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.

Prognostic value of comorbidities in patients with carcinoma of the major salivary glands.

This study aimed at evaluating the influence of comorbidities on outcome and survival in patients with carcinoma of the major salivary glands. Medical records of 109 patients with carcinoma of the major salivary glands treated at a single quaternary medical care and cancer center between 2003 and 2015 were reviewed. Comorbidities were classified according to the Age-Adjusted Charlson Comorbidity Index (ACCI) scoring system. 59 patients were males, 50 were females. Median age was 69 years and the median interval between tumor diagnosis and date of study inclusion was 71 months. Most carcinoma arose in the parotid gland (90 patients), and most patients presented with T 1 (25) or T 2 (37) cancer. Mean ACCI score was 2.9, and the most frequent ACCI score was 4. Mean overall survival was 119 months (ACCI 0-3) and 55 months (ACCI score >4), respectively (p = 0.005). Mean disease-free survival (ACCI 0-3) was 110 and 58 months (ACCI > 4), respectively (p = 0.02). Survival was significantly improved in low grade tumors, lower T and UICC stage and lymph node-negative patients. Sex, age, history of smoking and resection margins had no influence on overall survival. High comorbidity is an independent risk factor on overall and disease-free survival in patients with major salivary gland carcinoma. This is the first study evaluating comorbidity using the ACCI scoring system in this group of patients.

Carcinoma Ex Pleomorphic Adenoma of the Tongue: Difficulty in Diagnosis Between Metastasis of Breast Cancer and Salivary Tumor.

Carcinoma ex pleomorphic adenoma (CEPA) is a carcinoma that shows histologic evidence of arising in or from a benign pleomorphic adenoma. Carcinoma ex pleomorphic adenoma often occurs in parotid glands, but is extremely rarely in the tongue. A 53-year-old Japanese woman was referred to the Department of Oral and Maxillofacial Surgery, Nagasaki University Hospital, because of tumor of the right dorsum tongue. She had a history of surgery of breast cancer (invasive ductal carcinoma) and it was disseminated to the lung and bone. Macroscopic examination revealed an oval tumor with a smooth mucosal surface of 10 mm in diameter in the right dorsum tongue. A clinical diagnosis was metastasis from breast cancer or primary salivary gland tumor. Histologic diagnosis of the biopsy specimen was CEPA. She underwent partial glossectomy under general anesthesia. The final diagnosis of surgical materials was CEPA based on the differential diagnosis from breast carcinoma. She is alive bearing disseminated breast carcinoma without recurrence of CEPA at 6 months after glossectomy.

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.

Subglottic Basal Cell Adenocarcinoma.

OBJECTIVES: To discuss the presentation and management of a rare neoplasm in a previously unreported laryngeal subsite. METHODS: Case report and literature review. RESULTS: An 89-year-old woman presented with a subglottic mass, biopsy of which was consistent with basal cell adenocarcinoma. She was successfully treated with surgical intervention and remains disease free 29 months postoperatively. CONCLUSIONS: Basal cell adenocarcinoma is a rare salivary gland tumor, the laryngeal variant of which is even scarcer. Herein we describe the presentation and successful surgical management of the first reported case of subglottic basal cell adenocarcinoma. We additionally provide a histologic review followed by approaches to treatment.

Pleomorphic adenoma arising from the palpebral lobe of the lacrimal gland in a patient with thyroid-associated ophthalmopathy.

PURPOSE/BACKGROUND: To report a rare coexistence of pleomorphic adenoma arising from the palpebral lobe of the lacrimal gland and thyroid-associated ophthalmopathy in a Chinese patient. METHODS: Case report of clinical features and imaging findings, orbital surgery, and histopathologic examination. RESULTS: A Chinese woman (48 years old) with hyperthyroidism showed a gradually growing mass in the lateral region of the left upper eyelid that was present for 4 years and bilateral proptosis for 5 years. There was a slightly lower eyelid retraction in both eyes. Proptosis was 20 mm in the right eye and 22 mm in the left. A well-defined, nontender mass was palpable in the lateral upper eyelid of the left eye. Ultrasonography and computed tomography revealed a rounded homogenous mass measuring 14.1 × 13.2 mm in the lateral upper eyelid of the left eye and enlarged superior, medial, and inferior recti muscles in both eyes. At surgery, a grayish-white round mass was observed after removal of the palpebral lobe of the lacrimal gland. The histopathological diagnosis was pleomorphic adenoma of the lacrimal gland. CONCLUSIONS: The coexistence of a pleomorphic adenoma of the lacrimal gland and thyroid-associated ophthalmopathy may indicate a pleomorphic adenoma likely due to an unknown autoimmune disorder.

Paraneoplastic hypercalcemia in a canine patient with a mandibular salivary carcinoma.

OBJECTIVE: To describe a novel presentation of paraneoplastic hypercalcemia caused by a canine salivary carcinoma. ANIMAL: A 6-year-old intact male Husky with hypercalcemia and a spontaneous salivary carcinoma, stage III. CLINICAL PRESENTATION, PROGRESSION, AND PROCEDURES: The dog presented with polyuria, polydipsia, and hypercalcemia. Physical examination revealed a 37 X 43-mm firm mass in the ventrolateral aspect of the right-hand side of the neck, caudal to the temporomandibular joint. Incisional biopsy was suspicious of metastatic carcinoma to the mandibular lymph node. A full-body CT scan found a large, heterogenous, contrast-enhancing mass on the right ventrolateral neck that appeared to be originating from either the mandibular lymph node or right mandibular salivary gland. Parathyroid hormone-related protein was considered within normal reference intervals, and both parathyroid glands appeared ultrasonographically normal. TREATMENT AND OUTCOME: The patient was treated with a marginal surgical excision of the mass, without immediate complications. Histopathology confirmed the presence of a salivary carcinoma with narrow margins of excision and invasion of the mandibular lymph node. Twenty-four hours after surgery, ionized calcium returned to normal reference values and clinical signs completely resolved. CLINICAL RELEVANCE: Hypercalcemia is an urgent pathology with important systemic implications requiring prompt diagnosis and intervention. In this case report, we identify the first salivary carcinoma associated with a paraneoplastic hypercalcemia, including this pathology as a new differential diagnosis. The hypercalcemia resolved with marginal surgical excision, but interestingly the parathyroid hormone-related protein was not overexpressed, meaning that this neoplasia could mediate hypercalcemia by another pathophysiological mechanism.

An unusual tumour causing neonatal respiratory distress.

PROBLEM: We present the case of a term neonate referred shortly after birth because of breathing and feeding difficulties. METHODOLOGY: Fiber-endoscopic examination of the nasal cavity showed a pendulating mass in the nasopharynx. RESULTS: A complete surgical resection was performed and the baby recovered completely. Microscopic examination of the mass showed an overlying non-keratinized squamous cell lining with an atypical cell population in some fragments. Histological features were compatible with a high-grade epithelial tumour like a midline carcinoma, but a final diagnosis of a salivary gland anlage tumour was established. CONCLUSION: Flexible fiber endoscopy is the method of choice for examining the nasal passages and oropharynx in neonates with respiratory distress. Congenital salivary gland anlage tumour is a rare cause of neonatal nasal obstruction; it is benign and complete excision results in a cure. Histologically, it may mimic a malignant tumour owing to the high mitotic index.

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.

Pleomorphic adenoma of the nasopharyngeal mucosal space with locally aggressive appearance (2010: 11b).

Pleomorphic adenoma arising from submucosal minor salivary gland tissue in the head and neck is well described in the published literature. Where associated bone changes are present, benign-appearing bone remodelling is the rule, reflecting the slow growth of this benign tumour. Aggressive-appearing bone destruction was observed in this case. This atypical appearance has not been widely described.

Obstructive sleep apnoea and snoring - is examination necessary?

This article outlines two cases of snoring and obstructive sleep apnoea (OSA) secondary to parapharyngeal space tumours. Both patients were referred to a specialist sleep clinic where oropharyngeal masses were seen and biopsied. Both underwent surgery and this was curative of both their snoring and their OSA. Parapharyngeal space tumours are an extremely rare cause of OSA and snoring. However, all patients with OSA and snoring should have a full head and neck examination before referral; in rare cases this could enable early detection of a parapharyngeal space tumour.

Congenital salivary gland anlage tumor of the nasopharynx.

Nasal and upper respiratory tract obstruction in the neonatal period can result from a variety of conditions, and may be present with variable symptoms. Salivary gland anlage tumor, also referred as congenital pleomorphic adenoma, is a very rare benign congenital tumor of the nasopharynx, which may produce nasal obstruction and other associated, nonspecific symptoms. We report a case of congenital salivary gland anlage tumor causing a severe neonatal respiratory distress with pulmonary hypertension. The tumor was removed and the outcome was favourable without recurrence at five years of the follow up.

[Polymorphous low-grade adenocarcinoma of the maxilla simulating an odontogenic cyst]. / Adénocarcinome polymorphe de bas grade du maxillaire simulant un kyste odontogénique.

INTRODUCTION: In 1990, the WHO classified Polymorphous Low Grade Adenocarcinoma as a low-grade malignant tumor of the minor salivary glands. This tumor often originates from the hard or soft palate minor salivary glands. We report the first case revealed as an infected maxillary odontogenic cyst around an impacted tooth. CASE REPORT: A 50-year-old female presented 20 months ago with a bulging, painful oral mass in the right superior vestibule. The diagnosis was infected odontogenic cyst associated with an impacted canine tooth. A course of oral antibiotics was given and cyst enucleation and tooth extraction were performed 6 days later. Ameloblastoma was suggested on macroscopic findings; nevertheless, the pathologic end diagnosis was Polymorphic Low-Grade Adenocarcinoma of minor salivary gland origin. After negative carcinological screening, wide surgical resection was performed. Postoperative external radiotherapy was applied to the operated area as well as to cervical lymph node areas. DISCUSSION: Most of the 565 published cases concern a palatine location. Even though wide surgical resection is sufficient, external radiotherapy may be used on a case-by-case basis.

[A case of pulmonary tumor thrombotic microangiopathy caused by carcinoma (salivary duct carcinoma) ex pleomorphic adenoma].

A 53-year-old man with carcinoma (salivary duct carcinoma) ex pleomorphic adenoma was admitted to our hospital because of dyspnea. He received chemotherapy in July 2007, and was subsequently followed up without chemotherapy. A chest CT scan revealed multiple faint ground glass attenuation bilaterally. Contrast-enhanced CT did not reveal any emboli in the large pulmonary arteries. An echocardiogram showed severe pulmonary hypertension. Tc-99m-MAA lung perfusion images showed multiple small defects in both lungs. The serum level of vascular endothelial growth factor (VEGF) was normal. He died of heart failure 23 days after admission. An autopsy was performed, and microscopic examination revealed tumor cell embolism, intimal fibrocellular proliferation of the small arteries, fibrin thrombi and recanalization. A diagnosis of pulmonary tumor thrombotic microangiopathy (PTTM) was made. Immunohistochemical staining of the tumor cells for VEGF was weakly positive. To the best of our knowledge this is the first reported case of PTTM caused by a salivary gland tumor.

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.

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.

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.

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.