Adenoid Cystic Carcinoma Research Articles

Demographic, Disease, and Treatment Characteristics of Primary Tracheal Cancers

Abstract Background Primary tracheal cancers (PTCs) are rare neoplasms underreported in the literature. No consensus guidelines exist for the treatment of these cancers and multimodal management of these cancers has not been adequately explored for cases diagnosed over the past 2 decades. Methods The Surveillance, Epidemiology, and End Results (SEER) database was queried to identify patients with PTC. Cox proportional hazards and log-rank testing was used to assess the association between demographic and treatment variables and 5-year cause-specific survival (CSS). Results Among the 689 identified patients, age < 65 years at diagnosis (hazard ratio [HR] 0.64, p < 0.001), non-squamous cell carcinoma (SCC) histology (HR 0.22, p < 0.001), and treatment with surgery (HR 0.43, p < 0.001) were all associated with increased 5-year CSS. Regarding disease histology, patients with adenoid cystic carcinomas (ACCs) had increased 5-year CSS compared with those with neither SCC nor ACC histology and those with SCC histology (83.4% [76.0%, 90.8%] versus 50.3% [42.5%, 58.1%] and 28.8% [23.2%, 34.4%]; p < 0.001) based on univariate analysis. Despite the improved CSS associated with surgery, 55% of the identified cohort did not undergo surgery, with only 5.5% of these patients having ACC compared with 58% having SCC (p < 0.001). Conclusions Age < 65 years, ACC histology, and treatment with surgery were associated with improved 5-year CSS among patients with PTC, although the significant proportion of this group not receiving surgery represents an opportunity for improved outcomes.

Very late intracranial extraparenchymal solitary metastasis of adenoid cystic carcinoma of the parotid gland: A case report and literature review

Background: Adenoid cystic carcinoma (ACC) of the parotid gland often manifests as distant metastasis long after the initial surgery, resulting in a poor long-term prognosis. The most common sites of metastasis are the lungs, liver, and bones. Single intracranial metastasis is very rare. Case Description: A 43-year-old woman with a history of surgical removal of ACC 24 years prior presented with Gerstmann syndrome and right hemianopsia. Head magnetic resonance imaging revealed a solitary extra-axial tumor in the left occipital region. The tumor was surgically removed and pathologically diagnosed as a metastasis of cribriform-type ACC. Since she had no other systemic metastasis, she did not receive adjuvant treatment and has remained recurrence-free for 35 months postoperatively. Conclusion: Very late intracranial solitary metastasis of ACC is extremely rare. Due to the risk of delayed recurrence in pathologically confirmed cribriform type ACC, long-term follow-up is recommended.

PATH-42. METASTATIC ADENOID CYSTIC CARCINOMA OF BREAST WITH BRAIN METASTASES AND LEPTOMENINGEAL DISEASE

Abstract INTRODUCTION Adenoid cystic carcinoma (ACC) of the breast rarely metastasizes to the brain and leptomeninges. ACC is a rare type of breast cancer, accounting for less than 0.1% of cases. It is usually triple negative, but has a good prognosis when it is confined to the breast. Lymph node involvement is uncommon, occurring in less than 2% of cases, and distant metastases to the brain are extremely rare. Leptomeningeal disease has not been reported yet. Case: In this case, we describe a 57-year-old woman diagnosed with triple-negative ACC of the right breast, which had spread to the axillary lymph nodes and distant metastases to the lung. The diagnosis was confirmed by biopsies of both metastatic sites. The patient received vinorelbine and cisplatin treatment for 11 cycles followed by right needle localized lumpectomy. While awaiting for the next steps in treatment she was admitted to the hospital for altered mental status, saddle anesthesia, and urinary incontinence. Neuraxis MRI revealed numerous hemorrhagic and non-hemorrhagic brain metastasis with diffuse leptomeningeal disease. Cerebrospinal fluid analysis confirmed leptomeningeal disease with positive cytology for atypical epithelioid malignant cells. The patient underwent whole brain radiation and focal radiation treatment to specific areas of leptomeningeal disease. Although she initially improved after the first month of radiation, her condition deteriorated rapidly, preventing the initiation of the next line of treatment with high-dose methotrexate or capecitabine. DISCUSSION Adenoid cystic carcinoma of the breast is exceedingly rare however with a very good overall prognosis. It is uncommon for ACC to spread to regional lymph nodes, and even rarer for distant metastasis. Brain metastasis is also infrequently reported. There are no documented cases of metastatic ACC with leptomeningeal disease, so there is no consensus on the best course of treatment.

Medium-term oncological outcome endoscopic surgery malignant tumors of the nasal cavity and paranasal sinuses

Introduction. Transnasal endoscopic resection of sinonasal tumors is currently used in many clinics around the world. Short- and long-term oncological outcomes primarily depend on tumor histology. However, most currently existing studies are based on a small number of observations due to the rarity and heterogeneity of sinonasal malignancy.Aim. To evaluate short- and medium-term oncological outcomes of treatment of malignant sinonasal tumors. Materials and methods. The study is based on retrospective analysis of 95 patients who underwent endoscopic surgery for sinonasal malignancy between 2014 and 2023 at the N.N. Blokhin National medical Research Center of Oncology. Squamous cell carcinoma was diagnosed in 27 (28.4 %) patients, olfactory neuroblastoma in 13 (13.7 %), mucosal melanoma in 30 (31.6 %), adenocarcinoma in (6.3 %), adenoid cystic carcinoma in 7 (7.3 %), sarcomas in 12 (12.7 %). Results. In the study group, 2-years overall survival and recurrence-free survival were 81.3 and 61.4 % respectively, survival without local recurrence was 87.5 %. The outcomes significantly depended on tumor histology.Conclusion. Oncological outcomes of endoscopic resections of sinonasal tumors significantly depend on tumor histology, but in general they allow to achieve good local control. Small number of observations, variety of histological forms, and short follow-up periods do not allow to accurately evaluate the effectiveness of this approach for each tumor type. further research in large populations is needed to establish risk factors.

Positive results of treatment of adenocystic carcinoma of the parotid salivary gland with lenvatinib

Malignant tumors of the salivary glands account for 3–5 % of all head and neck cancers. Adenocystic carcinomas are malignant neoplasms that most often arise from large and small salivary glands. The disease is characterized by a progressive course with local and distant relapses.The article presents a clinical case of successful treatment with lenvatinib in suboptimal doses of adenocystic carcinoma of the parotid salivary gland.

Stroma-and Tumor-Associated Predictive Features inSalivary Gland Adenoid Cystic Carcinoma of the Headand Neck.

There is lack of knowledge on the utility of prognostic histopathologic characteristics in adenoid cystic carcinoma (ACC) of the head and neck. We evaluated the prognostic value of tumor and stroma-related histopathologic features in ACC. A total of 65 cases of ACC from minor and major salivary glands were included in this study. We evaluated tumor budding, tumor-infiltrating lymphocytes (TILs), and tumor-stroma ratio (TSR) in hematoxylin and eosin (HE) stained sections. Stroma-rich ACCs recurred more frequently (p = 0.029) during follow-up and associated with distant metastasis (p = 0.038). In multivariable analysis, stroma-rich tumors associated with poorer disease-specific survival with a hazard ratio of 3.76 (95% CI 1.10-12.83, p = 0.034). ACCs commonly showed a low infiltration of TILs as 89% of the tumors was characterized by an immune desert pattern. Low infiltration of TILs associated significantly with increased tumor budding (p = 0.039). Adverse features of TSR and tumor budding are widely expressed in ACC, and stroma-rich tumors are associated with poor prognosis. Low number of TILs in ACC tissue indicates a weak immune response by the host and illustrates the nature of ACC as a relentless malignancy.

Targeted RNA Sequencing of Head and Neck Adenoid Cystic Carcinoma Reveals SEC16A::NOTCH1 Fusion and MET Exon 14 Skipping as Potentially Actionable Alterations.

Adenoid cystic carcinoma (AdCC) of the head and neck harbors MYB/MYBL1::NFIB fusions in around 60% of cases, with unfavorable long-term survival due to frequent recurrences and metastases, currently lacking effective targeted therapy. The study aims to identify actionable alterations and to elucidate the molecular underpinnings of MYB/MYBL1::NFIB-negative AdCC using a large targeted RNA sequencing panel. We retrospectively searched our MSK-Solid Fusion clinical sequencing database for head and neck AdCC sequenced between2016and 2023. Of a total of 55 cases, 28 showed MYB::NFIB, 7 showed MYBL1::NFIB, and one case each harbored MYB::MPDZ (case 1) and FUS::MYB (case 2). One base of tongue tumor expressed both MYB::NFIB fusion and MET exon 14 skipping transcripts due to concurrent MET splice site mutation, D1010N (case 3). One parotid tumor lacked MYB/MYBL1 rearrangement but instead showed an in-frame SEC16A::NOTCH1 fusion that preserved the secretase cleavage site (case 4). Clinical records on 4cases with non-canonical sequencing findings were reviewed. Distant metastases were present at the initial diagnosis (case 2) or at recurrence (cases 1, 3, and 4). Disease-related mortality occurred in cases 2 and 4 despite radiotherapy and immunotherapy. The study improved the understanding of AdCC providing the first documentation of tumor clinical behavior associated with MYB::MPDZ and FUS::MYB fusions and reporting potentially actionable SEC16A::NOTCH1 fusion and MET exon 14 skipping mutation. Further research is needed to explore the therapeutic utility of MET inhibition and the efficacy of γ-secretase inhibitors against rare NOTCH1fusions in AdCC.

Primary adenoid cystic carcinoma of the rib with metastases: A rare case report and literature review

Primary adenoid cystic carcinoma of the rib with metastases: A rare case report and literature review

Clinicopathological features of lacrimal gland biopsy lesions in 582 Chinese patients

Background This study was conducted to assess the clinicopathological characteristics of biopsy lesions in the lacrimal gland, in response to evolving pathological technologies and the emergence of new diseases. A retrospective review of case notes was conducted for 582 patients with biopsied lacrimal gland lesions at West China Hospital of Sichuan University in China. This study, spanning from January 1, 2010, to December 31, 2021, recorded demographics, clinical features, imaging findings, histopathological diagnoses, and recurrence rates. In a cohort of 582 patients, the mean age was 47.5 years, with 318 being female. Bilateral disease was present in 127 patients. Common signs and symptoms included a palpable mass, lid swelling, proptosis, and decreased ocular movements or diplopia. The predominant pathologies identified were inflammation, epithelial tumors, and lymphoproliferative lesions. Patients with inflammation (mean 32.8 months) experienced symptoms for a longer period compared to those with lymphoproliferative lesions (mean 22.2 months) (95% confidence interval [CI] 1.53–19.27, P = 0.022). Younger patients tended to have structural lesions (mean 42.5 years) (95%CI 4.74–21.59, P = 0.003), inflammation (mean 42.9 years) (95%CI 0.46–6.57, P<0.001), or epithelial tumors (mean 46.4 years) (95%CI 6.21–12.38, P<0.001), while older patients more frequently had lymphoproliferative lesions (mean 55.7 years). The period from 2016 to 2021 saw a higher diagnosis rate of biopsied lacrimal gland lesions than from 2010 to 2015. During this later period, there was an increase in cases of immunoglobulin G4-related ophthalmic disease (IgG4-ROD) and malignant epithelial tumors, while lymphoid hyperplasia cases declined. Adenoid cystic carcinoma showed the highest recurrence rate, with lesion extent and Ki-67 index identified as independent risk factors in our study. Inflammation, epithelial tumors, and lymphoproliferative lesions were the three primary causes of lacrimal gland lesions in our study group. Among the inflammatory conditions, IgG4-ROD was the most specific. The incidence of lacrimal gland lesions has risen, particularly for IgG4-ROD. Adenoid cystic carcinoma among the lacrimal gland lesions was prone to recurrence, with the extent of the lesion and Ki-67 index serving as independent risk factors.

Development and validation of a nomogram for predicting overall survival of head and neck adenoid cystic carcinoma

This study aimed to develop and validate a nomogram using clinical variables to guide personalized treatment strategies for adenoid cystic carcinoma of the head and neck (ACCHN). Data from 1069 patients with ACCHN diagnosed between 2004 and 2015 in the Surveillance, Epidemiology, and End Results (SEER) database were used to construct the nomogram. External validation was performed using an independent cohort of 70 patients from Fujian Cancer Hospital. Multivariate Cox regression analysis was conducted using IBM SPSS version 26.0 and R Software version 4.2.3. The concordance index (C-index) and receiver operating characteristic (ROC) curves were used to assess the predictive accuracy of the nomogram. Age, tumor site, surgery, N stage, M stage, and TNM stage were identified as independent prognostic factors through univariate and multivariate Cox analyses. The nomogram demonstrated superior predictive performance compared to the TNM staging system, effectively stratifying patients into high-risk and low-risk groups. This nomogram offers a valuable tool for predicting overall survival in patients with ACCHN and tailoring individualized treatment approaches.

Development of a Tumor Growth-responsive Robust Planning Method for Head and Neck Cancer in Carbon-ion Radiotherapy.

The current study aimed to evaluate a treatment planning method that is robust against tumor growth and to assess its effectiveness in particle therapy for head and neck cancer. The proposed method optimizes dose distribution by replacing the relative stopping power ratio (rSPR) of the clinical target volume (CTV) cavity region with a tumor-equivalent rSPR (Condition 1). The optimized initial treatment plan template was then recalculated using in-room CT images acquired in the same treatment position, and the doses to the tumor and organs at risk were compared with those in the initial treatment plan. We evaluated this method in 10 patients with head and neck cancer treated with carbon ion radiotherapy. To evaluate the effectiveness of the proposed method, we compared it to the initial treatment plan without the replacement (Condition 2). CTV V95% reduction relative to that of the initial treatment plan at the end of treatment was 1.3%±2.9% and 2.6%±3.7% for Condition 1 and Condition 2, respectively, with Condition 1 (Replacement condition) providing better CTV coverage. Subgroup analysis showed a higher change in target coverage in the mucosal melanoma than in the adenoid cystic carcinoma, suggesting that this was influenced by the rate of tumor growth. The proposed treatment planning method, which adjusts for tumor growth by modifying the rSPR in the cavity region, improved robustness in carbon ion radiotherapy for head and neck cancer. Condition 1 (with replacement) achieved better CTV coverage than Condition 2 (without replacement), particularly in fast-growing tumors like mucosal melanoma. This method ensures consistent dose delivery to tumors while maintaining safe doses to organs at risk, offering potential for improved treatment outcomes.

Adenoid Cystic Carcinoma of Sinonasal Mass: A Rare Case Report

Adenoid cystic carcinoma (ACC) is a rare tumor, contributing to less than 1% of head and neck malignancies. It involves the salivary gland, nose, and paranasal sinus. Sinonasal malignancies constitute 1–2% of all malignancies. Out of all sinonasal malignancies, sinonasal adenoid cystic carcinoma (SNACC) is the third most common SNACC and is a slow-growing tumor that manifests in later stages. SNACC can extend into intracranial and intradural space, therefore it requires surgical treatment and radiotherapy. The prognosis of SNACC entirely depends on the histological subtype of the tumor and available treatment options. This case report is presented due to its rare histopathological findings.

Circulating Adenoid Cystic Carcinoma associated MYB transcripts enable rapid and sensitive detection of metastatic disease in blood liquid biopsies

Circulating Adenoid Cystic Carcinoma associated MYB transcripts enable rapid and sensitive detection of metastatic disease in blood liquid biopsies

Extrapleural Pneumonectomy for Treatment of Pulmonary Adenoid Cystic Carcinoma

Extrapleural Pneumonectomy for Treatment of Pulmonary Adenoid Cystic Carcinoma

Oncologic Considerations for Primary Facial Reanimation Following Parotid Adenoid Cystic Carcinoma Resection.

Oncologic Considerations for Primary Facial Reanimation Following Parotid Adenoid Cystic Carcinoma Resection.

Integrating Machine Learning with Web-Based Tools for Personalized Prognosis in Oral Adenoid Cystic Carcinoma

BackgroundAdenoid cystic carcinoma (ACC) of the oral cavity is a rare head and neck cancer. This rarity contributes to the paucity of comprehensive research on this cancer thereby complicating the development of evidence-based treatment strategies. This study aims to use machine learning (ML) techniques to analyze survival outcomes and optimize treatment approaches of ACC. MethodsThe SEER database (2000-2020) was used in this study. Cox regression analysis was used to identify the prognostic variables; prognostic models using five ML algorithms were constructed to predict the 5-year survival rates. A validation method incorporating the area under the curve (AUC) of the receiver operating characteristic (ROC) curve was used to validate the accuracy and reliability of ML models. Also, Kaplan-Meier survival analysis was performed. ResultsThis study's sample included 645 patients. The most common primary site for ACC was the hard palate, followed by the cheek mucosa. Survival rates varied across treatment groups, with the highest rates observed in patients who underwent surgery only. ML models revealed that the most significant prognostic factors were age, metastasis, and surgery. ConclusionsThis study contributes evidence and knowledge to the limited literature on ACC and emphasizes the importance of adjuvant radiotherapy. This study highlights that metastasis and age are key prognostic factors. Furthermore, the developed ML-based web tool offers a novel approach for the personalized prognosis of these rare cancer types.

Clinicopathological analysis of hybrid carcinomas of the salivary glands: A systematic review

Hybrid carcinomas (HC) are the association of two or more malignant neoplasms arising within the same topographical area. The present study is a systematic review of HC of the salivary glands. This study aimed to assess HC clinicopathological features and molecular profile. Observational studies, case series, and case reports were included. PubMed, Scopus, EMBASE, Lilacs, Web of Science, and gray literature were searched until June 2024. A total of 18 articles including 34 patients were included. HC of the salivary glands is presented as a painless mass affecting mainly the parotid gland of adults with a preference for male patients. Histologically, adenoid cystic carcinoma and epithelial-myoepithelial carcinoma were the most prevalent association, being surgical excision with radiotherapy the most common treatment. It's seen that the most aggressive component might guide the patient's treatment and prognosis. Further molecular studies are necessary to determine if HC are distinct entities or biologically identical to the individual neoplasms. In conclusion, this systematic review may contribute to a better understanding of this rare lesion, their biological behavior, treatments employed, and the outcome of these patients.

Postradiotherapy Response Assessment Using 18F-FDG PET/CT in Salivary Gland Carcinoma-A Multicenter Study.

This multicenter study investigates the efficacy of 18F-FDG PET/CT in postradiotherapy (post-RT) response evaluation in salivary gland carcinoma (SGC). We retrospectively reviewed 115 SGC patients who underwent definitive or adjuvant RT followed by 18F-FDG PET/CT between 2004 and 2021. Most tumors were parotid gland malignancies (50%). The most common histological subtypes were adenoid cystic (29%) and mucoepidermoid carcinomas (18%). The median follow-up was 65 months. Post-RT anatomic images (CT/MRI) revealed complete response (CR) in 51 patients (44%). Among 53 patients with partial response or stable disease, only 17 (32%) patients experienced locoregional recurrence, with a 5-year locoregional control rate of 69%. Post-RT 18F-FDG PET/CT documented metabolic CR in 81 patients (70%). Metabolic complete responders had significantly higher 5-year locoregional control (90% vs 43%), distant metastasis-free survival (80% vs 48%), progression-free survival (76% vs 24%), and overall survival rates (89% vs 42%) compared with non-complete responders (all P < 0.001), as confirmed in both univariate and multivariate analyses. It identified additional viable tumors in 18 cases (16%) and facilitated salvage local therapies in 7 patients (6%). The sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of post-RT 18F-FDG PET/CT were 63%, 91%, 70%, 88%, and 84%, respectively, in predicting locoregional recurrence. 18F-FDG PET/CT showed significantly higher sensitivity (88% vs 36%, P = 0.011) in tumors with pre-RT SUVmax ≥7.39 compared with those with SUVmax <7.39. Post-RT 18F-FDG PET/CT demonstrates high negative predictive value and specificity, with metabolic CR predicting excellent outcomes. Additionally, it exhibits higher sensitivity for high-SUVmax SGC, facilitating early detection of viable tumors.

Metastatic Adenoid Cystic Carcinoma at the Base of the Tongue and Duplicate Breast Cancer Diagnosed During Restaging.

Adenoid cystic carcinoma (AdCC) is a rare malignant tumor that primarily affects the salivary glands but can also occur in other organs. Low incidence and unpredictable clinical behavior make AdCC one of the most difficult head and neck tumors to treat. We present the case of a 54-year-old woman with AdCC localized at the base of the tongue, following radical surgical and oncological therapy. Due to advances in palliative oncological treatment, there is a more than five-year survival period before the progression of metastatic disease. Considering the rare occurrence of this disease, a literature search was also conducted, and therapy options are discussed. Ensuring a sufficient extent of the surgical procedure is still a challenge, and most specialists agree that subsequent postoperative radiotherapy reduces the risk of local recurrence. The effective dose of radiotherapy to the area of the primary tumor and lymph nodes is not clearly defined. The distinct biological behavior of AdCC results in varying sensitivity to chemotherapy or radiotherapy compared to treatments commonly used for head and neck squamous cell carcinomas. Treatment recommendations for these rarer tumors are based mainly on case reports and small clinical trials. The acquired therapeutic experience can contribute to prolonging the survival period of patients and improving their prognosis and quality of life.

Survival benefit added by adjuvant chemotherapy in adenoid cystic carcinoma of salivary gland

The aim of this study was to investigate the potential survival advantages associated with chemoradiotherapy (CRT) compared to radiotherapy (RT) as standalone modalities in the treatment of adenoid cystic carcinoma (ACC) of the salivary glands. Patients diagnosed with resected salivary gland ACC were retrospectively enrolled and categorized into two groups based on the type of adjuvant therapy received. The overall survival outcomes between the CRT and RT cohorts were evaluated using a multivariable Cox model. Post propensity score-matching, a total of 114 patients (57 in each treatment group) were included in the analysis. In the general patient population, CRT did not confer an additional survival benefit compared to RT alone. High-grade tumors, positive surgical margins, and the presence of five or more positive lymph nodes were identified as independent prognostic factors associated with poorer overall survival. Specifically, for patients with positive surgical margins, CRT was significantly associated with improved overall survival relative to RT, displaying a hazard ratio of 0.93 (95% CI: 0.81–0.99). Furthermore, in patients with more than four metastatic lymph nodes, CRT significantly reduced the risk of mortality by 6% (95% CI: 1-24%) when compared to RT alone. Conversely, in patients with high-grade tumors, the addition of adjuvant chemotherapy to RT did not yield significant alterations in survival outcomes compared to RT alone (p = 0.437, HR: 0.95, 95% CI: 0.75–2.07). CRT may offer an overall survival benefit for patients with salivary gland ACC, particularly those characterized by positive margin or the presence of five or more metastatic lymph nodes.