic carcinoma of head and neck (AdCCHN) is salivary gland malignancy known for its slow progression accompanied with perineural invasion, rare lymphatic spread to the neck, high rate of local recurrence and delayed onset of distant metastases. While local recurrence and distant metastases are most common patterns of relapse and cause of death in AdCCHN, it has been debated whether lymph node involvement affects the course of the disease. The aim of this systematic review was to address this important issue and to recommend guidelines on clinically node-negative neck management in patients with AdCCHN. A systematic review of the published literature on cases of elective neck dissection (END) among patients with AdCCHN was performed. This analysis included a total of 5767 AdCCHN patients with 2450 ENDs, ranging from 10 to 1190. Elective lymphadenectomy was employed in 42.5% of patients with AdCCHN (range 9.2-100%). The overall rate of occult neck metastases in patients with AdCCHN was reported to range between 0 and 43.7%, the average being 13.9%. If performed, END should be limited to levels I-III of the ipsilateral neck since occult metastases are exclusively located within these neck regions. Although END is associated with a prolonged regional recurrence-free period, it influence on final outcome or survival is still controversial. This review strongly supports conduction of prospective trials on indications, prognostic significance and extent of END in AdCCHN.

Genomic Alterations Landscape in Adenoid Cystic Carcinoma of Head and Neck

Introduction: Adenoid cystic carcinoma (ACC) accounts for 1% of all head and neck cancers and 10% to 22% of all malignant tumors of the salivary glands. ACC is associated with MYB- nuclear factor I/B-type (NFIB) gene fusion in about 50% of the cases. The genetic alteration (GA) landscape of ACC of head and neck region in this aspect has not been much studied, mainly due to small cohorts. We aim to describe the GA landscape of MYB-NFIB gene fusions, copy number alterations, and mutations of the related gene MYBL1 in human ACC by studying its association with regard to demographics and histopathological parameters. Materials and Methods: Using the GA data of ACC from a peer-reviewed web portal, we studied the presence of MYB-NFIB gene fusion with respect to age and gender distribution, site involved (major/minor salivary gland, lacrimal gland, cancer metastasis, others), histopathological diagnosis, perineural invasion (PNI), and the overall number of mutations. Descriptive statistics and Chi square test were performed. Data were entered and analyzed using SPSS version 23. The mutation counts between the type of gene fusions involved and the gender of patients was studied using analysis of variance tests. Results: Of the 214 reported ACC cases, 65 (30%) cases had MYB-NFIB fusion alone, 15 (7%) other had rare fusions, and 12 (6%) other had GAs. Perineural invasion was found to be associated with MYB gene fusion cases with or without copy number alterations (P = 0.022). The number of mutations were significantly associated with the gender of patient (P = 0.003) and NFIB gene fusions (P = 0.001). The other factors had no statistical significance. Conclusion: MYB-NFIB gene fusion and associated GA correlates with perineural involvement in ACC.

Adenoid cystic carcinoma of head and neck: A retrospective clinical analysis of a single institution

In this study, patients with head and neck adenoid cystic carcinoma were investigated with respect to disease characteristics, treatments, and potentially prognostic variables. Between January 1995 and December 2010, 29 patients with ACC of the head and neck treated at our institution were retrospectively reviewed. The 29 patients had a mean age of 54.7 years. Of the 29 patients, 4 (13.8%) underwent surgical resection only, 21 (72.4%) underwent surgery with radiotherapy, and 4 (13.8%) received postoperative chemoradiotherapy. Of 9 patients (31.0%) who experienced a recurrence within the follow-up period, 3 (10.3%) had local recurrence and 5 (17.2%) had distant metastasis; 1 (3.4%) patient had both local recurrence and distant metastatic spread. ACC is a relatively rare tumor associated with long survival, even in metastatic settings. Despite aggressive therapy with surgery, radiotherapy, and chemo- therapy, a marked potential for local and distant recurrence still remains.

EP-1053: Analysis of outcomes of Adenoid cystic Carcinoma of Head and Neck region: Single Institution Study

EP-1053: Analysis of outcomes of Adenoid cystic Carcinoma of Head and Neck region: Single Institution Study

EP-1067: is adenoid cystic carcinoma (ACC) radioresistant?: the effect of radiotherapy for ACC of head and; neck

EP-1067: is adenoid cystic carcinoma (ACC) radioresistant?: the effect of radiotherapy for ACC of head and; neck

Clinicopathologic and prognostic factors in adenoid cystic carcinoma of head and neck minor salivary glands: A clinical analysis of 130 cases

PurposeThis study was to investigate clinicopathologic characteristics and prognostic factors in adenoid cystic carcinoma of head and neck minor salivary glands. Materials and methodsWe conducted a retrospective review of 130 patients with adenoid cystic carcinoma of head and neck minor salivary glands that were evaluated between 2000 and 2013 in Beijng Tongren Hospital. ResultsFive-year overall survival and disease-free survival rates were 80.8% and 55.6%. Local recurrence rate was 40%, regional recurrence 3.8%, and distant metastasis was 28.5%. On univariate analysis, solid histological subtype, perineural invasion, positive surgical margins and advanced stages were found to be poor prognostic indicators. On multivariate analysis, solid histological subtype and positive surgical margins were significant prognostic factors of worse overall survival. ConclusionsSolid histological subtype and positive surgical margins were the most important predictors of poor outcome in adenoid cystic carcinoma of minor salivary glands. Surgery with postoperative radiation were recommended treatment and offered durable local control.