Metastatic salivary gland neoplasms to pleural effusion: diagnostic challenges and prognostic significance in a series with 9 patients.

INTRODUCTION: Pleural effusions can present a diagnostic challenge as they are not always caused by malignancy in patients with a history of typical visceral primaries. MATERIAL AND METHODS: At 2 major academic medical centers, we have identified several cases in which salivary gland neoplasms metastasized to pleural effusions in patients who have been aggressively managed with various treatment modalities including chemotherapy, radiation, and/or surgical excision. RESULTS: Herein, we present a range of primary salivary gland tumors that metastasized to serous effusions and characterize their cytomorphology, immunoprofiles, and clinical courses. Our case series shows that many tumor types metastasize to pleural effusions and they present unique diagnostic challenges in each case. We found that metastasis of a salivary gland neoplasm to a pleural effusion is a late-stage event and is often associated with poor prognosis. CONCLUSIONS: This series serves as a resource to demonstrate the cytomorphologic and immunohistochemical features of malignant pleural effusions due to salivary gland neoplasms and draws attention to poor prognosis in cases of salivary duct carcinoma, mucoepidermoid carcinoma and adenoid cystic carcinoma.

Sclerosing mucoepidermoid carcinoma of the salivary glands: report of three cases with special concern to the counterpart accompanied by eosinophilia.

Sclerosing mucoepidermoid carcinoma (SMC) is described as a "sclerosing variant" of mucoepidermoid carcinoma, and it is characterized by dense fibrosis and sclerosis of the stroma. SMC with eosinophilia (SMCE) is another and more rare subtype characterized by eosinophilia in addition to the sclerotic stroma common to SMC. However, unlike SMC, SMCE is not listed in the current 4th edition of WHO classification. Here, we describe three cases: one SMC in the parotid gland, one SMCE in the submandibular gland and one SMCE in the minor salivary gland of the oral cavity. The patients included a 71-year-old Japanese male, a 74-year-old Japanese female, and an 81-year-old Japanese female. They each complained of mass formation and underwent surgical resection. Histologically, the tumors mainly consisted of squamous cells with scarce keratinization that formed irregular large and small nests along with cystic structures containing mucous cells against the background of sclerotic stroma. One oral SMCE showed fine nesting and trabecular invasion. The two SMCEs included dense aggregates of eosinophils as well as more prominent lymphoid infiltration. Fluorescence in situ hybridization for MAML2 confirmed split signals in SMC, but not in SMCE.

Myotonic dystrophy and recurrent pleomorphic adenomas: Case report and association hypothesis.

We report a case of a patient with concurrent myotonic dystrophy and recurrent pleomorphic adenoma and hypothesize the association between both diseases. A 58-year-old man with classic myotonic dystrophy type 1 was diagnosed with pleomorphic adenoma. Appropriate treatment was commenced. Massive recurrences occurred within 15, 28 and 22 months respectively, after repeated surgical removal. Three case reports on similar occurrences of synchronous myotonic dystrophy and pleomorphic adenoma are discussed and an association between both disease entities is hypothesized. A conceivable association between myotonic dystrophy and pleomorphic adenoma is hypothesized by upregulation of the Wnt/Beta-catenin signaling pathway, initiated by a decreased expression of microRNA, pleomorphic adenoma gene 1 induced Beta-catenin accumulations and alterations in tumor suppressor genes and oncogenes due to RNA processing defects induced by the expanded repeat in the DMPK gene.

Laboratory-Proven Asymptomatic SARS-CoV-2 (COVID-19) Infection on 18F-FDG PET/CT.

An 87-year-old man with primary salivary duct carcinoma and hepatic metastases was referred for restaging. F-FDG PET/CT demonstrated new FDG-avid ground-glass opacities with intralobular septal thickening in both lungs with a peripheral dominant distribution. This was interpreted as a pattern highly suggestive of COVID-19 pneumonia, and the referring physician was contacted. The patient was currently asymptomatic and without clinical evidence of active pneumonia. However, the patient tested positive for COVID-19 using real-time reverse transcription-polymerase chain reaction. This case demonstrates that even asymptomatic outpatients undergoing PET/CT should be evaluated for radiologic findings suggestive of COVID-19 during this pandemic.

Paraneoplastic manifestations of salivary gland tumours: A case report and review.

Salivary gland cancers are an uncommon and heterogenous group of malignancies, accounting for approximately 3% of head and neck tumors. We describe a case of a patient who presented with paraneoplastic Cushing's syndrome secondary to metastatic salivary ductal carcinoma (SDC). Paraneoplastic ACTH secretion initially responded to chemotherapy with complete resolution of clinical symptoms. To our knowledge, this is the first described case of an ACTH-secreting SDC. We also review other paraneoplastic syndromes (PNS) that have been reported in association with salivary gland cancers.

Xanthogranulomatous Sialadenitis, an Uncommon Reactive Change is Often Associated with Warthin's Tumor.

Xanthogranulomatous sialadenitis (XGS) is rare in salivary glands and only reported in the literature as single cases. Here we report a cohort of four cases with XGS and summarize the clinicopathologic features of these cases. All four patients had persistent mass lesions concerning for neoplasm. In two patients (patient 1 and 3), the initial fine needle aspirations (FNAs) contained oncocytic cells consistent with or suspicious for Warthin's tumor, but follow-up FNAs showed only inflammation and/or debris indicating tumor infarction after FNA. All patients eventually had surgical resection. Histologically, all cases contained abundant macrophages with necrosis and fibroblastic proliferation. Warthin's tumor with a grossly identifiable tumor nodule (0.7 cm) was noted in patient 1 and a microscopic focus (0.2 cm) of Warthin's tumor was identified in patient 3. No identifiable tumor was observed in patient 2 and 4. There are a total of 10 XGS cases in the literature (including four from this series) and Warthin tumor was identified in 50% of reported cases of XGS, suggesting that XGS is an uncommon reactive process to spontaneous or procedure-induced infarction of Warthin tumor. As a diagnostic mimicker for malignancy, a thorough examination and generous sampling of surgical resection specimen is warranted, although a benign salivary gland neoplasm, commonly Warthin's tumor, is often identified.

Nasopharyngeal Salivary Gland Anlage Tumour: A rare cause of neonatal respiratory distress.

A salivary gland anlage tumour (SGAT) is a very rare type of benign tumour that usually presents in early infancy with respiratory distress which is exacerbated upon feeding. We report a full-term male neonate who was referred to the Al Nahdha Hospital, Muscat, Oman, in 2015 with severe neonatal respiratory distress due to a nasopharyngeal obstruction immediately after birth. Computed tomography and magnetic resonance imaging revealed a well-circumscribed mass in the nasopharynx, without intracranial extension. Histopathological analysis of the lesion confirmed a diagnosis of SGAT. Following excision of the tumour, the postoperative period was uneventful. No recurrence was observed over the next two years. This case report highlights the importance of the early recognition of this extremely rare and potentially life-threatening, yet easily curable, condition.

[Research progress of salivary glands mucosa-associated lymphoid tissue lymphoma].

Salivary glands mucosa-associated lymphoid tissue lymphoma (SGML) is a distinct subtype of marginal zone B-cell type non-Hodgkin's lymphoma (NHL), which is commonly seen in middle aged females. SGML is usually associated with autoimmune diseases such as Sjögren's syndrome or with chronic infection such as hepatitis C virus (HCV) infection. Chromosomal abnormalities are frequently seen in SGML, which usually activate nuclear factor-κB molecular pathway to modulate cell survival and proliferation, resulting in lymphoma occurrence. SGML tends to arise from parotid gland, presenting frequently as a localized and indolent lesion, a long-term follow-up and biopsy are needed for accurate diagnosis. Surgery, radiotherapy and chemotherapy are usually effective disseminated diseases at multiple sites need combined treatment. SGML has a relatively better prognosis with a higher relapse rate than other types of NHLs, dissemination or higher degree of malignant transformation may occur. Thus, a long-term and close follow-up is essential for patients with SGML.

Tongue Atrophy in Sjögren Syndrome Patients with Mucosa-associated Lymphoid Tissue Lymphoma: Autoimmune Epithelitis beyond the Epithelial Cells of Salivary Glands?

OBJECTIVE: Primary Sjögren syndrome (pSS), an autoimmune epithelitis, bears the risk of evolving to non-Hodgkin lymphoma and most frequently to the mucosa-associated lymphoid tissue (MALT) subtype. Based on the observation that pSS patients with MALT present a more atrophic and more intensely fissured tongue, we aimed to semiquantify severity of tongue atrophy and clinically assess lingual appearance in pSS patients with and without MALT, and investigate whether tongue atrophy and fissured appearance could serve as clinical indicators/signs of MALT. METHODS: A blinded complete oral examination was performed in pSS patients with and without MALT. Tongue atrophy was scored using a semiquantified atrophy score. Clinical and laboratory variables were recorded for all patients. RESULTS: After excluding pSS patients with oral candidiasis, iron deficiency, and megaloblastic anemia, 19 pSS patients with salivary MALT were matched 1:3 for age, sex, and disease duration with 57 pSS patients without MALT. The pSS-MALT patients had increased prevalence of salivary gland enlargement, lymphadenopathy, monoclonal gammopathy, rheumatoid factor positivity, higher focus and Tarpley scores in the minor salivary gland biopsy, and hyposalivation, compared to the pSS non-MALT patients. A significantly higher prevalence of tongue atrophy (68% vs 30%, p = 0.006) and fissured tongue (89% vs 33%, p < 0.001) was observed in the former group. Multivariate analysis showed that fissured tongue appearance, hyposalivation, and lymphadenopathy associate independently with salivary MALT in pSS. CONCLUSION: These results suggest that pSS patients with lymphoid malignancy exhibit a more atrophic and more fissured tongue. This particular clinical tongue appearance can serve as an additional clinical sign for salivary MALT lymphoma in pSS patients.

Syncope caused by a pleomorphic adenoma: Case report and literature review.

Pleomorphic adenomas are considered the most common salivary gland tumors, although they rarely occur in the parapharyngeal space. To the best of our knowledge, this is the first case report of a parapharyngeal parotid pleomorphic adenoma causing syncope. A 57-year-old man was admitted for left-sided blurred vision, left-sided weakness, dysarthria, lightheadedness, and syncope. Upon his admission, an electrocardiogram showed sinus bradycardia, and computed tomography of the neck with contrast showed a large parapharyngeal mass involving the prestyloid compartment, leading to compression of blood flow through the internal carotid artery. The mass was biopsied via intraoral fine-needle aspiration, which revealed cytology consistent with pleomorphic adenoma. The mass was resected via a transcervical approach, and a total parotidectomy was performed. The patient's hypotensive and bradycardic episodes disappeared after surgery. Surgical pathology showed a benign pleomorphic adenoma with a hemorrhagic and necrotic center and without capsular invasion or malignant transformation.

Refractory Facial Paralysis: A Case Report.

PURPOSE: To present a case of salivary gland malignancy initially mimicking Bell's palsy. CASE REPORT: A 75-year-old woman with hypertension visited our neurological outpatient department,complaining of persistent right facial paralysis for more than a year after oral glucocorticoid therapy with recent development of vertigo and unsteady gait. She was previously diagnosed as having Bell's palsy and was prescribed oral glucocorticoid. However, her right facial muscles were still completely paralyzed, with no signs of improvement. The patient visited the outpatient department of neurology for 3 weeks, seeking treatment for the recent onset of vertigo and ataxia. Brain contrast magnetic resonance imaging (MRI) revealed the right mastoid air cells to be filled with high T2 signal intensity and low T1 signal, with destruction of the bony structure of mastoid, extending to the right jugular bulb. Results obtained from excisional biopsy and pathological analyses were used to diagnose the patient with adenoid cystic carcinoma of the salivary gland. The patient then received a thorough cancer workup and chemoradiotherapy, with the malignancy being under control. However, after a 1-year follow-up, the patient still had permanent right facial palsy. CONCLUSION: Salivary gland malignancy should be considered in patients with acute and subacute facial nerve paralysis, in addition to Bell's palsy. Brain imaging with contrast agents should be performed for differential diagnosis.

Predictive Factors and a Survival Score for Patients Irradiated for Metastatic Spinal Cord Compression from Carcinoma of the Salivary Glands.

AIM: To our knowledge, this is the first study focusing on metastatic spinal cord compression (MSCC) from carcinoma of the salivary glands. PATIENTS AND METHODS: Nine patients receiving radiation alone were evaluated for improvement of motor deficits, post-radiation gait function and survival. RESULTS: Of nine characteristics (radiation program, age, sex, additional metastases to bone or to other organs, dynamic of motor deficits, pre-radiation gait function, number of vertebrae affected by MSCC, general condition), strong trends were found for associations between improved motor deficits and their dynamic (p=0.05), post-radiation gait function and pre-treatment ambulatory status (p=0.08) and between survival and additional metastases to other organs (p=0.07), dynamic of motor deficits (p=0.07) and general condition (p=0.07). In addition, a survival score was created. Patients with 2-3 points had a significantly better 6-month survival than those with 0-1 points (100% vs. 0%, p=0.027). CONCLUSION: Characteristics predicting outcomes identified in this study and the new survival score can guide physicians when making treatment decisions.

Diagnóstico, tratamiento y seguimiento de un tumor de reciente descripción: el carcinoma análogo secretor de mama (MASC) de glándula salival. A propósito de 2 nuevos casos / Diagnosis, treatment and follow-up of a recently described tumour: Mammary analogue secretory carcinoma (MASC) of the salivary gland. A report of 2 new cases

Introducción. El carcinoma análogo secretor de mama (MASC) es un tumor de glándula salival de reciente aparición con pocos casos descritos. Tiene un diagnóstico preoperatorio difícil y su tratamiento y seguimiento todavía siguen en estudio. Material y métodos. Revisión sistemática de 367 tumores parotídeos intervenidos entre enero de 2011 y diciembre de 2015 en el Hospital Universitario Miguel Servet. De los 367 casos operados, 45 eran malignos, y de estos, encontramos 2 únicos casos de MASC. Resultados. Cuarenta y cinco de los 367 tumores parotídeos eran malignos, siendo 2 de ellos MASC. La parotidectomía subtotal y superficial asociada a la radioterapia ha sido el tratamiento de elección. Tras 12 y 18 meses, no se han evidenciado recidivas. Discusión. Revisión de los estudios publicados en la literatura sobre su diagnóstico diferencial con otros tumores de glándula salival y su tratamiento, teniendo en cuenta los resultados citológicos e inmunohistoquímicos de cada uno de ellos, y destacando cuáles son los marcadores más específicos para el diagnóstico de MASC. Conclusión. El diagnóstico preoperatorio de este tumor casi nunca es acertado, y todavía no se conoce su tratamiento y supervivencia a largo plazo, por lo que más estudios prospectivos y nuevos casos deben ser documentados y seguidos (AU)

Introduction. Mammary analogue secretory carcinoma (MASC) is a new salivary gland tumour with few cases reported. Pre-operative diagnosis is difficult, and treatment and follow-up are still under study. Material and method. A systematic review was conducted on 367 parotid tumours treated between January 2011 and December 2015 at the University Hospital Miguel Servet, Spain. Of the 367 cases operated on, 45 were malignant, and of these, only two cases were MASC. Results. Of the 367 parotid tumours reviewed, 45 were malignant, and two of them MASC. Sub-total and superficial parotidectomy combined with radiation therapy has been the treatment of choice. They showed no recurrence after 12 and 18 months. Discussion. A review is presented on the studies published in the literature on the differential diagnosis with other salivary gland tumours, taking into account their cytological and immunohistochemical results, focussing on the most specific markers for the diagnosis of MASC. Conclusion. Pre-operative diagnosis of MASC is rarely successful. Its treatment and long-term survival is largely unknown, thus there is a need for more prospective studies, as well as new cases that should be documented and followed-up (AU)

Repeated haemorrhages in peripheral nerve sheath tumours of the salivary glands after minor injury.

Repeated haemorrhages in peripheral nerve sheath tumours of the salivary glands are rare. We report the case of a patient with neurofibromatosis type 1 who had two episodes of massive haemorrhage in his right parotid gland the day after a minor injury. Oral and maxillofacial surgeons should be aware that vasculopathy may occur in patients with these tumours.

Socioeconomic disparities and comorbidities, not race, affect salivary gland malignancy survival outcomes.

OBJECTIVES/HYPOTHESIS: This study sought to determine whether comorbidities, race, and socioeconomic factors affect 5- and 10-year survival outcomes for patients with salivary gland malignancies treated at a single large academic institution with a large African American population. STUDY DESIGN: A retrospective cohort study was performed of all patients with salivary gland malignancies, from 1990 to 2015, at a large academic medical center. METHODS: Standard statistical analysis was performed using Kaplan-Meier survival curve analysis and Cox proportional hazard models. RESULTS: The overall 5- and 10-year survival rates decreased with age ≥ 60 years (P < .001), stage 3 or 4 (P < .001), clinical T stage 3 or 4 (P < .001), and clinical N stage 1, 2, or 3 (P < .001). Living in a ZIP code with an increasing proportion of residents with a high school degree or less (P < .05), being male (P < .05), increasing age at the time of diagnosis (P < .001), and increasing Charlson comorbidity index (P < .05) detrimentally impacted survival at 5 and 10 years. Race was associated with socioeconomic variables, but race was not a prognostic indicator of survival. CONCLUSIONS: Socioeconomic factors and comorbidities, not race, were negative prognostic indicators of survival of patients with salivary gland malignancies. Using race as a marker for socioeconomic status should be used with caution. LEVEL OF EVIDENCE: 4. Laryngoscope, 127:2545-2550, 2017.