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Rational: Pleomorphic adenoma (PA) is a rare benign tumor mainly affecting the major salivary glands, known for its diverse histological appearances that can mimic malignancies. When it occurs in the hard palate it present diagnostic and management challenges compared to other sites due to the anatomical location and potential proximity to critical structures. This case reports a rare presentation PA starting as an ulcer, alongside a review of rare cases of PA reported in last 5 years. We aim to highlight clinical challenges and emphasize the need for awareness in diagnosis of this diverse entity amongst the clinicians before reaching a definitive conclusion., Patient Concerns: A 41-year-old female reported an asymptomatic large swelling on the right side of the posterior palatal region. Clinical diagnosis revealed a firm, rubbery, and non-tender swelling of approximately 4 cm × 4 cm diameter. A triangular incisional biopsy was performed to confirm the diagnosis., Diagnosis: The histopathological evaluation confirmed the presence of a PA with a well-encapsulated and compressed salivary gland. A wide surgical dissection was made to remove the entire encapsulated tumor mass, including the mucoperiosteum and eroded bone of the palate. The borderline of the tumor was carefully identified in the surrounding healthy tissue. The hemostasis was obtained using a simple interrupted suture., Lesson: The diagnosis of PA is difficult as it usually involves extensive squamous and mucous metaplasia, confusing it with malignant disorders. Histopathological and clinical examinations are important for differentiating this lesion from other tumors. Complete surgical excision is reported as the first line of treatment., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2024 the Author(s). Published by Wolters Kluwer Health, Inc.)

When neglected for a long time, salivary gland pleomorphic adenoma (PA) can attain a considerable size, increasing the patient's morbidity along with the risk of malignant transformation. Very few case reports are available describing PA of the parotid glands presenting as a large cervicofacial mass. We report a case of epithelial myoepithelial carcinoma -a rare subtype of carcinoma ex-PA (Ca-Ex-PA) of non-luminal differentiation, that developed over a long period in a primary PA of the parotid gland and presented as a giant cervicofacial mass., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

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., Competing Interests: No competing interests were disclosed., (Copyright: © 2024 Samia M et al.)

Purpose: Pleomorphic adenoma (PA), the most common benign tumour of the parotid gland, requires accurate preoperative diagnosis owing to its capacity for malignant transformation. The aim of this study was to evaluate our experience with ultrasound-guided fine-needle aspiration biopsy (FNAB) in the diagnostic algorithm for patients with PA and to assess clinical outcomes for those with different surgical approaches., Material and Methods: We carried out a retrospective analysis of patients treated for parotid gland mass between 2010 and 2016. These had had preoperative FNAB and had undergone subsequent surgery., Results: 165 patients had FNAB with the result of PA and the definitive histology confirmed PA in 159 cases (96.4%). On the other hand, in 179 patients, the definitive histology showed PA and the preoperative FNAB result corresponded in 159 cases (88.9%). The measured sensitivity, specificity and accuracy of ultrasound-guided FNAB in the diagnosis of PA were, respectively, 88.83%, 96.23% and 92.31%. Most of the patients underwent superficial or partial superficial parotidectomy, followed by extracapsular dissection which was associated with statistically lower risk of facial nerve injury (P=0.04)., Conclusion: Ultrasound-guided FNAB is simple, accurate and valuable in the diagnosis of PA and provides results that can lead to the choice of less invasive operative treatment., Competing Interests: The authors report no conflicts of interest in this work.

While acinic cell carcinoma (AciCC) can undergo high-grade transformation (HGT) to high-grade adenocarcinoma or poorly differentiated carcinoma, other morphologies such as spindle cell/sarcomatoid carcinoma are rare and not well-characterized. We herein report a novel case of AciCC with squamoglandular and chondrosarcomatous HGT mimicking a so-called 'carcinosarcoma ex-pleomorphic adenoma'. The patient is an 81-year-old male with a two-month history of neck swelling and referred otalgia who presented with a left parapharyngeal space mass extending into retropharyngeal space and pterygoid muscles. On resection, the tumor showed considerable morphologic diversity with high-grade serous and mucous acinar components as well as cribriform to solid apocrine-like components with comedonecrosis and squamous differentiation, all of which were embedded in a chondromyxoid background ranging from paucicellular and bland to a high-grade chondrosarcoma/pleomorphic sarcoma-like appearance. Only a minor conventional AciCC component was noted. Immunostains were negative for AR and only focally positive for GCDFP-15 arguing against a true apocrine phenotype, while PLAG1 and HMGA2 were negative arguing against an antecedent pleomorphic adenoma. On the other hand, SOX-10, DOG-1 and PAS after diastase highlighted serous acinar differentiation, and mucicarmine, and NKX3.1 highlighted mucous acinar differentiation. NR4A3 immunohistochemical staining and NR4A3 fluorescence in situ hybridization were positive in the carcinomatous and sarcomatoid components while sequencing analysis of both components revealed identical alterations involving TP53, PIK3CB, ARID1A, and STK11. This unique case warrants caution in designating all salivary sarcomatoid carcinomas with heterologous elements as part of the 'carcinoma ex-pleomorphic adenoma' family., (© 2024. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Calcification within pleomorphic adenomas of the lacrimal gland is well recognized but uncommon, being seen more readily in lacrimal gland carcinomas. Bony formation, ossification, in pleomorphic adenomas of the lacrimal glands is even rarer. Together with extensive sclerosis, or "coagulative necrosis," ossification and necrosis should alert the clinician to the risk of malignant transformation. However, both can mimic carcinomatous change, leading to misinterpretation of malignancy in an otherwise benign lacrimal gland neoplasm. We present 2 case reports of patients with clinically presumed pleomorphic adenomas of the lacrimal gland whose histopathology demonstrated lacrimal gland ossification and necrosis without features of malignancy or invasive disease., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2024 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Chondroid syringoma (CS) is a benign, slow-growing mixed tumour that arises from the sweat glands and usually presents in the head and neck area. Histopathological examination is important for proper diagnosis, as CS is often confused with epidermal cysts due to its rare presentation. This article presents a man in his 40s with a right upper lip mass that emerged 6 months prior to presentation. An intraoral surgical excision was performed and the histopathological analysis revealed solid epithelial cells that formed multiple, non-branching ducts lined by cuboidal epithelium. Cystic spaces were filled by heterogeneous eosinophilic material embedded in chondromyxoid stroma. Histopathology identified the lesion as an eccrine-variant CS. The patient recovered well., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2024. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: Carcinoma ex pleomorphic adenoma (CXPA) is a rare malignant salivary gland tumor. Although multiple reviews have been published on salivary gland malignancies, it has been a decade since the last dedicated systematic review pertaining to CXPA alone was published. This study examines molecular factors in CXPA diagnosis., Data Sources: MEDLINE, CINAHL, Embase, Scopus, Web of Science (BIOSIS), Cochrane CENTRAL, Health Collection (Informit), OpenDOAR, and GreyNet International., Review Methods: Systematic review and meta-analysis from inception to October 31, 2022 for all English language studies pertaining to "carcinoma ex pleomorphic adenoma." Predicted incidence of each biomarker was calculated with meta-analysis. Comparison against pleomorphic adenoma (PA) and salivary duct carcinoma (SDC) when reported within the same study are performed. Risk of bias performed with JBI tool for prevalence studies., Results: Of 19151 unique studies undergoing abstract screening, 55 studies (n = 1322 patients) underwent data analysis. Biomarkers with >3 studies were p53, HER2, AR, EGFR, PLAG1, ERBB, ER, PR, HMGA2, p16, p63, a-SMA, RAS, PTEN, PDL1, BRAF, PIK3CA, and c-kit. Highest incidence was seen in AR, EGFR, p16, and p53. Significant differences were demonstrated compared with PA and SDC. There was high heterogeneity and overall high risk of bias within studies., Conclusion: Molecular factors are an area of interest in the diagnosis of CXPA. Our study results support examining CXPA as a discrete cohort in future targeted therapy trials. Laryngoscope, 134:1042-1053, 2024., (© 2023 The American Laryngological, Rhinological and Otological Society, Inc.)

Purpose: This systematic review analyzed the clinical behavior and odds of malignancy of the palatal recurrent pleomorphic adenomas., Methods: Systematic review of patients with recurrent pleomorphic adenoma arising in the palate. Database search: MEDLINE, Scopus, Web of Science, Cochrane, EMBASE, Virtual Health Library, Google Scholar, and OpenGrey. A binomial logistic regression was performed to assess the odds of detecting recurrence five, 10 and 20 years after the treatment of primary tumor., Results: Thirteen studies (n = 18 patients) out of 336 were included. The recurrent pleomorphic adenoma in palate was more common in females (61.6%), average age was 49 years old (range 9-73 years old). Four patients progressed to malignant transformation. The odds ratio (OR) of detecting a recurrence at 10 (OR = 5.57; 95% confidence interval - 95%CI 1.13-27.52), and 20 years (OR = 18.78; 95%CI 3.18-110.84) after treatment of primary pleomorphic adenoma was significantly higher than at one-year follow-up., Conclusions: The recurrence of pleomorphic adenoma in palate remains a rare event of late occurrence. It mainly affects middle-aged female and carries a risk of malignant transformation. Although uncommon, patients with palatal pleomorphic adenoma should be warned about the possibility of recurrence or malignant transformation of tumor at advanced ages.

Abstract: Chondroid syringoma is a benign, rare, asymptomatic, slow-growing mixed tumor. The authors present the case of a 17-year-old adolescent boy who had a tumor on the tip of his nose for a duration of 8 months. An excisional biopsy was performed under local anesthesia. Based on histopathologic analysis, the resected lesion was identified as a chondroid syringoma., (Copyright © 2023 Wolters Kluwer Health, Inc. All rights reserved.)

Salivary gland neoplasms account for 3% of all head and neck tumours. Pleomorphic adenoma (PA) is the most common salivary gland tumour that mainly occurs in the parotid gland, followed by minor salivary glands of the oral cavity, however, the occurrence of PA inside the jaw bones is exceedingly rare and very few cases have been reported in the literature. Inside jaw bones these lesions tend to imitate large osteolytic lesions encompass a diagnostic challenge. An exhaustive review of the literature revealed only 10 cases of central pleomorphic adenoma. We present a rare case of primary PA that occurred inside the mandible and was provisionally diagnosed as ameloblastoma.

Pleomorphic adenoma (PA) is a benign tumor characterized by slow-growing mixed tumors in the craniofacial area. It is relatively common in salivary glands; however, PA of the nasal cavity, which arises in the minor salivary glands, is rare. We present the case of a large PA in the nasal cavity of an adult immunocompetent woman with nasal obstruction and intermittent epistaxis. Based on preoperative radiologic examinations, she was misdiagnosed with an inverted papilloma. Endoscopic resection was performed under general anesthesia. Pathologically, the patient was confirmed to have PA, which has great cellularity and few stromal components. No complications or recurrences during the 1-year follow-up period were observed., Competing Interests: The authors report no conflicts of interest., (Copyright © 2023 by Mutaz B. Habal, MD.)

To investigate the clinical characteristics of patients with primary pleomorphic adenoma of the lower respiratory tract. The clinical manifestations, laboratory results, pathological and imaging, treatment and prognosis of 7 patients with primary pleomorphic adenoma of the lower respiratory tract who were treated in Hunan Provincial People's Hospital from December 2015 to May 2020 were analyzed. Among the 7 patients, 5 patients had cough and expectoration as the main clinical manifestations, and the other 2 patients had no symptoms. Pleomorphic adenomas of the lower respiratory tract are mostly located in the trachea or bronchus, and the chest computed tomography findings are circular or ellipsoid masses, or flake high-density shadows and local thickening of the tracheobronchial wall. Tumor histological features and immunohistochemistry can diagnose pleomorphic adenoma and its origin. In this study, 2 asymptomatic patients refused further treatment, 1 survived for more than 3 years, and the other was lost to follow-up during follow-up. One patient with surgical resection was followed up for 5 years after surgery and had a good survival status. The other 4 patients with respiratory symptoms who could not undergo surgery were mainly treated with bronchoscopic interventional therapy on demand, and the survival time up to now was 2 to 5 years. Primary pleomorphic adenoma of the lower respiratory tract is rare in clinic. Its clinical symptoms are related to the location and size of the tumor. Both surgical resection and bronchoscopic intervention have a good clinical prognosis. The cause of death of patients with such diseases is mostly dyspnea caused by tumors in the airway. Early diagnosis, timely intervention and regular follow-up can make patients obtain better curative effect., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Chondroid syringoma is a rare adnexal tumor of skin, with few cases diagnosed on fine needle aspiration cytology (FNAC). Hyaline cell-rich chondroid syringoma (HCRCS) is a very uncommon variant of chondroid syringoma described in histopathologic studies. This variant differs from the usual chondroid syringoma in its site of presentation, cytohistologic features, and morphologically mimics malignant neoplasms. To the best of our knowledge, cytologic features of this entity have never been described. This case report highlights some unusual features of this tumor and discusses the cytologic features of HCRCS along with neoplastic mimics., (© 2023 Wiley Periodicals LLC.)

Objective: Pleomorphic adenoma (PA) of the lip is not a common phenomenon, and existing literature provides limited information on the clinicopathological features of labial PA., Study Design: Patients diagnosed with labial PA at our single institution over the past 20 years (2001-2020) was retrospectively screened and analyzed to investigate the epidemiologic and clinicopathological features of these tumors., Results: A total of 173 cases were screened out, and the average age was 44.3 (range 7-82) years, with a peak incidence rate during the third decade. A slight predilection for men (52%) was observed, and PA occurs more frequently in the upper lip than in the lower lip, with a ratio of 14.7:1. On clinical examination, labial PAs usually present as painless masses that develop slowly with no systemic symptoms. Histologically, labial PAs contain myoepithelial and polygonal epithelial cells in myxoid, hyaline, fibrous, chondroid, and even osseous tissues, similar to those in other sites. Specifically, 15 of 173 patients with labial PA presented with cutaneous PA., Conclusion: Labial PA presents over a wide age range and dominantly occurs at the upper lip. Surgical resection is the major treatment strategy, and postoperative recurrence or malignant transformation of labial PA was extremely rare., Competing Interests: Declaration of Competing Interest None., (Copyright © 2023 Elsevier Masson SAS. All rights reserved.)

Background: Basal cell adenoma (BCA) and pleomorphic adenoma (PA) are among the most common benign neoplasms of the salivary glands. The aim of this study was to analyze and compare the diagnosis, treatment, and recurrence rate of these two different types of parotid benign tumors., Methods: A retrospective analysis of all cases of parotid gland BCA and PA surgically treated between January 1, 1990, and December 31, 2019, was performed at our university., Results: A total of 349 patients were enrolled in the present study, 311 of which (89.1%) were affected by PA, and 38 patients (10.9%) by BCA. The most frequently performed surgery was partial parotidectomy for both groups (85.9% in PA and 65.8% in BCA). Perioperative complications - often transient and of short duration - occurred within 48 hours of surgery and were observed in 30.6% of PA patients and in 18.4% of BCA patients; furthermore, recurrences were noticed in 19 PA patients (6.2%) and in 3 BCA patients (7.9%) (rates in range with the available literature data)., Conclusions: To the best of our knowledge, this study is one of the largest single-center series in the literature comparing diagnosis, treatment, recurrence rate and clinical-pathological features of two different types of benign parotid gland tumors, BCA, and PA.

Context.—: Salivary gland neoplasms are rare lesions in the head and neck (H&N) pathology realm. There are more than 20 malignant and 15 benign salivary gland neoplasms in the 5th edition of the World Health Organization classification of H&N tumors. These neoplasms consist of heterogeneous groups of uncommon diseases that make diagnosis and treatment challenging for the clinical team. Using an algorithmic immunohistochemical approach-defined tumor origin and type has proven to be effective and advantageous. Immunohistochemistry may be used as sort of a "diagnostic looking glass," not as a positive or negative type tool, but as an indispensable complement to a hematoxylin-eosin morphologic pattern-based approach. Furthermore, the understanding of the novel discoveries of the salivary gland gene fusions and the molecular aspects of these tumors makes the process easier and improve the diagnosis as well as treatment aspects. This review reflects our experience with more recent diagnostic antibodies, which include MYB RNA, Pan-TRK, PLAG1, LEF1, and NR4A3. Each of these is linked with a specific type of neoplasm; for example, gene fusions involving the PLAG1 and HMGA2 oncogenes are specific for benign pleomorphic adenomas, and MYB is associated with adenoid cystic carcinoma., Objective.—: To review these more recent antibodies, which highly enhance salivary gland neoplasm diagnosis., Data Sources.—: The study sources involved literature PubMed searches, including multiple review articles, case reports, selected book chapters, and Geisinger Medical Center cases., Conclusions.—: Salivary gland tumors are a rare, varied group of lesions in H&N pathology. We need to have continuous readings and revisions of the molecular consequences of these fusion oncoproteins and their subsequent targets, which will eventually lead to the identification of novel driver genes in salivary gland neoplasms., (© 2023 College of American Pathologists.)

Introduction: Myoepithelial carcinoma (MECA) is an infrequently recognized salivary gland (SG) neoplasm that commonly develops within a preexisting pleomorphic adenoma (MECA ex PA). Fine-needle aspiration (FNA) biopsy reports of this neoplasm are largely restricted to small series and single case reports., Methods: Our cytopathology files were searched for examples of SG MECA/MECA ex PA having confirmatory histopathologic verification. Conventional FNA biopsy smears were performed, and exfoliative specimens processed using standard techniques., Results: Thirteen cases from 9 patients (M:F = 3.5:1; age range: 36 to 95 years, mean age = 60 years) met inclusion criteria. FNA biopsy sites included parotid gland (4), trunk (2), scalp (2), and neck (2). Exfoliative specimens included pleural fluid (1), bronchial brushing (1), and bronchoalveolar lavage (1). Most cases were metastatic deposits (8; 62%), 4 were primary neoplasms, and 1 a local recurrence. FNA diagnoses were MECA ex PA (6; 46%), myoepithelial neoplasm (2), PA (2), basaloid neoplasm (1), atypical myoepithelial cells (1), and myxoma (1). Ancillary testing in 2 cases showed positive staining for myoepithelial markers. Cytologic features were that of a low-grade neoplasm composed principally of epithelioid/polygonal cells exhibiting minimal if any cytologic atypia. Myxoid and chondromyxoid stroma was often the dominant feature in MECA ex PA aspirates., Conclusion: In the primary setting, a cytologic diagnosis of MECA/MECA ex PA is extremely challenging if at all possible. Due to overwhelming amounts of stroma, the diagnosis may be challenging in some cases of metastatic MECA ex PA., (Copyright © 2023 American Society of Cytopathology. Published by Elsevier Inc. All rights reserved.)

Background: Lacrimal gland adenoma is a benign tumour of the lacrimal gland mostly involving the orbital part of the gland and composed of epithelial and myoepithelial components. It involves the third and fourth decade of life as a gradual painless enlargement of the lacrimal gland., Case: This is a case report of a 30-year-old female presenting with the forward bulging of the right eye causing the eye to be displaced medio-inferiorly over the course of one year. After careful clinical examination and MRI, a clinical diagnosis of lacrimal gland adenoma was established and was planned for right lateral orbitotomy (without marginotomy) via eyelid crease incision and transcutaneous-transseptal approach with complete excision of the tumour under general anaesthesia., Observations: This case was presented to the out patient department with mild painful non-axial proptosis of the right eye. After clinical examination, her MRI report showed a well-defined altered signal intensity enhancing lesion on the extraconal compartment of the right orbit in the antero-supero-lateral aspect. After complete surgical removal of the mass, it was sent for histopathological analysis and it confirmed it as a pleomorphic adenoma. The patient has been following up every 6 months for 2 years and is asymptomatic., Conclusion: Being the most prevalent lacrimal gland tumour, pleomorphic adenoma affects the unilateral lacrimal gland causing non-axial proptosis. Complete removal of mass has an excellent prognosis with complete resolution of symptoms., (© NEPjOPH.)

Purpose: Pleomorphic adenoma (mixed tumor) is the most common neoplasm of the parotid gland and one of the most frequent types of salivary gland tumor, generally with benign behavior and relatively slow growing. The adenomas could arise from the superficial, deep or from both superficial and deep parotid's lobes., Methods: The aim of this review is to retrospectively analyze the surgical management of patients with pleomorphic adenoma of the parotid gland performed at the Department of Otorhinolaryngology (Department of Sense Organs of "Azienda Policlinico Umberto I" in Rome), from 2010 to 2020, with a focus on the percentage of recurrence and on the complication related to surgery to suggest an optimal diagnostic and therapeutic algorithm for patients with recurrent pleomorphic adenoma. The analysis of the complications observed in case of different surgical approaches was performed using the X 2 test., Results: The choice of a surgical approach (superficial parotidectomy-SP, total parotidectomy-TP, extracapsular dissection-ECD) depends on several elements, such as the location and the size of the adenoma, the availability of existing technical facilities and the professional experience of the surgeon. A transient facial palsy was present in 37.6%, 2.7% reported a permanent facial nerve palsy, 1.6% developed a salivary fistula, 1.6% a post-operative bleeding and 2.3% showed Frey Syndrome., Conclusion: The surgical management of this benign lesion is required, even in asymptomatic cases, to prevent the progressive growing and to reduce the risk of malignant transformation. The goal of surgical excision is to obtain the complete resection to minimize the risk of tumor recurrence and avoiding facial nerve disability. Therefore, an accurate preoperative study of the lesion and the choice of the most appropriate surgical treatment are essential to minimize the rate of recurrence., (© 2023. The Author(s).)

Background: Pleomorphic Adenoma (PA) and Basal cell adenoma (BCA) are benign salivary gland tumors that may pose a diagnostic challenge if typical features are not present. Due to the increased relapse and malignant transformation rate of the former, a correct diagnosis carries relevant prognostic information. Even though immunohistochemistry (IHC) plays a limited role in the diagnosis of these tumors, the use of IHC panels could increase diagnostic accuracy. In the present work, we aimed to demonstrate that the use of an IHC panel consisting of Glial Fibrillary Acid Protein (GFAP), B-Catenin and Discovered On GIST 1 (DOG-1) can aid in the differential diagnosis between PA and BCA., Methods: We analyzed 18 cases of benign salivary gland tumors (Pleomorphic adenomas and Basal cell adenomas) with overlapping histologic features. First, a head and neck pathologist diagnosed the cases relying on morphology alone. Afterwards, cases were re-evaluated considering the IHC panel results. Inter-observer IHC scoring concordance was evaluated with pre-defined marker cut-off points using Cohen's Kappa scores., Results: Based on morphology alone, 9 cases were classified as PA while the remaining tumors were considered to be BCA. Five out of nine BCA cases showed GFAP staining and absent nuclear B-catenin and DOG-1 positivity. Conversely, 2 PA cases showed absent GFAP and positive nuclear B-catenin with concurrent DOG-1 expression. Therefore, after IHC evaluation, up to 40% of morphologic diagnoses were reconsidered. Overall, the inter-observer concordance for IHC evaluation was good (resulting Kappa Scores between 0.78 and 1)., Conclusion: Our work supports the use of a concise IHC panel to improve the diagnostic accuracy of benign salivary gland tumors with overlapping histologic features., (© 2022. The Author(s).)

Mammary pleomorphic adenoma is a biphasic tumor, characterized by epithelial-myoepithelial components with myxochondroid stroma, resembling the prototypic pleomorphic adenoma of the salivary glands. We report the multiple diagnostic pitfalls raised by a mammary pleomorphic adenoma, initially diagnosed as mucinous carcinoma on fine-needle aspiration (FNA) cytology and invasive carcinoma on needle core biopsy. The final diagnosis was made on the surgical specimen. As the term "pleomorphic" suggests, this tumor can present various phenotypes, some of which might be misleading on both FNA cytology or needle core biopsy. Rearrangements in PLAG1 and HMGA2 genes have not been detected in our patient. Mammary pleomorphic adenoma is considered a benign tumor, despite rare local recurrence and malignant behavior. Its correct identification, despite the difficulties, is essential to avoid unnecessary aggressive treatment.

Objective: Fine needle aspiration (FNA) is a well-established tool in preoperative diagnosis of salivary gland lesions with diagnostic accuracy of 90%. Pleomorphic adenoma (PA) is the most common salivary gland tumor comprising 45%-74% of all salivary gland tumors with FNA diagnostic accuracy of 89.5%-96.2%. The aim of the present study was to determine and analyze potential cytomorphological pitfalls and evaluate the diagnostic accuracy in FNA diagnosis of PA., Methods: Salivary gland specimens with both cytological and histological diagnoses were searched over a 10-year-period (2009-2018) from a laboratory information system of Pathology Department, Fimlab Laboratories, Tampere and matched to determine concordant and discordant PA cases. Sufficient material in histological and cytological sample was found in 401 cases. In 218 cases (54.4%) diagnosis was true-negative PA, in 169 cases (42.1%) diagnosis was true-positive PA and there were 14 discordant cases: 4 false-positive cases and 10 false-negative cases. False-negative cases were reclassified and subgrouped according to The Milan System for Reporting Salivary Gland Cytopathology (MSRSGC)., Results: Cytomorphologically, cell type predominance was more often myoepithelial in true-positive cases (65%) and epithelial both in false-negative (70%, p = .007) and false-positive cases (75%, p = .027). Well-formed ducts were present in cytology in all true-positive cases (p < .001). Only 10% of true-positive cases did not show any matrix in cytology (p < .001). Nuclear changes were common in false-negative cases (80%, p = .002) and false-positive cases (75%, p = .003). Beneficial cell block (CB) was more common in true-positive cases (85%) than in false-negative cases (50%, p = .041) or in false-positive cases (50%, p = .116) and a lack of beneficial CB led more often to a false diagnosis (70% false diagnosis without beneficial CB versus 29% false diagnosis with beneficial CB)., Conclusion: The present study showed diagnostic accuracy of 96.5% for FNA in PA diagnosis. Sensitivity, specificity, positive predictive value and negative predictive value were 94.4%, 98.2%, 97.7%, and 95.6%, respectively. The benefit of CBs was more evident in true-positive cases (85%)., (© 2023 Wiley Periodicals LLC.)

Background: Very little is currently known about molecular alteration of matrix-producing carcinoma of the breast. However, the morphological similarity with other neoplasm with a myxo-chondroid component is remarkable. In this pilot study we evaluated the molecular alterations involving PLAG1 and MYC genes in 12 cases of matrix producing carcinoma., Methods: We evaluated PLAG1 rearrangements as Break-Apart and Gene Copy Gain, and MYC as amplification and polysomy in 12 cases of matrix producing carcinoma using a FISH method., Results: Among the 12 cases of matrix producing carcinomas we found that the three cases harboring MYC amplification were all negative for PLAG1 break-apart; four cases with MYC polysomy were associated to PLAG1 break-apart and high Gene Copy Number; among four cases wild type for MYC , three showed a PLAG1 - break-apart signal and of them two died with disease. One of the deceased patients showed an amplification of MYC with PLAG1 - wild-type and the other showed a PLAG1 break-apart (6%) and a MYC wild-type., Conclusion: This is the first report to the best of our knowledge that shows a possible correlation between a matrix producing carcinoma with PLAG1 and MYC involvement in the development and progression of this kind of tumor. We can suppose that MYC amplification behaves in an aggressive way together with PLAG1- break-apart in the cases of matrix producing carcinoma presented here. The gene copy gain is a useful diagnostic tool in the case of difficult diagnosis because an increase was observed in more than 50% of cases.

Rationale: Salivary gland tumors account for approximately 3% of all tumors, most of which are benign, with pleomorphic adenomas being the most common, occurring mostly in middle-aged women, mostly originating from the major salivary glands and, to a lesser extent, from the minor salivary glands, with the tongue being a very rare site of occurrence. To date, case reports of pleomorphic adenoma at the root of the tongue are also rare., Patient Concerns: A 56-year-old male patient with no obvious cause of foreign body sensation in the pharynx, sputum, no pain, no blood in the sputum, no dysphagia, and no difficulty in swallowing and breathing, which was significantly aggravated in the past 2 weeks, with difficulty in swallowing, breath-holding on lying down., Diagnoses: computed tomography and magnetic resonance imaging revealed a soft tissue mass at the root of the left tongue, which involved the tongue body in the forward direction. Electronic laryngopharyngoscopy showed a left-sided tongue root mass with a poorly smooth mucosa, covered with a mucous white pseudomembrane and a localized brownish-black crust without active bleeding. The final pathological findings showed a pleomorphic adenoma., Interventions: Postoperative symptomatic treatment was given, and the patient recovered well. Eight days after surgery, the patient was discharged from the hospital, and the pharyngeal pain basically subsided at the time of discharge, with no fever and no pharyngeal discomfort. Postoperative laryngoscopy showed smooth mucosa of the pharyngeal cavity, good pseudomembrane formation in the operated area, no active bleeding, no purulent secretions, and normal blood routine on recheck. The medical advice after discharge was firstly, full rest for 1 week, secondly, continue the oral anti-inflammatory treatment, 1 week after the operation need to review the outpatient clinic, finally, if there are any uncomfortable symptoms, seek medical attention in time., Outcomes: At present, the patient has been followed up for half a year and has recovered well from the operation without any discomfort., Lessons: It is very rare to find a pleomorphic adenoma of the tongue, and it occurs mostly in middle-aged women. In clinical diagnosis, it is sometimes difficult to distinguish it from malignant tumor of the tongue., Competing Interests: The authors have no funding and conflicts of interest to disclose., (Copyright © 2023 the Author(s). Published by Wolters Kluwer Health, Inc.)

Background: Salivary gland tumors (SGT) are a diverse group of neoplasms arising from the major and minor glands. The oral cavity is the most common site for minor SGT (IMSGT), and these lesions frequently pose a challenge to the pathologist due to overlapping histopathological features and limited material for analysis. Our objective was to determine specific clinical and histopathological features associated with challenges in IMSGT diagnoses and pathologists' agreement., Methods: We conducted a retrospective analysis of 248 IMSGT received between 2010 and 2019. We evaluated the diagnostic challenge of the cases by stratifying according to whether a definitive, favored, or indeterminate (challenging) diagnosis was provided. Inter-observer agreement and concordance of biopsy diagnoses with the final diagnoses after tumor resection were evaluated., Results: Of the 248 biopsies, 191 had a definitive diagnosis, 38 favored diagnoses, and 19 were indeterminate. The predominant diagnoses considered for the indeterminate category were pleomorphic adenoma/myoepithelioma (PA), polymorphous adenocarcinoma (PAC), adenoid cystic carcinoma (AdCC), and low-grade adenocarcinoma. Using multivariate analysis of clinical features, younger patient age, smaller tumor size, and larger biopsy size increased the likelihood of a definitive diagnosis (p = 0.014, p = 0.037, p = 0.012). The inter-observer agreement for 68 representative cases was moderate overall (Fleiss's Kappa 0.575) and good for the 40 cases with a definitive diagnosis (Fleiss's Kappa 0.66). Sixty-five biopsy diagnoses were matched with corresponding tumor resection diagnoses and found to show a good concordance (Cramer's V test 0.76). The discordant diagnoses predominantly involved PA, carcinoma exPA, PAC, AdCC, and adenocarcinoma NOS., Conclusion: Diagnostic challenges in IMSGT incisional biopsies were infrequent, especially if multiple pathologists were consulted. PA, PAC, AdCC, and adenocarcinoma NOS were the histologic types more commonly posing diagnostic challenges. Younger patient age, smaller tumor size, and larger biopsy are associated with a definitive diagnosis. This data highlights the importance of appropriate sampling in IMSGT., (© 2022. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

A case of a 64-year-old woman is reported, who developed new-onset pain over a preexisting area of right mandibular fullness. Clinical examination, MRI, and fine-needle aspiration cytology confirmed the diagnosis of a benign parotid gland tumor-pleomorphic adenoma, which was treated by total parotidectomy with complete removal of the tumor. When evaluating a patient with orofacial pain, oral health care providers should be cognizant of all potential differential diagnoses, especially in the setting of red flags such as persistent or enlarging facial swelling/fullness., Competing Interests: Disclosure The authors do not have any disclosures. The authors do not have any conflicts of interest associated with this publication, and there has been no financial support for this work that could have influenced its outcome. This manuscript is original, has not been previously published, and is not currently under consideration by another journal., (Copyright © 2022 Elsevier Inc. All rights reserved.)

Carcinoma ex pleomorphic adenoma (Ca ex PA) is defined as an infiltrative carcinoma arising from a primary or recurrent benign pleomorphic adenoma. Ca ex PA with the histologic subtype mucoepidermoid carcinoma is extremely uncommon. Only 2 previous reports in the lacrimal gland have been documented. We reported a 55-year-old lady with a firm, solid, nontender mass in the supralateral quadrant of the right orbit at the area of the lacrimal gland. After wide excision of mass, pathology revealed high-grade mucoepidermoid carcinoma Ex pleomorphic adenoma. To reduce tumor recurrence 60 Gray radiation was delivered to the orbital cavity. She is still tumor-free 1 year after completion of treatment., Competing Interests: The authors have no financial or conflicts of interest to disclose., (Copyright © 2022 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Aims: To estimate the proportion of de novo lacrimal gland pleomorphic adenomas (PAs) and carcinomas expleomorphic adenomas (CEPAs), together with age at presentation and first symptom. Conjectural models of tumor growth are considered., Methods: Retrospective review of patients with orbital lobe PA or CEPA. The presenting age was examined for conformation to a Gaussian distribution and the cumulative distribution function derived for both tumor types. The risk of CEPA with age was estimated by logistic regression., Results: About one-sixth (27/172; 16%) of these primary orbital lobe tumors were CEPAs, with 145 PAs (76/145 male; 52%) and 27 CEPAs (12/27 male; 44%). The mean presenting age for PAs was 48.3 years (median 47.7; range 11-84 years) and 57.7 years for CEPAs (median 61.2, range 27-91 years) ( p = 0.0062), and the standard deviations for each group are almost identical (16.3 for PAs, 15.9 for CEPAs; p = 0.92). Five (3.4%) PAs and 1 (3.7%) CEPA were asymptomatic: otherwise, the median symptom duration was 24 months for both PAs and CEPAs ( U test: p = 0.65). The odds of CEPA rises significantly with age, increasing 1.04-fold annually ( p = 0.0079)., Conclusion: The almost identical measures of dispersion for the presenting ages of PA and CEPA suggests that, once malignant transformation occurs, there might be a relatively constant period before it is evident. CEPAs present about a decade after PAs, this unexpectedly later presentation for the malignancy possibly being explained by a gradual replacement of the PA by the newly arising carcinoma within the preceding benign tumor., Competing Interests: The authors have no conflicts of interest to disclose., (Copyright © 2022 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Cystic salivary gland cytology can be challenging due to the fact that a cystic mass can be the clinical presentation of both non-neoplastic and neoplastic conditions. Neoplastic lesions consist of both benign and malignant neoplasms. The cytomorphologic features of these entities can overlap and the cystic background may additionally contribute to the complexity of these lesions and their interpretation. Ancillary studies have been reported in several studies to be beneficial in further characterization of the cellular components and subsequent diagnosis of the cystic lesions of the salivary gland. Fluorescence in situ hybridization, real-time polymerase chain reaction, and next-generation sequencing are now being utilized to detect molecular alterations in salivary gland neoplasms. MALM2 rearrangement is the most common gene fusion in mucoepidermoid carcinoma. PLAG1 rearrangement is present in more than half of pleomorphic adenomas. AKT1:E17K mutation is the key diagnostic feature of the mucinous adenocarcinoma. NR4A3 overexpression is highly sensitive and specific for the diagnosis of acinic cell carcinoma. MYB fusion is noted in adenoid cystic carcinoma. ETV6:NTRK3 fusion is helpful in diagnosis of secretory carcinoma. p16 and human papillomavirus (HPV) studies differentiate HPV-related squamous cell carcinoma from non-HPV-related neoplasms with overlapping features. NCOA4:RET fusion protein is the main fusion in intraductal carcinoma., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

Chondroid syringoma is a rare benign adnexal tumor and tends to occur in the head and neck region. Involvement in the axilla is very unusual, and the differential diagnosis of such presentations includes lymphadenopathy and cyst. Fine needle aspiration cytology (FNAC) is a very useful tool for the diagnosis of chondroid syringoma. The characteristic feature of chondroid syringoma in cytology is the presence of distinct biphasic cell populations of epithelial and myoepithelial cells in the chondromyxoid stroma. If the typical biphasic cellular and chondromyxoid stromal elements are not visible in smears, it may be misdiagnosed in cytology. Here, we describe a case of axillary chondroid syringoma that was initially misdiagnosed as a metastatic carcinoma by FNAC. Although chondroid syringoma rarely occurs in the axilla, it should be included among the differential diagnoses of an axillary mass. Cytopathologists need to discern the unique cytological features of chondroid syringoma and avoid misdiagnosis for prompt management of the patient., (© 2022 Wiley Periodicals LLC.)

Abstract: Salivary gland tumors are rare, accounting for 6% to 8% of head and neck tumors. Detecting them in primary care is important so that adequate treatment can be provided. This case report describes a 58-year-old woman whose unilateral pulsatile tinnitus and ear fullness led to the diagnosis of a salivary gland tumor, specifically a pleomorphic adenoma of the parotid gland., (Copyright © 2022 American Academy of Physician Associates.)

Salivary gland tumors are diagnostically challenging owing to the morphological diversity within any tumor type and overlapping histomorphology and immunohistochemistry amongst different tumours. In past two decades, rapid progress has been made in the field of understanding the pathogenesis of these tumours with the discovery of many tumour specific translocations and rearrangements. This includes CRTC1-MAML2 and CRTC-MAML2 in mucoepidermoid carcinoma, MYBNFIB and MYBL1-NFIB fusions in adenoid cystic carcinoma, PLAG1 and HMGA2 in pleomorphic adenoma, ETV6-NTRK3 in secretory carcinoma, NR4A3 rearrangements in acinic cell carcinoma, PRKD1 mutations in polymorphous adenocarcinoma and EWSR1-ATF1 in clear cell carcinoma. This review is a lens for progress made till date in the molecular pathology of salivary gland tumours with a special focus on their role as diagnostic tools and implications on clinical management of the patient as prognostic and predictive markers., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Pleomorphic adenoma of the salivary gland is a well-established and recognized diagnosis; however, pleomorphic adenomas of the breast are rare and can be difficult to diagnose on cytology or intraoperatively. We report a case that was misdiagnosed on a fine needle aspiration and frozen section. The pitfalls, differential diagnoses, and current management are discussed., (© 2022 Wiley Periodicals LLC.)

Myoepithelial carcinoma (MECA) is a rare salivary gland (SG) neoplasm (0.1-0.45% of all SG tumors) that often presents with bland cytomorphology and can be misclassified as cellular pleomorphic adenoma (PA) or myoepithelioma. This is particularly challenging in MECA ex-PA cases, especially if tumor shows minimal to no capsular invasion. We report a rare case of a 76-year-old female; history of left superficial parotidectomy with diagnosis (outside hospital) of cellular PA, who re-presented 9 months post surgery with enlarging left parotid mass, neck lymphadenopathy and facial nerve deficits. FNAB of parotid and neck lymph node revealed cellular aspirates with loosely cohesive clusters of myoepithelial cells with occasional chondromyxoid stroma. Prior resection slides were reviewed, and diagnosis of MECA ex-PA was made. Patient underwent left radical parotidectomy, selective neck dissection, with facial nerve sacrifice (due to extensive encasing by tumor). Histology showed a multinodular tumor with pushing borders, zonal arrangement comprising of a hypocellular, necrotic/myxoid center, and a peripheral rim of myoepithelial cells, confirmed by positive S100, and p63. Tumor extensively infiltrated peri parotid soft tissues with multiple foci of lymphovascular and perineural invasion; and metastatic neck lymph nodes. Next generation sequencing revealed a novel TERT promoter mutation (c.-124C > T), not usually described in SG neoplasms. Further, PD-L1 immunohistochemistry showed positive expression, making patient eligible for anti-PDL-1 immunotherapy. This case highlights importance of recognizing the subtle malignant features of MECA in distinguishing it from benign mimics like PA. In addition, presence of TERT mutation opens a new arena for future research to explore potential treatment targets., (© 2021. The Author(s), under exclusive licence to Springer Science+Business Media, LLC, part of Springer Nature.)

Chronic granulomatous inflammation occurs rarely alongside pleomorphic adenomas of the major salivary glands but would not appear to have been reported with lacrimal gland adenomas. We describe the clinical features, imaging and histopathology for 4 patients (3 female) who had granulomatous inflammation alongside lacrimal gland adenomas-the patients being with age 39, 44, 48, and 53 years at time of surgery. One patient had an asymptomatic lesion found on imaging, and the other 3 had symptoms for between 3 years and several decades. Conjecturally, this rare phenomenon might arise from an inflammatory response to leakage of secretions from the ductular components of the glands., (Copyright © 2021 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc.)

Objective: Squamous metaplasia is not uncommonly observed in salivary gland neoplasms, including pleomorphic adenomas (PAs). It poses as a diagnostic pitfall with cytomorphologic features overlapping with low-grade epithelial malignances and carcinoma ex-PA. We report series of aspirates of PAs with squamous metaplasia and review the literature for the clinicopathological and cytologic features of this diagnostically challenging entity., Methods: Three cases including index aspiration cytology, post-aspiration specimen cytology, cell block immunohistochemistry (IHC), histologic correlation, and clinical data were reviewed. Literature search was performed for cases of PAs with squamous metaplasia, yielding 22 case reports including 11 with aspiration cytology performed., Results: Metaplastic squamous components were seen in all index aspirations, but foreign body reaction and cystic changes were observed only after aspiration or in irritated lesions. PLAG1 IHC demonstrated reliable immunoreactivity in cell block preparations. Summarizing cases in this series and in the literature, cytologic features reported were variable and no consistent diagnostic feature was identified. Most reported cases were initially diagnosed as suspicious or malignant, but all ran a benign clinical course. Significant morbidity was reported only in one case due extensive surgery based on a malignant diagnosis., Conclusions: Squamous metaplasia in PAs is not associated with adverse outcomes, but may induce reactive changes upon aspiration/irritation, resulting in worrisome cytologic features. PLAG1 IHC is a useful adjunct when characteristic cytologic features for PAs are absent or obscured. Prudent use of the diagnostic category salivary gland neoplasm of uncertain malignant potential (SUMP) in the Milan system can avoid overtreatment., (© 2021 Wiley Periodicals LLC.)

Objective: Salivary gland tumors are a heterogeneous group of neoplasms with complex clinicopathologic behavior. These tumors often show an extremely diverse morphology and significant histologic patterns, making diagnosis a difficult task. Owing to the considerable differences in prognosis, treatment, and follow-up, accurately distinguishing these histologic mimickers is imperative. The aim of the present systematic review was to establish the diagnostic utility of p63/p40 immunostaining in histologic differentiation of salivary gland tumors., Study Design: An electronic search was carried out using MEDLINE by PubMed, Scopus, Google Scholar, Trip, Cochrane Library, and EMBASE databases. Articles in which both p63 and p40 immunohistochemical expressions were assessed were included for systematic review and their sensitivity and specificity were calculated. Risk of bias was analyzed for each study using the Quality Assessment of Diagnostic Accuracy Studies tool., Results: Ten eligible articles were included in the quantitative synthesis, which revealed a concordant positivity of p63 and p40 by nearly all cases of adenoid cystic carcinoma, pleomorphic adenoma, and mucoepidermoid carcinoma and most polymorphous adenocarcinomas showed discordant immunohistochemical expression (p63+/p40-)., Conclusions: The current evidence supports that integrated immunostaining of p63/p40 is a valuable adjunct for discerning enigmatic salivary gland tumors with true myoepithelial and/or squamous differentiation., (Copyright © 2021 Elsevier Inc. All rights reserved.)

PLAG1 rearrangements have been described as a molecular hallmark of salivary gland pleomorphic adenoma (PA), carcinoma ex pleomorphic adenoma (CEPA), and myoepithelial carcinoma (MECA). Several fusion partners have been described, however, commonly no further assignment to the aforementioned entities or a morphological prediction can be made based on the knowledge of the fusion partner alone. In contrast, TGFBR3-PLAG1 fusion has been specifically described and characterized as an oncogenic driver in MECA, and less common in MECA ex PA. Here, we describe the clinicopathological features of three TGFBR3-PLAG1 fusion-positive salivary gland neoplasms, all of which arose in the deep lobe of the parotid gland. Histopathology showed high morphological similarities, encompassing encapsulation, a polylobular growth pattern, bland basaloid and oncocytoid cells with myoepithelial differentiation, and a distinct sclerotic background. All cases showed at least limited, unusual foci of minimal invasion into adjacent salivary gland tissue, including one case with ERBB2 (Her2/neu) amplified, TP53 mutated high-grade transformation, and lymph node metastases. Of note, all cases illustrated focal ductal differentiation. Classification remains difficult, as morphological overlaps between myoepithelial-rich cellular PA, myoepithelioma, and MECA were observed. However, evidence of minimal invasion advocates classification as low-grade MECA. This case series further characterizes the spectrum of uncommon cellular myoepithelial neoplasms harboring TGFBR3-PLAG1 fusion, which show recurrent minimal invasion of the adjacent salivary gland tissue, a predilection to the deep lobe of the parotid gland, and potential high-grade transformation., (© 2021 The Authors. Genes, Chromosomes and Cancer published by Wiley Periodicals LLC.)