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Pleomorphic adenoma (PA) most commonly manifests in the parotid gland, though it occasionally emerges in atypical locations. We present a case involving an 87-year-old female who exhibited chronic left-sided nasal symptoms, leading to the diagnosis of PA in the nasal cavity. This diagnosis was confirmed through rhinoscopy and subsequent pathological examination following the surgical excision of an 8x8 mm mass. The procedure, which included tumor-based cautery, alleviated her symptoms effectively. A follow-up strategy was established to monitor for any signs of recurrence. The patient has shown no signs of recurrence at subsequent three-month follow-up visits, highlighting the success of the intervention. This case underscores the importance of early recognition and intervention in atypical presentations of PA, which is crucial to prevent potential complications and ensure favorable outcomes.

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Benign salivary gland tumors are a rare and diverse group of neoplasms with significant variations in their site of origin, histological features, and biological behavior. This report describes the case of a 93-year-old woman with a markedly enlarged left cervical mass. Physical inspection uncovered a tumor of approximately 32 x 30 cm, featuring necrotic and ulcerated areas. The neoplasm, diagnosed as a pleomorphic adenoma (PA) through prior biopsies, had been growing gradually over fifteen years, with delayed surgical intervention due to concerns about her age and the tumor's size. Preoperative contrast-enhanced CT imaging showed a large left-sided cervical mass in close proximity to the airway, but without displacement or infiltration into major structures. An elective surgical approach was undertaken, involving complete resection of the giant PA, confirmed by histopathological evaluation. During the first month of postoperative follow-up, the patient experienced partial facial nerve paralysis but showed no evidence of tumor recurrence. Despite the tumor's considerable size, proximity to the airway, and the patient's advanced age, curative surgical intervention proved feasible. This case underscores that, with meticulous preoperative planning and careful surgical execution, age should not be a contraindication for surgery.

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Pleomorphic adenoma (PA) is the most prevalent benign salivary gland tumor. Although rare, among the minor salivary glands, palatal PA exhibits the highest incidence. Unlike other benign tumors, PA infiltrates the surrounding tissues, posing challenges for complete removal through conservative measures. Surgeons often resort to aggressive surgical procedures involving resection of adjacent tissue to ensure clear margins and prevent recurrence. This study aims to analyze diverse histological characteristics of palatal PA, seeking statistical correlations for early prediction of tumor aggressiveness. The goal is to facilitate the preservation of the periosteum during surgical resection and attain conservative surgical margins. A retrospective histopathological investigation encompassed 18 patients diagnosed with palatal PA who underwent surgical treatment at Hadassah Medical Centre, Jerusalem, Israel. Evaluated parameters included tumor size, pseudocapsule thickness, tumor-periosteum distance, and the presence of pseudopodia and satellite nodules indicating tumor penetration. Statistical significance was set at P < 0.05. Tumors of varying sizes, whether large or small, lack consistent features. Neither tumor size, pseudocapsule thickness, nor tumor-periosteum distance displayed correlations with tumor penetration features. Palatal PA exhibits varied histological attributes impacting surgical technique. The absence of correlations among these attributes impedes early prediction of tumor aggressiveness, casting doubt on periosteum preservation. The periosteum is sufficiently robust to contain the tumor and should be excised. There is no data to support either ostectomy or a through-and-through surgical resection as part of the treatment.

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Salivary gland tumors are a rare and heterogeneous group of neoplasms of the head and neck region. The mixed category of these tumors include the following entities: pleomorphic adenoma (PA), carcinoma ex pleomorphic adenoma (CEPA), salivary carcinosarcoma (CS), and metastasizing PA (MPA). The most common benign tumor of the salivary glands is PA. Metastasis and malignant degeneration have been reported in cases of PA of a salivary gland origin. Judging by their behavior, MPA, CEPA, and CS can be considered aggressive tumors. Invasive CEPA has been identified in the parotid gland more frequently. MPA and CS cases reported in the current literature are rare. In this paper, we present, narratively, the clinico-morphological features of this group of mixed tumors.

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Pleomorphic adenoma (PA) is the most common neoplasm of the salivary gland, presenting with a variety of histological features. In some cases, PA can undergo malignant transformation to carcinoma ex pleomorphic adenoma (CXPA). The transition from PA to CXPA is associated with complex molecular alterations, particularly involving the pleomorphic adenoma gene 1 (PLAG1) and high mobility group protein gene (HMGA2). This review investigates the molecular alterations of PLAG1 and HMGA2 in all domains in the malignant transformation of PA. Our analysis highlights that these markers are key alterations in the etiopathogenesis of PA and CXPA, with gene fusion and amplification being frequently reported mechanisms. Although the exact role of PLAG1 and HMGA2 in the oncogenic process remains unclear, further studies on the HMGA2 and PLAG1, are needed particularly in HMGA2-PLAG1-IGF2 which is proving to be a potential pathway for the development of clinically applicable therapies, especially for CXPA management.

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We present the case of a 71-year-old man who, after undergoing postoperative radiotherapy for epithelial carcinoma, developed progressively enlarging erythema. Initially, the condition resembled radiation dermatitis or erysipelas, and topical steroids and antibacterial agents were administered without success. A biopsy was performed for further evaluation, revealing a cutaneous invasion of parotid carcinoma. The lesion continued to enlarge, leading to dysphagia and ultimately necessitating a tracheostomy.

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OBJECTIVES: Details about salivary gland tumor histogenesis remain unknown. Here, we established a newly generated murine salivary gland tumor model that could overexpress pleomorphic adenoma gene 1 (PLAG1) and attempted to clarify the events that occur during the early phase of salivary gland tumor histogenesis. METHODS: Salivary gland tumors were generated using murine models (Sox9IRES-CreERT2; ROSA26-PLAG1). Lineage tracing of Sox9-expressing cells was performed using Sox9IRES-CreERT2; ROSA26-tdTomato mice, which were generated by crossing Sox9CreERT2/- and ROSA26-tdTomato mice (expressing the tdTomato fluorescent protein). Organ-cultured embryonic salivary glands from the murine model were morphologically analyzed, and mRNA sequencing was conducted two days after tumor induction for gene enrichment and functional annotation analysis. RESULTS: Salivary gland tumors exhibited epithelial features with acinar-like structures because of gene rearrangements in the luminal cells. Structural disturbances in the duct-acinar unit of the salivary gland were observed and cancer-related pathways were enriched among the differentially upregulated genes in the early phase of tumor induction in an organ-cultured embryonic salivary gland tumor model. CONCLUSIONS: The newly generated murine salivary gland tumor model may show that the tumorization of luminal stem/progenitor cells can result in the development of salivary gland tumors comprising only luminal cells.

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PURPOSE: To establish a radiomics nomogram based on MRI radiomics features combined with clinical characteristics for distinguishing pleomorphic adenoma (PA) from warthin tumor (WT). METHODS: 294 patients with PA (n = 159) and WT (n = 135) confirmed by histopathology were included in this study between July 2017 and June 2023. Clinical factors including clinical data and MRI features were analyzed to establish clinical model. 10 MRI radiomics features were extracted and selected from T1WI and FS-T2WI, used to establish radiomics model and calculate radiomics scores (Rad-scores). Clinical factors and Rad-scores were combined to serve as crucial parameters for combined model. Through Receiver operator characteristics (ROC) curve and decision curve analysis (DCA), the discriminative values of the three models were qualified and compared, the best-performing combined model was visualized in the form of a radiomics nomogram. RESULTS: The combined model demonstrated excellent discriminative performance for PA and WT in the training set (AUC=0.998) and testing set (AUC=0.993) and performed better compared with the clinical model and radiomics model in the training set (AUC=0.996, 0.952) and testing model (AUC=0.954, 0.849). The DCA showed that the combined model provided more overall clinical usefulness in distinguishing parotid PA from WT than another two models. CONCLUSION: An analytical radiomics nomogram based on MRI radiomics features, incorporating clinical factors, can effectively distinguish between PA and WT.

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BACKGROUND: Cellular angiofibroma, a rare benign mesenchymal neoplasm, is classified within the 13q/RB1 family of tumors due to morphological, immunohistochemical, and genetic similarities with spindle cell lipoma. Here, genetic data reveal pathogenetic heterogeneity in cellular angiofibroma. METHODS: Three cellular angiofibromas were studied using G-banding/Karyotyping, array comparative genomic hybridization, RNA sequencing, and direct cycling sequencing. RESULTS: The first tumor carried a del(13)(q12) together with heterozygous loss and minimal expression of the RB1 gene. Tumors two and three displayed chromosome 8 abnormalities associated with chimeras of the pleomorphic adenoma gene 1 (PLAG1). In tumor 2, the cathepsin B (CTSB) fused to PLAG1 (CTSB::PLAG1) while in tumor 3, the mir-99a-let-7c cluster host gene (MIR99AHG) fused to PLAG1 (MIR99AHG::PLAG1), both leading to elevated expression of PLAG1 and insulin growth factor 2. CONCLUSION: This study uncovers two genetic pathways contributing to the pathogenetic heterogeneity within cellular angiofibromas. The first aligns with the 13q/RB1 family of tumors and the second involves PLAG1-chimeras. These findings highlight the diverse genetic landscape of cellular angiofibromas, providing insights into potential diagnostic strategies.

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AIM: The purpose of this retrospective patient chart review was to analyze the clinical data of 52 patients with benign parotid tumors who underwent modified buried vertical mattress sutures and to assess the postoperative complication rate and patient scarring. METHODS: A total of 52 patients with benign parotid tumors underwent total parotidectomy and modified buried vertical mattress suture. Variables included general characteristics (age, gender, tumor diameter, and pathologic type), surgical indicators (suture time, wound healing time, operative time, hospital stay, bleeding volume, and drainage volume), complication rates, and Sunnybrook facial neurological function score, visual scar scale (VSS) score and patient and observer scar assessment scale (POSAS) score. RESULTS: Most tumors were less than 3 cm in diameter, with pleomorphic adenomas being the most common. Suture time was 14.83 ± 1.61 min, operative time was 58.90 ± 15.76 min and hospital stay were 5.12 ± 0.96 days. Postoperatively, salivary fistulae developed in one patient, Frey's syndrome in two patients, temporary facial paralysis in six patients and temporary numbness in the incision area in six patients. At 6 months postoperatively, 86.5% of patients had a Sunnybrook score of more than 80, and VSS scores and POSAS scores were between one and two. CONCLUSION: The postoperative complication rate was 30.8%, and the scarring in the facial incision area was mild and close to normal skin at 3 years postoperatively.

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OBJECTIVES: To conduct a comprehensive proteomic analysis of normal salivary gland tissue, pleomorphic adenoma (PA), and carcinoma ex-pleomorphic adenoma (CXPA), and validate the proteomic findings using immunohistochemistry. METHODS: Six normal salivary gland tissues, seven PA and seven CXPA samples underwent laser microdissection followed by liquid chromatography coupled to mass spectrometry. Protein identification and quantification were performed using MaxQuant software. Statistical analysis and functional enrichment were conducted using the Perseus platform and STRING tool, respectively. Immunohistochemistry was used for validation. RESULTS: Comparative proteomic analysis revealed 2680 proteins across the three tissue types, with 799 significantly altered between groups. Translocation protein SEC63 homolog, Annexin A6 and Biglycan were up-regulated in CXPA compared to PA. Decorin was markedly up-regulated in both PA and CXPA compared to normal salivary gland (log2 fold changes of 7.58 and 7.38, respectively). Validation confirmed elevated levels of Biglycan and Decorin in the extracellular matrix of CXPA compared to PA. CONCLUSIONS: Proteomic analysis identified differential protein expression patterns associated with malignant transformation of PA into CXPA. Findings indicate a crucial role for extracellular matrix proteins, specifically Biglycan and Decorin, in the tumorigenic progression of PA and CXPA.

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INTRODUCTION AND IMPORTANCE: Extensive Palatal post-operative defect management following excision of neoplasm is one of the most difficult challenges for oral and maxillofacial surgeons regarding its limited surgical access and visibility on narrow area, airway management difficulty during intubation, richness of maxillary vascular network resulting in enormous bleeding risk. Decision making regarding its surgical approach and impact on speech and mastication is important. This case series aim to describe comprehensive step by step perioperative and palatal defect management approach based on tumor pathological characteristic and anatomical perspective to achieve good surgical outcome. CASES PRESENTATION: Two cases of massive palatal pleomorphic adenoma were presented. Both of cases occurs in female patients. Lesions was crossing the midline, impair speech and causing discomfort. Preoperative diagnostic from CT scan and FNAB result was pleomorphic adenoma. CLINICAL DISCUSSION: Surgery for both cases done with wide periosteal sacrificing excision, ostectomy and surgical obturator placement from intraoral approach under general anesthesia with nasal intubation. Eventually the wounds healed without wound dehiscence and fistula, no speech impairment and no sign of reccurency. CONCLUSION: Understanding pathological characteristic of pleomorphic adenoma and basic anatomy of surrounding structure are important to formulate minimal invasive surgical and post-operative defect management planning and improve patient's quality of life.

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Tumors in the nasal septum originating from salivary glands are uncommon, and among them, pleomorphic adenomas represent a distinctive manifestation.This case study explores a female in her early thirties with a right-sided nasal mass, nasal obstruction, and intermittent bleeding. CT imaging revealed a lesion arising from the nasal septum with bony erosion. Histopathology confirmed pleomorphic adenoma,emphasizing the importance of thorough clinical evaluation, imaging, and biopsy for accurate diagnosis. Pleomorphic adenomas, typically found in major salivary glands, can occur in the respiratory tract, presenting challenges in distinguishing them from malignant tumors. Treatment involves wide local resection, and postoperative recurrence may necessitate radiotherapy. While intranasal pleomorphic adenomas generally have a favorable prognosis, those arising from the nasal septum have an elevated likelihood of malignancy. Vigilant monitoring is crucial due to the potential for recurrence, malignant transformation, and metastasis.

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The standard surgical procedure for treating the parotid gland's recurrent pleomorphic adenoma (RPA) is parotidectomy with facial nerve preservation (FN). Treatment of RPA remains challenging since controversies occur regarding recurrence, degree of revision surgery, postoperative radiation, and difficulty in conserving the FN. A retrospective review of patient's medical records treated for benign parotid neoplasms was conducted between 2017 and 2022 to identify individuals who underwent surgery for RPA. Demographic information, surgical intervention details, pre-and postoperative facial nerve function, histopathological analysis, and recurrence rates were collected. These variables were compared in patients with single recurrent tumors versus patients with multiple recurrent tumors. Twenty-one patients met the criteria, including 13 with a first recurrence, 7 with a second recurrence, and 1 with a third recurrence. Following surgery for multiple RPA, long-term FN outcomes were significantly worse (P = 0.005). There were no observable risk factors for tumor recurrence. The interval between the initial revision surgery and subsequent ones was drastically shortened. Our study suggests that the risk of permanent facial paralysis is greater with subsequent surgical procedures. Early detection of recurrence can aid in early re-operation.

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INTRODUCTION: Currently, the diagnosis of salivary gland tumors using imaging techniques is unreliable. METHODS: In this monocentric retrospective study, we examined patients who received a 68Ga-DOTATOC PET/CT and subsequently underwent a salivary gland tumor resection between 1 January 2010 and 31 December 2021. PET/CT image assessment was compared with somatostatin receptor (SSTR) expression and histology. RESULTS: Thirteen patients (five pleomorphic adenoma (PA) and eight other parotid lesions (OPL)) received a 68Ga-DOTATOC PET/CT. Imaging displayed strong focal tracer uptake in all PA except for one with strong tumor to background discrimination. PA revealed higher SUVmax, SUVmean, liver and blood pool quotients than those of Warthin tumors (WT) and of OPL. In comparison to the contralateral parotid, SUVmax (p = 0.02), SUVmean (p = 0.02), liver quotient (p = 0.03) and blood pool quotient (p = 0.03) were all significantly higher. In contrast, WT and OPL showed in relation to the contralateral parotid no significant differences of SUVmax (WT p = 0.79; OPL p = 0.11), SUVmean (WT p = 1.0; OPL p = 0.08), liver quotient (WT p = 0.5; OPL p = 0.08) and blood pool quotient (WT p = 0.8; OPL p = 0.19). Two PA and one granuloma were not available for examination. In the immunohistochemal analysis, all PA demonstrated the highest intensity of SSTR2 expression (grade 3). Furthermore, PA had a high percentage of cells expressing SSTR2 (20%, 80% and 55%). CONCLUSIONS: A strong tracer uptake in PA was shown in 68Ga-DOTATOC PET/CT. This may allow physicians to utilize radioligated somatostatin analogue PET CT/MR imaging to accurately diagnose PA. Additionally, it may be possible in the future to treat the PA with a noninvasive peptide receptor radionuclide therapy or with somatostatin analogues.

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No previous study has published magnetic resonance imaging (MRI) findings for a subglottic pleomorphic adenoma. Here, we describe the case of a 62-year-old man with a subglottic pleomorphic adenoma. Endoscopic findings revealed a smooth-surfaced tumor arising from the subglottic posterior wall. MRI revealed the lesion as an isointense region on T1-weighted images, which was homogeneously enhanced. This lesion showed a heterogeneously hyperintense region on T2-weighted images. Diffusion-weighted imaging (DWI) showed slightly high intensity in the same area, with a normal or only slightly high apparent diffusion coefficient (ADC). Laryngomicrosurgery was performed for transoral excision of the subglottic tumor, resulting in a postsurgical diagnosis of pleomorphic adenoma.

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AIMS: Most salivary gland neoplasms are distinguished by specific recurrent gene fusions. Recently, a subset of pleomorphic adenomas (PAs) originated from the parotid gland harboring the HMGA2:WIF1 fusion was described with a canalicular adenoma-like morphology and a greater propensity for recurrence and carcinomatous transformation. METHODS AND RESULTS: This study delineates the clinicopathological attributes of 54 cases of PAs exhibiting HMGA2 alterations, predominantly characterized by the HMGA2:WIF1 fusion, alongside a comparative analysis of their morphological and immunohistochemical profiles. The cohort consisted of 23 females and 31 males (n = 54), mean age was 56.7 (25-84), tumors predominantly originated from the parotid gland (94.4%, 51/54), with 3 cases from seromucous glands (5.6%). Mean tumor size was 2.6 cm (0.8-7.5). No clinical difference (demographic, follow-up) was observed among histological subsets (conventional, hybrid, and pure). Complete excision was performed in all cases, with follow-up data available for 41% (22/54) of patients, showing 13.6% of recurrence (3/22) between 5 and 8 months. Various histological growth patterns were identified, with the pure hypercellular monomorphic subset being the most prevalent. The HMGA2:WIF1 gene was identified in all subsets without any particular predominance. Novel gene partners of HMGA2 were identified, comprising NRXN1, INPP4B, MSRB3, PHLDA1, and FLJ41278. CONCLUSIONS: The present study reports that the HMGA2:WIF1 gene fusion was present in all subsets of PAs without significant predominance. However, further investigations are warranted to explore the relationship between histological subsets of PAs and the molecular alterations underlying them.

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Pleomorphic adenomas (PA) are the most prevalent benign salivary gland neoplasms. They may occur at any age, with a peak incidence between 40 and 60 years of age. They are more commonly observed in females (60%). These tumors can arise in both the major and minor salivary glands. Approximately 80% of these tumors are diagnosed in the parotid gland, whereas 10% arise in the minor salivary glands, mainly affecting the palates, followed by the lips and cheeks. This report describes two cases of unusual lesions that were diagnosed as (PA) in the minor salivary glands in our department via a review of the relevant literature. The first case involved an 83-year-old man who presented with a slow-growing swelling on the right side of the upper lip, and the second case involved a 45-year-old woman who presented with a slow-growing lesion on the palate. The presence of PA was confirmed histopathologically after surgical resection. Although relatively rare, PA is a benign lesion, the diagnosis of which must be known for appropriate therapeutic management.

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PURPOSE: This study aimed to investigate whether multiparametric magnetic resonance imaging (MRI) including dynamic contrast-enhanced (DCE) and diffusion weighted (DW) MRI can differentiate pleomorphic adenoma (PA) from schwannoma in the parapharyngeal space. METHODS: Forty-six patients with pathologically proven PAs and 47 schwannomas in the parapharyngeal space were enrolled. All patients underwent conventional MRI, and DW-MRI and DCE-MRI were performed in 30 and 33 patients, respectively. Fisher's exact, Mann-Whitney-U tests and Independent samples t-test were used to compare variables between PAs and schwannomas. Multivariate logistic regression analysis was used to examine the diagnostic performance of MRI parameters. RESULTS: The PAs usually show lobulation sign, posterior displacement of ICA and attached to the parotid gland deep leaf, while bird beak configuration, anterior displacement of ICA and involvement of foramen jugular were more commonly seen in the schwannomas(all p < 0.001). The washout rate of PAs was found to be higher than that of schwannomas (p = 0.035), whereas no significance was found in the other DCE-MRI parameters and in ADCs(p > 0.05). Using a combination of conventional MRI features including lobulation sign, bird beak configuration, direction of internal carotid artery(ICA) displacement and attached to the parotid gland in multivariate logistic regression analysis, sensitivity, specificity, and accuracy in differential diagnosis of PAs and schwannomas were 97.8%, 91.5% and 94.6%, respectively. CONCLUSION: Conventional MRI can effectively differentiate PAs from schwannomas in the parapharyngeal space with a high diagnostic accuracy. The DCE-MRI and DWI have limited added diagnostic value to conventional MRI in the differential diagnosis.