Clinical behavior of recurrent pleomorphic adenoma in the palate: a systematic review.

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.

Molecular Factors in Carcinoma Ex Pleomorphic Adenoma: Systematic Review and Meta-Analysis.

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.

A Large Pleomorphic Adenoma in Nasal Cavity Misdiagnosed as Inverted Papilloma.

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.

Pleomorphic adenoma with extensive squamous and mucinous metaplasia and a novel MALAT1::PLAG1 fusion gene.

Cases of metaplastic pleomorphic adenoma can be diagnostically challenging. Many of these cases fall into the Milan system's SUMP category, and some may be misdiagnosed. The author shows a case of pleomorphic adenoma with extensive squamous and mucinous metaplasia and a novel MALAT1::PLAG1 fusion.

Chondroid Syringoma on the Tip of the Nose: A Case Report.

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.

Analysis of clinical features of 7 cases of primary pleomorphic adenoma of lower respiratory tract and review of literature.

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.

Hyaline cell-rich chondroid syringoma: A potential pitfall on cytology.

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.

Basal cell adenoma and pleomorphic adenoma of the parotid gland: a single center experience.

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.

Clinicopathological characteristics of pleomorphic adenoma from the labial gland: A 20-year experience with 173 cases in one single institution.

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.

Cytopathology of salivary gland myoepithelial carcinoma: A study of 13 cases and review of the literature.

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.

Updated Salivary Gland Immunohistochemistry: A Review.

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.

Clinico-histopathological review of 255 patients who underwent parotidectomy for pleomorphic adenoma: a 10-year retrospective study-a proposal for an optimal diagnostic and therapeutic algorithm for patients with recurrent pleomorphic adenoma.

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 X2 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.

Pleomorphic adenoma of the tongue: A case report.

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.

Chondroid syringoma of an upper eyelid tumor: Unusual case report.

We present the case report of a 58-year-old man with recurrent chondroid syringoma, which was histopathologically confirmed, who underwent exenteration surgery of the right eye. Furthermore, the patient was receiving postoperative radiation therapy, and presently there is no local and/or distant evidence of disease in the patient.

The diagnostic accuracy of pleomorphic adenoma in fine needle aspiration: Features leading to false positive and false negative diagnoses and the utility of cell blocks.

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%).

A challenging diagnosis of mammary analogue secretory carcinoma (MASC) on fine needle aspiration cytology and cell block: A cytopathologist's perspective.

Mammary analogue secretory carcinoma (MASC) is a recently described salivary gland carcinoma that resembles the secretory carcinoma of the breast and is characterised by t(12;15) (q13;q25) translocation, which results in an ETV6-NTRK3 gene fusion product. On cytomorphology, it is characterised by papillary fragments, clusters, and singly dispersed tumour cells. These tumour cells are large and have abundant vacuolated cytoplasm. Acinic cell carcinoma of the salivary gland is the most common differential diagnosis of MASC. Other differentials include mucoepidermoid carcinoma, salivary duct carcinoma, pleomorphic adenoma, and oncocytic salivary gland neoplasms. Immunohistochemistry and morphology are critical in establishing the correct diagnosis. We present a case of a 46-year-old male patient diagnosed as MASC of the parotid gland on fine needle aspiration cytology and cell block.