Canalicular Adenoma of Parotid- A Rare Case Report.

Salivary gland canalicular adenoma is rare yet benign. It accounts for fewer than 1% of salivary tumors and is the third most common non-cancerous mass in the small salivary glands. A 45-year-old presented to the outpatient department with complaints of swelling in the left parotid region for the past 3 years. Examination revealed a firm swelling in the left parotid region. USG and MRI revealed features suggestive of pleomorphic adenoma. Superficial parotidectomy was planned but intra-operatively, the mass was seen between the two lobes of the parotid, which was removed, and on histopathological examination, it was diagnosed as canalicular adenoma. Post-operatively, the patient's condition is excellent.

Revealing Enigma in Parotid Gland: Venolymphoma-A Rare Case Report.

Venolymphoma (VL) is a benign condition of oral cavity, but it is misdiagnosed as pleomorphic adenoma. Exact diagnosis can be made only after histopathological examination. In this report the author has presented a case of a 45-year-old male patient with VL of right parotid which was operated. The treatment indicated was complete excision of the lesion, which showed an excellent prognosis with low recurrence rate.

Salivary gland-like tumors of the breast: Histopathologic and genetic features with clinical implications.

Salivary gland-like tumors of the breast are rare neoplasms that share morphologic, immunophenotypic, and/or genetic features with their salivary gland counterparts, highlighting a shared underlying histopathogenesis in most cases. Salivary gland-like carcinomas included in the World Health Organization classification of breast tumors are adenoid cystic carcinoma, secretory carcinoma, mucoepidermoid carcinoma, acinic cell carcinoma, and the exceedingly rare polymorphous adenocarcinoma. These carcinomas are usually triple negative for estrogen receptor and progesterone receptor expression and HER2 overexpression, yet generally have favorable prognosis, in contrast to high-grade triple negative carcinomas of no special type. On the other hand, a small subset, such as solid-basaloid adenoid cystic carcinoma, rare high-grade carcinomas, and those associated with transformation to other types of high-grade invasive carcinoma can behave more aggressively. Other salivary gland-like tumors of the breast, such as pleomorphic adenoma and adenomyoepithelioma, are usually benign but can rarely undergo malignant transformation. Although clinical experience with salivary gland-like breast tumors is overall limited, their recognition and accurate classification has important implications for prognosis and clinical management, especially to avoid overtreatment of salivary gland-like carcinomas. The identification of characteristic genetic alterations and/or immunohistochemical surrogates in many of these tumors has practical applications to establishing an accurate diagnosis and directing clinical management. This review highlights the histopathologic and genetic characteristics of salivary gland-like breast tumors and the implications of the diagnosis for current clinical management.

Distinct copy number signatures between residual benign and transformed areas of carcinoma ex pleomorphic adenoma.

The mechanisms involved with the pathogenesis of carcinoma ex pleomorphic adenoma (CXPA) seem to be associated with the accumulation of molecular alterations in the pleomorphic adenoma (PA). In this sense, using array-based comparative genomic hybridization (aCGH) a rare series of 27 cases of CXPA and 14 residual PA (rPA) adjacent to the transformation area, we investigated the profile of the copy number alterations (CNAs) comparing benign residual and transformed areas. The main findings were correlated with the histopathological classification by histologic subtype and degree of invasion. The distribution of losses (p = 0.187) and amplifications (p = 0.172) was not statistically different between rPA and CXPA. The number of gains was increased in the transformed areas compared to the benign residual areas (p = 0.005). PLAG1 gain was maintained along the malignant transformation, as it was observed in both residual PA and CXPA samples, likely being an earlier event during transformation. The amplification of GRB7 and ERBB2 may also be an initial step in the malignant transformation of PA to CXPA (salivary duct carcinoma subtype). Furthermore, the amplification of HMGA2 and RPSAP52 were the most prevalent alterations among the studied samples. It was noteworthy that amplified genes in the transformed areas of the tumors were enriched for biological processes related to immune signaling. In conclusion, our results underscored for the first-time crucial CNAs in CXPA, some of them shared with the residual benign area adjacent to the transformation site. These CNAs included PLAG1 gain, as well as amplification of GRB7, ERBB2, HMGA2, and RPSAP52.

Ultrasonography-Guided Fine Needle Aspiration Cytology Versus Histopathology: Diagnostic Consistency in Salivary Gland Neoplasms.

Introduction Salivary gland lesions include a diverse range of histological types and biological behaviors, making accurate diagnosis and effective treatment challenging for specialists. Fine needle aspiration cytology (FNAC) plays a significant role in making preoperative diagnoses and further plans of surgery. In differentiating malignant from benign salivary gland tumors, the diagnostic accuracy of fine needle aspiration cytology (FNAC) is high. However, the final diagnosis is histopathological. The aim of this study is to correlate cytological and histopathological interpretations in salivary gland lesions. Materials and method Thirty patients were included in this study, all of them were diagnosed with salivary gland swellings and operated on in the ear, nose, and throat (ENT) department of Mata Gujri Memorial (MGM) Medical College, Kishanganj, in the northeastern region of India, between December 2020 and November 2022. Ultrasonography (USG) and computed tomography (CT) scans were performed in each case, and FNAC was done to make a preoperative diagnosis. After surgery, specimens were collected for histopathological examination. A correlation was then made between the findings of the preoperative cytological examination and the results of the postoperative histopathological examination. The comparison was performed using a 2×2 table, and the analysis of sensitivity, specificity, positive predictive value (PPV), and negative predictive value (NPV) was carried out using Microsoft Excel (Microsoft Corp., Redmond, WA) and SPSS software (IBM SPSS Statistics, Armonk, NY). Results An equal number of male and female samples were included in the study, in which parotid involvement was predominant (60%). On FNAC, 53% of parotid gland tumors were found to be benign, whereas only 23.3% of submandibular gland tumors were benign. Pleomorphic adenoma was the most common finding. In the present study, the sensitivity, specificity, positive predictive value, and negative predictive value of USG-guided FNAC, compared to the gold standard histopathology, were 96%, 33.3%, 92.3%, and 50%, respectively. The diagnostic accuracy was found to be 89.3%. Conclusion The findings of this study suggest that FNAC is a reliable technique for the preoperative diagnosis of salivary gland tumors, as it is minimally invasive and offers valuable diagnostic information. An accurate cytological diagnosis can help avoid unnecessary surgery.

Pleomorphic Parotid Adenoma in a Child Affected with Cri du Chat Syndrome: Clinical, Cytogenetic, and Molecular Analysis.

Salivary gland pleomorphic adenoma (SGPA) is the most common type of benign epithelial tumor; it is observed more commonly in females (with a female-to-male ratio of 1.43:1), and the age at diagnosis ranges between 40 and 59 years, with only 2% of cases diagnosed before age 18. Cri du Chat (CdC) is a rare syndrome caused by deletions of various sizes in the short arm of chromosome 5. Tumors in CdC patients are extremely rare: in Danish, Spanish, Australian, and Japanese groups of cases, no tumors have been reported, while a few cases have been described among 321 CdC patients collected in Italy and Germany. These cases all involve tumors that appear at a young age. We here report the case of a parotid pleomorphic adenoma in an 8-year-old boy with CdC. Exome analysis did not identify variants certainly significant for the development of SGPA. A CGH array, analyzed both in peripheral blood and tumor samples, failed to recognize anomalies previously associated with SGPA but identified a de novo duplication in 7p15.2, which contains part of a gene, SKAP2, in which the increased copy number is associated with the development of a different type of tumor such as pancreatic duct adenocarcinoma. The assumption that the duplication in 7p15.2 is relevant for the development of SGPA in our patient implies that CGH array studies must be included early in life in routine work-ups of CdC to identify CNVs with possible pathogenic roles for tumor development. This is particularly also relevant in relation to the severely impaired possibility for patients with CdC to report discomfort or pain related to tumor development. Constitutional CNVs in addition to the deletion in 5p should also be extensively studied to verify if their presence in some patients could explain why, in these cases, tumors develop at an age younger than expected.

HMGA2-positive salivary gland neoplasms with prominent trabecular/canalicular morphology: a focus on carcinomas arising within this phenotype.

AIMS: Pleomorphic adenoma (PA) with a prominent trabecular/canalicular morphology has consistent HMGA2 protein expression, and association with HMGA2 fusions. We report our experience with this subtype, with emphasis on the carcinomas that can arise in this context. METHODS AND RESULTS: A retro- and prospective review (2013-2024) of major salivary gland tumours with prominent trabecular/canalicular morphology was performed. Twenty-one parotid tumours met the criteria: 14 benign (66.7%), six carcinomas (28.6%), and one of uncertain behaviour (4.7%). HMGA2 immunohistochemistry (IHC) was performed on all cases. Next-generation sequencing was successfully performed on 18. Seven benign cases had a conventional PA component. In all cases, the tumour cells in these trabecular/canalicular areas demonstrated variable papillary thyroid carcinoma-like nuclear changes, including chromatin clearing, overcrowding, membrane irregularities, and intranuclear pseudoinclusions. Benign tumours were well-demarcated, whereas carcinomas demonstrated either a multinodular pattern of invasion or subtle infiltration. Two carcinomas showed increased cytologic atypia and architectural complexity and one had perineural invasion. By IHC, all were positive for HMGA2. In the trabecular/canalicular areas, there was consistent strong expression of CAM5.2, S-100, and SOX-10 and variable expression of p63 but negative p40. HMGA2 alterations were detected in 16 of 18 cases (89%). Follow-up was available on two carcinomas, with one being locally recurrent. CONCLUSION: While most HMGA2-positive salivary gland neoplasms with a prominent trabecular/canalicular growth pattern are benign, they, like traditional PAs, may give rise to carcinomas that can locally recur. These carcinomas can be deceptively bland, subtly infiltrative, or have a multinodular pattern of invasion.

Decoding a Rare Case of Pleomorphic Adenoma: A Case Report and Review of the Literature.

Diagnosing palatal swellings is crucial for various reasons, mainly because these swellings can signal a range of health problems, from benign conditions to more serious diseases. Here, we have reported an interesting case of long-standing palatal swelling. Accurate diagnosis typically involves a combination of clinical examination, patient history, imaging studies, and possibly biopsy or other laboratory tests. Since each condition has unique characteristics and treatment approaches, differential diagnosis is essential for ensuring effective management. This case highlights the importance of considering pleomorphic adenoma in the differential diagnosis of palatal tumors and demonstrates the effectiveness of surgical management. A literature review is also presented, discussing the clinical, radiological, and histopathological features of this rare entity. Accurate diagnosis and timely intervention are crucial for effective treatment and maintaining overall oral health.

Development and Evaluation of a Convolutional Neural Network for Microscopic Diagnosis Between Pleomorphic Adenoma and Carcinoma Ex-Pleomorphic Adenoma.

AIMS: To develop a model capable of distinguishing carcinoma ex-pleomorphic adenoma from pleomorphic adenoma using a convolutional neural network architecture. METHODS AND RESULTS: A cohort of 83 Brazilian patients, divided into carcinoma ex-pleomorphic adenoma (n = 42) and pleomorphic adenoma (n = 41), was used for training a convolutional neural network. The whole-slide images were annotated and fragmented into 743 869 (carcinoma ex-pleomorphic adenomas) and 211 714 (pleomorphic adenomas) patches, measuring 224 × 224 pixels. Training (80%), validation (10%), and test (10%) subsets were established. The Residual Neural Network (ResNet)-50 was chosen for its recognition and classification capabilities. The training and validation graphs, and parameters derived from the confusion matrix, were evaluated. The loss curve recorded 0.63, and the accuracy reached 0.93. Evaluated parameters included specificity (0.88), sensitivity (0.94), precision (0.96), F1 score (0.95), and area under the curve (0.97). CONCLUSIONS: The study underscores the potential of ResNet-50 in the microscopic diagnosis of carcinoma ex-pleomorphic adenoma. The developed model demonstrated strong learning potential, but exhibited partial limitations in generalization, as indicated by the validation curve. In summary, the study established a promising baseline despite limitations in model generalization. This indicates the need to refine methodologies, investigate new models, incorporate larger datasets, and encourage inter-institutional collaboration for comprehensive studies in salivary gland tumors.

Revision parotidectomy - analysis of indications for the procedure and treatment results based on 10 years of follow-up in a single center.

<b>Introduction:</b> Surgical removal of recurrent parotid gland tumours is the first-line treatment but presents an increased risk of facial nerve injury and a considerable re-recurrence failure rate.<b>Aim:</b> Identification of individuals exposed to a higher risk of re-procedure, raising awareness in the preoperative setting, and proposing an optimal follow-up.<b>Methods:</b> The retrospective review included 72 patients treated with revision surgery in a single centre. The demographics, clinicopathologic variables, and operative details were analysed.<b>Results:</b> Recurrent pleomorphic adenoma (PA) was the main reason for reoperation (66.7%), followed by new monomorphic adenoma (13.9%), resection extension (12.5%), and malignancy recurrence (6.9%). Time to revision surgery was on average 68.6 months and was the shortest for extended resection cases (average 1.9 months). The period was substantially longer in recurrent PA (90.8 months). The final facial nerve function according to the House-Brackmann scale (HBS) decreased in 37% of patients after reoperation. The number of recurrences per patient ranged from one in 61% of cases to eight in a solitary case.<b>Conclusions:</b> The rate of revision parotid surgery was 8.4%. Negative margins at the first resection were not of protective significance. Recurrent PA was the main cause of revision surgery and over one-third of this cohort had a subsequent relapse. As many as 37% of patients experienced a decrease in facial nerve function following revision surgery.

Life-Threatening Bleeding in Atypical Pleomorphic Adenoma of the Soft Palate.

Introduction: Atypical pleomorphic adenoma (PA) is an uncommon tumor, more frequent in submandibular and parotid glands. PA is classified as atypical when it presents hypercellularity, necrosis or hyalinization, dysplasia, capsular violation or distant metastases. Case Report: We described a case of a 39-year-old female presented with a slowly growing mass involving the soft palate. A life-threatening bleeding from PA with hemorrhagic shock occurred and required ligation of the external carotid artery with tracheotomy. A transoral en-bloc excision of the mass (70 x 50 x 40 mm) was performed. Pathological exam demonstrated an atypical PA, with hypercellular fields and myoepithelial and squamous differentiation. Conclusion: An appropriate diagnostic evaluation and a prompt intervention are essential to avoid dangerous complications, even for benign neoplasms.

Metastasizing pleomorphic adenoma of parotid gland: Case report.

Pleomorphic adenoma is a benign tumor and the most common salivary gland neoplasm. Metastasizing pleomorphic adenoma shares histological characteristics with pleomorphic adenoma but exhibits malignant behavior, including local lymph node involvement and/or distant metastasis. Several potential risk factors for metastasizing pleomorphic adenoma have been identified, some of which are associated with incomplete tumor clearance due to inadequate surgical techniques used in the treatment of primary pleomorphic adenoma. Here, we present a rare case of metastasizing pleomorphic adenoma originating from the parotid gland and describe its clinical features. The patient was a 75-year-old female with a surgical history of enucleation of a pleomorphic adenoma in the left parotid gland. Total parotidectomy and left neck dissection were performed for the left parotid tumor this time, and the patient attended one postoperative outpatient visit but subsequently was lost to follow-up.

Insights into the molecular alterations of PLAG1 and HMGA2 associated with malignant phenotype acquisition in pleomorphic adenoma.

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.

Erysipelas-Like Carcinoma of the Parotid Gland With Cutaneous Metastasis to the Neck Requiring Tracheostomy: A Case Report.

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.

"The correlation between histopathological pattern and surgical treatment for palatal pleomorphic adenoma. Can we choose a more conservative approach?"

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.

Clinical and Morphological Aspects of Aggressive Salivary Gland Mixed Tumors: A Narrative Review.

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.

Pleomorphic Adenoma of the Nasal Cavity.

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.

Effective Management of a Giant Deforming Pleomorphic Adenoma With Airway Displacement in a 93-Year-Old Patient: A Case Report.

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.

Comprehensive perioperative and minimally invasive post-operative defect approach of massive palatal pleomorphic adenoma: Case series.

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.

PLAG1 overexpression in salivary gland duct-acinar units results in epithelial tumors with acinar-like features: Tumorization of luminal stem/progenitor cells may result in the development of salivary gland tumors consisting of only luminal cells.

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.