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Background: Due to the relative rarity of malignant sublingual gland tumors, diagnosing and treating them clinically pose challenges. Hence, there's a need to explore the pathological types, characteristics, treatment methods, and prognosis of primary malignant tumors of the sublingual gland to improve our understanding and management of these rare yet highly malignant conditions. Methods: This study reviewed cases of primary malignant sublingual gland tumors, analyzing their characteristics. The treatment methods included surgical excision, with additional radiotherapy, or brachytherapy for advanced stages or positive surgical margins. The study also summarized different treatment approaches, including lymph node dissection and soft tissue reconstruction using free flaps such as the anterolateral thigh flap and forearm flap. Results: We have gathered 23 cases of sublingual gland malignancies treated at the Department of Oral and Maxillofacial Surgery, School and Hospital of Stomatology, Wuhan University, from January 2013 to May 2024. The most common pathological types were adenoid cystic carcinoma and mucoepidermoid carcinoma, with rare cases of mucosa-associated lymphoid tissue (MALT) lymphoma and nonspecific salivary gland clear cell carcinoma. Early diagnosis and surgical intervention were crucial for a favorable prognosis. Marginal mandibulectomy was necessary for cases involving the mandible. Patients with positive preoperative lymph node detection required cervical lymph node dissection. Extensive tissue defects in the floor of the mouth were effectively reconstructed with free flaps to prevent oral-mandibular fistula. Conclusion: Surgical excision remains the preferred treatment for malignant sublingual gland tumors. Early diagnosis and comprehensive surgical management are essential for improving prognosis. The study's limitations include a small sample size and short follow-up duration, necessitating further research with larger clinical samples to confirm these findings. [ABSTRACT FROM AUTHOR]

Objectives: We explored Trichorhinophalangeal syndrome type 1 (TRPS1) expression in special types of breast carcinoma, and analyzed the correlation between TRPS1 and androgen receptor (AR) expression in triple-negative breast cancer (TNBC). Methods: TRPS1 expression was analyzed in 801 patients with special types of breast carcinoma. A total of 969 TNBC were used to analyze the correlation between the expression of TRPS1 and AR. TRPS1 expression was evaluated in 1975 cases of breast cancer with different molecular types. Results: A total of 801 special types of breast cancers were stained with TRPS1.TRPS1 was positive in 100% (63/63) of mucinous carcinoma, 100% (7/7) adenoid cystic carcinomas (4 classic adenoid cystic carcinomas and 3 solid-basaloid adenoid cystic carcinomas), 100% (4/4) tubular carcinomas, 100% (2/2) secretory carcinomas, and 99.59% (243/244) invasive lobular carcinomas, 99.26% (267/269) invasive micropapillary carcinomas, 97.44% (38/39) ER-positive neuroendocrine tumors, 94.44% (34/36) metaplastic breast carcinomas (MBCs), 63.73% (65/102) apocrine carcinomas. TRPS1 was negative in all triple-negative neuroendocrine carcinomas (0/7).TRPS1 was positive in 92.86% (26/28) of metastatic special types of breast cancer. TRPS1 and AR expression were analyzed in 969 cases of TNBC. 90.40% were positive for TRPS1, and 42.41% were positive for AR. A significant inverse correlation between TRPS1 and AR expression was shown in TNBC (p <.001). TRPS1 showed a higher positive rate (93.13%) in TNBC compared to GATA binding protein 3 (GATA3), gross cystic disease fluid protein 15 (GCDFP-15) and forkhead box transcription Factor C 1 (FOXC1). Conclusions: In conclusion, our study demonstrated that TRPS1 is a highly sensitive marker for most special types of breast carcinoma. TRPS1 was positive in 63.73% of apocrine carcinomas. TRPS1 and AR expression was inversely correlated in TNBC. [ABSTRACT FROM AUTHOR]

Aim Materials and methods Results Conclusion Although immune checkpoint inhibitors (ICPi) for salivary gland cancer (SGC) have been investigated in clinical trials, details of the tumor immune microenvironment (TIME) remain unclear. This research aimed to elucidate the TIME of SGC and its relationship with tumor mutation burden (TMB) and to explore the rationale for the applicability of ICPi.We selected five pathological types, namely adenoid cystic carcinoma (ACC); adenocarcinoma, not otherwise specified (ANOS); salivary duct carcinoma (SDC); and low/high‐grade mucoepidermoid carcinoma (MEClow/high). We investigated the TIME and TMB of each pathological type. TIME was evaluated by multiplexed fluorescent immunohistochemistry. TMB was measured by next‐generation sequencing.ACC and MEChigh showed the lowest and highest infiltration of immune effector and suppressor cells in both tumor and stroma. ANOS, SDC, and MEClow showed modest infiltration of immune effector cells in tumors. Correlation analysis showed a positive correlation between CD3+CD8+ T cells in tumor and TMB (r = 0.647). CD3+CD8+ T cells in tumors showed a positive correlation with programmed cell death‐ligand 1 expression in tumor cells (r = 0.513) and a weak positive correlation with CD3+CD4+Foxp3+ cells in tumors (r = 0.399). However, no correlation was observed between CD3+CD8+ T cells and CD204+ cells in tumors (r = ‐0.049).The TIME of ACC was the so‐called immune desert type, which may explain the mechanisms of the poor response to ICPi in previous clinical trials. On the other hand, MEChigh was the immune‐inflamed type, and this may support the rationale of ICPi for this pathological subtype. [ABSTRACT FROM AUTHOR]

Objective: To determine the relative frequency of orbital lesions based on the site of origin and histopathology at a Tertiary care hospital (Mayo Hospital, Lahore Pakistan) from 1996 till 2022 (27 years). Methods: This descriptive case series included 2651 patients of all age groups presenting with orbital lesions who initially got enrolled at Institute of Ophthalmology Mayo Hospital, Lahore from 1996 till 2022. Of these, 583 patients left against medical advice. So, clinical data of 2068 patients were completely analyzed. Lesions were managed medically and/ or surgically. Final clinical diagnosis, with the help of histopathology, was used to classify the lesions. Results: There were 1258 (60.9%) adults and 810 (39.1%) children, 1358 (65.66%) were neoplastic while 710 (34.33%) non-neoplastic lesions. Amongst the neoplastic lesions, 405 (29.8 %) were benign and 953 (70.2%) malignant. Primary orbital lesions were 1676 (81.04%), Secondary orbital lesions were 300 (14.51%), Endocrine/ hematopoietic reticuloendothelial system lesions were 84 (4.06%) and Metastatic lesions from distant organs were 08 (0.39%). Conclusion: Retinoblastoma, rhabdomyosarcoma, optic nerve gliomata were common in children. Pleomorphic adenoma & adenocystic carcinoma of lacrimal gland, cavernous hemangioma, optic nerve meningioma, neurofibroma, schwannoma, squamous cell carcinoma of eyelid, carcinoma of maxillary antrum and lymphomas were more common in adults. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Adenoid cystic carcinoma (ACC) is a malignant tumor primarily originating from the salivary glands, capable of affecting multiple organs. Although ACC typically exhibits slow growth, it is notorious for its propensity for neural invasion, local recurrence, and distant metastasis, making it a particularly challenging cancer to treat. The complexity of ACC's histological and molecular features poses significant challenges to current treatment modalities, which often show limited effectiveness. Recent advancements in single‐cell RNA‐sequencing (scRNA‐seq) have begun to unravel unprecedented insights into the heterogeneity and subpopulation diversity within ACC, revealing distinct cellular phenotypes and origins. This review delves into the intricate pathological and molecular characteristics of ACC, focusing on recent therapeutic advancements. We particularly emphasize the insights gained from scRNA‐seq studies that shed light on the cellular landscape of ACC, underscoring its heterogeneity and pathobiology. Moreover, by integrating analyses from public databases, this review proposes novel perspectives for advancing treatment strategies in ACC. This review contributes to the academic understanding of ACC by proposing novel therapeutic approaches informed by cutting‐edge molecular insights, paving the way for more effective, personalized therapeutic approaches for this challenging malignancy. [ABSTRACT FROM AUTHOR]

Comprehensive information on genetic alterations in salivary gland cancer (SGC) is limited. This study aimed to elucidate the genetic and clinical characteristics of patients with SGC using the Center for Cancer Genomics and Advanced Therapeutics (C‐CAT) database, a Japanese national genomic database. We analyzed data of 776 patients with SGC registered in the C‐CAT database between June 1, 2019, and June 30, 2023. Adenoid cystic carcinoma was the most common histologic type, followed by salivary duct carcinoma (SDC) and adenocarcinoma not otherwise specified. Genetic data of 681 patients receiving FoundationOne® CDx were analyzed. We identified specific features of the combination of TP53 and CDKN2A alterations among the histological types. Specific LYN amplification was mainly detected in carcinoma ex pleomorphic adenoma and myoepithelial carcinoma. For SDC, the frequency of ERBB2 and BRAF alterations were higher in cases with metastatic lesions than in those with primary lesions. Although 28.6% patients were offered recommended treatment options, only 6.8% received the recommended treatments. This study highlights the differences in genetic alterations among the histological types of SGC, with comprehensive genomic profiling tests revealing lower drug accessibility. These findings could contribute to the development of personalized treatment for patients with SGC. [ABSTRACT FROM AUTHOR]

The article focuses on a 59-year-old woman with a longstanding breast lesion and recent neurological symptoms, who was referred to a multidisciplinary breast oncology clinic. Topics include the diagnosis of acute infarction, positive SARS-CoV-2 screening, and imaging findings of a large breast mass with potential metastases and evidence of ischemic stroke.

Simple Summary: Adenoid cystic carcinoma is a rare but devastating disease. Currently, treatment options are very poor, and ACC does not respond well to conventional chemotherapy or radiation therapy and has a high rate of recurrence or metastasis. The molecular drivers that govern disease pathology are currently poorly understood. We conducted a molecular analysis of adenoid cystic carcinoma of the salivary and lacrimal glands in order to better understand these mechanisms and pave the way for the development of future therapeutics. Adenoid cystic carcinoma (ACC) of head and neck origin is associated with slow but relentless progression and systemic metastasis, resulting in poor long-term survival rates. ACC does not respond to conventional chemotherapy. Determination of molecular drivers may provide a rational basis for personalized therapy. Herein, we investigate the clinical and detailed molecular genomic features of a cohort of patients treated in Ireland and correlate the site of origin, molecular features, and outcomes. Clinical and genomic landscapes of all patients diagnosed with ACC over a twenty-year period (2002–2022) in a single unit in Ireland were examined and analyzed using fluorescence in situ hybridization, DNA sequencing, and bioinformatic analysis. Fourteen patients were included for analysis. Eleven patients had primary salivary gland ACC and three primary lacrimal gland ACC; 76.9% of the analyzed tumors displayed evidence of NFIB-MYB rearrangement at the 6q23.3 locus; 35% had mutations in NOTCH pathway genes; 7% of patients had a NOTCH1 mutation, 14.3% NOTCH2 mutation, and 14.3% NOTCH3 mutation. The presence of epigenetic modifications in ACC patients significantly correlated with worse overall survival. Our study identifies genetic mutations and signaling pathways that drive ACC pathogenesis, representing potential molecular and therapeutic targets. [ABSTRACT FROM AUTHOR]

Adenoid cystic carcinoma (AdCC) is a salivary gland neoplasm that infrequently appears in the sinonasal region. The aim of this study was to evaluate the outcome and clinicopathological parameters of sinonasal AdCC. A retrospective analysis was conducted on all cases of AdCC affecting the nasal cavity or paranasal sinuses between 2000 and 2018 at the University Hospital Zurich. Tumor material was examined for morphological features and analyzed for molecular alterations. A total of 14 patients were included. Mean age at presentation was 57.7 years. Sequencing revealed MYB::NFIB gene fusion in 11/12 analyzable cases. Poor prognostic factors were solid variant (p < 0.001), histopathological high-grade transformation (p < 0.001), and tumor involvement of the sphenoid sinus (p = 0.02). The median recurrence-free survival (RFS) and OS were 5.2 years and 11.3 years. The RFS rates at 1-, 5-, and 10-year were 100%, 53.8%, and 23.1%. The OS rates at 1-, 5-, and 10- years were 100%, 91.7%, and 62.9%, respectively. In Conclusion, the solid variant (solid portion > 30%), high-grade transformation, and sphenoid sinus involvement are negative prognostic factors for sinonasal AdCC. A high prevalence of MYB::NFIB gene fusion may help to correctly classify diagnostically challenging (e.g. metatypical) cases. [ABSTRACT FROM AUTHOR]

Herein, we report an unusual presentation of a 38-year-old heathy lady with a painless slowly growing lesion in the volar aspect of her left distal forearm reaching the thenar area for >15 years. Magnetic resonance imaging of the lesion showed multilobulated soft tissue mass with low and high signal in T1- and T2-weighted images with homogenous contrast enhancement. Additionally, a stable serpiginous bony lesion was noted in the first metacarpal bone showing low T1 and high T2 signals with peripheral enhancement. Histopathological examination was consistent with cylindroma and enchondroma, respectively. The patient had full recovery with no recurrence at a 1-year follow-up visit. While enchondroma is the most common benign tumor of the hand, sporadic dermal cylindroma, in contrast, rarely affects the upper extremity. The coexistence of these two pathological entities was not previously reported and it may complicate the diagnosis. [ABSTRACT FROM AUTHOR]

This article presents several cases of rare tumors and their clinical and pathological characteristics. The first case discusses a highly aggressive tumor called sinonasal teratocarcinosarcoma, which can be difficult to diagnose due to its morphological heterogeneity. The second case focuses on oncocytic lesions in the salivary glands, highlighting the challenges in distinguishing them from other conditions. The third case examines papillary thyroid carcinoma arising within a branchial cleft cyst, emphasizing the need for further investigation. The fourth case explores an unusual presentation of hepatocellular carcinoma as an intraoral mass. Additionally, the article discusses IgG4-related disease in the head and neck region, emphasizing the importance of accurate diagnosis based on histological features, radiology, and serum IgG4 levels. [Extracted from the article]

Adenoid cystic carcinoma (ACC) is an uncommon malignant neoplasm that predominantly arises from major and minor salivary glands, accounting for about 1% of head and neck malignancies. ACCs originating from the pterygopalatine fossa (PPF) are extremely rare. In this case report, we present a 77-year-old male patient who experienced a two-year hemi-facial numbness and mild trismus, with intact nasal mucosa. He underwent Computed Tomography (CT) and Magnetic Resonance Imaging (MRI) which revealed opacification of the left PPF with extension to the left infratemporal fossa and osteolytic areas of the sphenoid bone. Histopathology, following blind biopsies, revealed ACC of the PPF, which was subsequently treated with combined therapy (radiotherapy and chemotherapy). Due to their slow and progressive growth, as well as their tendency for perineural invasion, ACCs should always be considered in the differential diagnosis of painless swelling or nerve dysfunction. To our knowledge, this case is the fifth described in the English literature. [ABSTRACT FROM AUTHOR]

Adamantinomatous craniopharyngioma is a grade 1 tumor that arises in a sellar/suprasellar location. Despite being a grade 1 tumor, there is high recurrence and endocrinal insufficiency. Malignancy arising in craniopharyngioma is extremely rare, has a dismal prognosis, and is currently not included as a separate entity in the World Health Organization Classification of Central Nervous System 5th edition. Here we describe a case of adamantinomatous craniopharyngioma and its malignant counterpart. The malignant part had unique histomorphology and basaloid cells with pseudoglandular architecture and a myxoid background. It bore a striking resemblance to adenoid cystic carcinoma. Both the benign and malignant counterparts were beta‐catenin and SOX‐2 positive, providing proof of the malignant part arising from the benign part. Tumors like squamous cell carcinoma and odontogenic ghost cell carcinoma have been described in cranipharyngioma. This case study is the first to describe this unique morphology of adenoid cystic carcinoma‐like features. The possibility of adenoid cystic carcinoma was excluded by immunohistochemistry. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Purpose: Maxillary sinus carcinomas usually present as a locally advanced disease at the time of diagnosis and it is extremely unusual to have a second primary maxillary carcinoma on the contralateral side after many years of completion of treatment of the first malignancy. We present here a case report of a sphenopalatine artery (SPA) pseudoaneurysm mimicking the second primary maxillary carcinoma. Methods: We reviewed the literature for SPA pseudoaneurysm. Results/case report: This report describes the case of a 90-year-old man with a background of adenoid cystic carcinoma of the right maxillary sinus, diagnosed and treated with surgery and radiotherapy 14 years ago, who presented with a history of multiple episodes of epistaxis. The radiological evaluation showed a heterogeneously enhancing mass with a central hemorrhagic component and surrounding bony erosions in the left maxillary sinus and the patient was planned for biopsy from the suspicious mass along with SPA ligation. However, on opening the maxillary antrum there was excessive bleeding and it was determined unsafe to proceed further. The patient was subsequently taken to interventional radiology for diagnostic angiography which revealed an SPA pseudoaneurysm that was subsequently embolized successfully. Conclusions: Sphenopalatine artery pseudoaneurysms should be considered as a differential for recurrent epistaxis in patients with a history of sinonasal malignancy. In such cases, endovascular embolization is a viable management option. [ABSTRACT FROM AUTHOR]

Background: The preoperative diagnosis of salivary gland cancer (SGC) is crucial for the application of appropriate treatment, particularly involving the extension of the resection. Methods: 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. Results: 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 65 years, the hazard ratio of 7.955 and 6.486, respectively. Conclusions: 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. [ABSTRACT FROM AUTHOR]

• Bartholin gland carcinomas are rare, accounting for 5.8% of all vulvar cancers. • Misdiagnosis leads to significant diagnostic delays of up to 19 months. • Surgery was the preferred treatment modality for early-stage Bartholin gland carcinoma. • Primary chemoradiation ± surgical nodal assessment was favoured for stage II/III tumors. • Five-year recurrence free and overall survival were 76% and 64%, respectively. To evaluate the management and outcomes of Bartholin gland cancer at a single tertiary institution. A single institution retrospective review of 9 cases of BGC between 2004 and 2022 was conducted. Demographics, pathological characteristics, treatment, follow up and oncologic outcomes were extracted from clinical records. Data are summarised using descriptive statistics and survival probabilities are presented with Kaplan Meier graphs. Ten cases of BGC were identified at our institution over a period of 18 years. Nine out of ten clinical records were available for analysis. Eight patients presented with vulval swelling and four were treated initially for Bartholin cyst or abscess. One patient had a histological diagnosis of adenoid cystic carcinoma while the remaining were squamous cell carcinomas. With the exception of stage I disease chemoradiation was the primary mode of treatment. Adverse events included skin desquamation (4/9), venous thrombo-embolism (2/9), gastro-intestinal (1/9) and neurotoxicity (1/9). Median follow up was 60 months with a 5-year recurrence free and overall survival at 76 % and 64 % respectively. BGC may present after a long duration of symptoms and at advanced stages. Primary chemoradiation appears to be a feasible treatment option in advanced disease with the benefit of decreased morbidity. [ABSTRACT FROM AUTHOR]

While several studies reported epidermal growth factor receptor (EGFR) expression in salivary gland cancer (SGC), results varied due to a lack of unified definition of EGFR positivity. In this study, we assessed the EGFR expression level using both EGFR positive score and cumulative EGFR score in the patients with SGC. Between January 2010 and April 2021, 102 patients with SGC who underwent surgical resection were reviewed retrospectively by immunohistochemistry. The membrane staining intensity was scored as follows: no staining (0), weak staining (1+), intermediate staining (2+), and strong staining (3+). The cumulative EGFR score was determined on a continuous scale of 0–300 using the formula:1 × (1+: percentage of weakly stained cells) + 2 × (2+: percentage of moderately stained cells) + 3 × (3+: percentage of strongly stained cells). EGFR expression in SGC varied widely even among the same as well as different histopathological types. The average EGFR positive scores were 46.0 %, 55.7 %, 51.6 %, 1.0 %, 26.8 %, 50 %, and 76.8 % for mucoepidermoid carcinoma (MEC), salivary duct carcinoma (SDC), adenoid cystic carcinoma (AdCC), acinic cell carcinoma (AcCC), adenocarcinoma NOS (ACNOS), carcinoma ex pleomorphic adenoma (CAexPA), and squamous cell carcinoma (SqCC), respectively. The average cumulative EGFR scores were 82, 91, 80, 1, 52, 93, and 185 for MEC, SDC, AdCC, AcCC, ACNOS, CAexPA, and SqCC, respectively. EGFR positive scores and cumulative EGFR scores in SGCs varied among the various histological types, and even in the same histological type. These scores may predict the clinical outcome of SGC treated with EGFR-targeting therapies, such as head and neck photoimmunotherapy, and need to be evaluated in future studies. [ABSTRACT FROM AUTHOR]

This document is a correction notice for an article titled "Expression and inactivation of glycogen synthase kinase 3 alpha/beta and their association with the expression of cyclin D1 and p53 in oral squamous cell carcinoma progression." The authors of the original article discovered that an incorrect representative image was used and have provided a corrected version. The correction does not change the conclusions of the paper. The article discusses the expression of GSK3α and GSK3β proteins in various types of oral tumor tissue samples and their association with oral cancers. The corrected image shows that GSK3β is significantly overexpressed in squamous cell carcinoma compared to other types of oral cancers. The publisher remains neutral regarding jurisdictional claims and institutional affiliations. [Extracted from the article]

This article explores the use of 18F-choline-PET/CT imaging for non-FDG-avid salivary gland cancer. Salivary gland tumors are rare and have diverse characteristics. While cross-sectional imaging with contrast-enhanced CT or MRI is the standard for initial staging, FDG-PET has also been used. However, some tumors do not show high FDG uptake, so the study aimed to assess the potential of the non-FDG tracer 18F-choline. The study included five patients with malignant salivary gland tumors and found promising results. Choline-PET was found to be somewhat superior to FDG-PET for salivary gland cancer, especially for low-grade tumors and adenoid cystic carcinoma. However, the high background uptake of choline affected the evaluation of the primary tumor. The authors concluded that more specific radiopharmaceuticals are needed for certain types of primary salivary gland carcinoma. [Extracted from the article]

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. [ABSTRACT FROM AUTHOR]

Objective: Rare disease Background: Immune checkpoint inhibitors (ICIs) have been linked to various immune-related adverse events, including pneumonitis, necessitating early recognition and potential treatment discontinuation. Acute eosinophilic pneumonia (AEP) induced by ICIs, particularly with no reported cases involving anti-TIGIT therapy, is rare. This report describes a case of AEP following treatment with pembrolizumab and anti-TIGIT therapy. Case Report: A 46-year-old woman with lung adenoid cystic carcinoma and chronic hypoxemic respiratory failure on longterm oxygen therapy presented with fever, cough, and shortness of breath. She underwent left pneumonectomy and radiation therapy at diagnosis 9 years earlier. She was participating in a clinical trial using pembrolizumab and anti-TIGIT EOS-448, due to cancer progression. After starting therapy, she developed stable peripheral eosinophilia and a skin rash, suggestive of a drug reaction. On admission, she was in acute-on-chronic hypoxemic respiratory failure, febrile, with an elevated eosinophil count and new multifocal infiltrates in the right lung. Despite broad antibiotics coverage for pneumonia, she developed worsening respiratory symptoms and eosinophilia. She was then empirically started on intravenous methylprednisolone for acute eosinophilic pneumonia without confirmatory bronchoscopy as she was at high risk with her previous pneumonectomy. She subsequently had rapid improvement in her symptoms. Conclusions: AEP should be considered in patients treated with ICIs who develop immune-related adverse effects. Although bronchoscopy findings are part of AEP's diagnostic criteria, this case underscores the importance of clinical judgment in the prompt initiation of steroids, even without confirmatory bronchoscopy, in rapidly progressing cases. The role of anti-TIGIT therapy in this context remains uncertain. [ABSTRACT FROM AUTHOR]

Introduction Adenoidectomy is one of the most common procedures performed by otolaryngologists. Traditional adenoid curettage is performed blindly, which can result in inadequate removal of the adenoid and injury to the surrounding structures. Objective To perform transnasal endoscopic examinations to assess the nasopharynx after conventional curettage adenoidectomy. Methods The present prospective study included 100 children with a mean age of 4.2 ± 3.07 years. It is composed of two steps: conventional curettage adenoidectomy by a resident trainee; and endoscopic evaluation of the nasopharynx through a 0° telescope to assess adenoidal remnants, injury to the surgical field or adjacent structures, and bleeding points. Results Adenoid remnants were observed in 42% of the cases after conventional adenoid curettage in multiple locations, such as the roof of the nasopharynx over the choana (24%), the tubal tonsil (12%), the posterior pharyngeal wall (4%), and the posterior end of the nasal septum (2%). Injury to the surgical field and adjacent structures was observed in 46% of the cases (posterior pharyngeal wall: 23%; lateral pharyngeal wall: 11%; Passavant ridge: 10%; and the Eustachian tube orifice: 2%). Endoscopic bleeding was observed in 29% of the cases; 13% of the cases were from adenoid remnants, 10%, from the mucosa, and 6%, from the pharyngeal muscles. Bleeding was mild in 19% of the cases, moderate in 9%, and severe in 1%. Conclusion Endoscopic evaluation of the nasopharynx following conventional adenoid curettage provides important data regarding adenoid remnants, injury to the surgical field or nearby structures, and bleeding points, which aids in the provision of optimal care and in the achievement of a better outcome. [ABSTRACT FROM AUTHOR]

Introduction: Leukotrienes play a significant role in the pathogenesis of adenoid hypertrophy (A.H.). Therefore, we aimed to analyse the role of montelukast, a leukotriene receptor antagonist, alone or in combination with mometasone, a potent local intranasal steroid, for the treatment of A.H. Methods: Participants were children with A.H. were treated with montelukast alone or montelukast and mometasone furoate. The main outcome measures were effect of montelukast on clinical symptoms of A.H. A literature review was conducted using online search engines, Cochrane Library, PubMed, Web of Science and Scopus, for randomized clinical trials assessing children with A.H. treated with montelukast alone or montelukast and mometasone furoate. Seven randomized clinical trials (RCTs) were included with 742 children. Results: Our study reveals that montelukast alone or in combination with intranasal mometasone furoate significantly improves clinical symptoms of adenoid hypertrophy such as snoring, sleeping disturbance, mouth breathing and A/N ratio. Montelukast was superior to placebo in decreasing snoring (SMD = −1.00, 95% CI [−1.52, −0.49]), sleep discomfort (SMD = −1.26, 95% CI [−1.60, −0.93]), A/N ratio (MD = −0.11, 95% CI [−0.14, −0.09]) and mouth breathing (SMD = −1.36, 95% CI [−1.70, −1.02]). No difference was detected between montelukast and mometasone versus mometasone alone in snoring (SMD = −0.21, 95%CI [−0.69, 0.27]); however, the combination group was superior to the mometasone alone in mouth breathing (SMD = −0.46, 95% CI [−0.73, −0.19]). Conclusions: The limitation of studies included a small sample size, with an overall low to medium quality. Thus, further larger, higher‐quality RCTs are recommended to provide more substantial evidence. [ABSTRACT FROM AUTHOR]

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. 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. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Adenoid cystic carcinoma (ACC) is an uncommon malignancy of head and neck. Although the cornerstone of treatment is surgery, concurrent chemoradiotherapy (CRT) might be used as an effective treatment for unresectable tumors. Herein we report a case of massive ACC of base of tongue with durable complete response to definitive CRT. Adenoid cystic carcinoma (ACC) is a rare tumor accounting for 1% of all head and neck cancers. The best treatment option is complete surgical resection with or without adjuvant radiotherapy. When surgical resection is not feasible, definitive radiotherapy with or without concurrent chemotherapy can be considered. Herein we report a non‐smoker 72‐year‐old woman presented with throat discomfort and sensation of a lump. Evaluation revealed an unresectable adenoid cystic carcinoma of the base of tongue in whom complete clinical response was achieved after definitive concurrent chemoradiation. Although the cornerstone of treatment is complete surgical resection, this case report indicates that concurrent chemoradiotherapy might result in complete clinical response and could be used as a definitive treatment in selected ACC tumors. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Necrotising sialometaplasia (NSM) is a non‐neoplastic lesion mainly arising in the minor salivary glands of the oral cavity. In the clinical features, NSM shows swelling with or without ulceration, and can mimic a malignant disease such as squamous cell carcinoma. Histopathologically, NSM usually shows the lobular architecture that is observed in the salivary glands. Additionally, acinar infarction and squamous metaplasia of salivary ducts and acini are observable. The aetiology of this lesion remains unknown, although it has a characteristic feature that sometimes requires clinical and histopathological differentiation from malignancy. In this study, we investigated upregulated genes in NSM compared with normal salivary glands, and focused on the TGF‐β3 (TGFB3) gene. The results of the histopathological studies clarified that fibroblasts surrounding the lesion express TGF‐β3. Moreover, in vitro studies using mouse salivary gland organoids revealed that TGF‐β3 suppressed salivary gland cell proliferation and induced squamous metaplasia. We demonstrated a possible aetiology of NSM by concluding that increased TGF‐β3 expression during wound healing or tissue regeneration played a critical role in cell proliferation and metaplasia. © 2024 The Pathological Society of Great Britain and Ireland. [ABSTRACT FROM AUTHOR]

Adenoid cystic carcinoma of minor salivary glands (AdCCmSG) represents a 'rarity in the rarity,' posing a clinical challenge in lack of standardized, evidence-based recommendations. At present, AdCCmSG management is mostly translated from major salivary gland cancers (MSGCs). Ideally, AdCCmSG diagnostic-therapeutic workup should be discussed and carried out within a multidisciplinary, high-expertise setting, including pathologists, surgeons, radiation oncologists and medical oncologists. The present review provides an overview of epidemiology and pathologic classification. Moreover, the most recent, clinically relevant updates in the treatment of AdCCmSG (Pubmed searches, specific guidelines) are critically discussed, aiming to a better understanding of this rare pathologic entity, potentially optimizing the care process, and offering a starting point for reflection on future therapeutic developments. The management of rare cancers is often hindered by limited data and clinical trials, lack of evidence-based guidelines, and hardly represented disease heterogeneity, which cannot be successfully tackled with a 'one-size-fits-all' approach. Our goal is to address these potential pitfalls, providing an easy-to-use, updated, multidisciplinary collection of expert opinions concerning AdCCmSG management as of today's clinical practice. We will also cover the most promising future perspectives, based on the potential therapeutic targets highlighted within AdCCmSG's molecular background. [ABSTRACT FROM AUTHOR]

Adenoid cystic carcinoma is a rare malignant tumor that primarily occurs in the salivary glands. There are few reports of sublingual gland adenoid cystic carcinoma with lung metastases on which 18F-fluorodeoxyglucose positron emission tomography/computed tomography (18F-FDG-PET/CT) was performed. We report the case of a 57-year-old Japanese woman with an adenoid cystic carcinoma of the sublingual gland with lung metastases in whom the FDG uptake of the lung metastasis was low despite high FDG uptake in the primary lesion. The pathological examination revealed that solid components were more visible and the Ki-67 index was more positive in the primary lesion compared to the metastatic lesion. We speculate that differences in tumor growth ability might have resulted in the differences in FDG uptake. This case demonstrates that significant differences might occur in the FDG uptake between primary and metastatic tumors.

Cervical tracheal adenoid cystic carcinoma (TACC) invading the thyroid gland is very rare and easily misdiagnosed as thyroid tumor, this paper reports a TACC invading thyroid in a 33-year-old woman who had been diagnosed as a thyroid follicular tumor in right lobe of thyroid by sonography and ultrasound guided fine needle aspiration in other hospital. She accepted surgical treatment in our hospital and was diagnosed as TACC by pathology, locally involving the thyroid. This paper presents the patient's clinical data, imaging findings, pathological diagnosis, treatment process and reviews the literature of TACC mimicking a thyroid tumor.

Adenoid cystic carcinoma (ACC) is a rare malignancy predominantly found in the salivary, lacrimal, upper gastrointestinal, and respiratory mucosal glands; adenoid cystic carcinoma of the external auditory canal (EAC) is particularly rare. Characterized by a proclivity for recurrence and metastasis, advanced adenoid cystic carcinoma often proves refractory to conventional chemotherapy. Novel therapeutic approaches, notably targeted therapies, are imperative to enhance treatment response rates in advanced adenoid cystic carcinoma patients. Herein, we present a case of primary adenoid cystic carcinoma affecting the right external auditory canal in a 56-year-old male presenting with symptoms of pruritus, swelling, pain, and hearing loss in the affected ear. Despite multiple attempts with diverse chemotherapy regimens yielding no success, the patient developed advanced lung metastases, prompting the utilization of anlotinib-targeted therapy. Subsequent administration of anlotinib led to a partial remission (PR), resulting in approximately 8 months of progression-free survival (PFS) in this individual.

Cylindroma is a benign adnexal tumor histologically characterized by clusters of small basaloid cells arranged in a pattern resembling a jigsaw puzzle. Breast cylindromas are extremely rare with approximately 20 reported cases throughout literature. We present a case of a 71-year-old female with a slow growing cystic breast mass, originally identified 8 years prior to biopsy. Mammography and ultrasound demonstrated features of a simple cyst with circumscribed margins and anechoic internal echogenicity, respectively. Biopsy was performed due to increase in size, revealing the pathologic entity of cylindroma. The patient ultimately underwent surgical excision, which confirmed the diagnosis. We discuss the radiology, pathology and clinical course of breast cylindroma.