Clinico-pathological study of esophageal mucoepidermoid carcinoma: a 10-year survival from a single center.

BACKGROUND: Mucoepidermoid Carcinoma of the Esophagus (MECE) is a relatively rare tumor type, with most of the current data derived from case reports or small sample studies. This retrospective study reports on the 10-year survival data and detailed clinicopathological characteristics of 48 patients with esophageal MEC. METHODS: Data were collected from 48 patients who underwent curative surgery for esophageal MEC at the Fourth Hospital of Hebei Medical University between January 1, 2004, and December 31, 2020. These were compared with contemporaneous cases of Esophageal Squamous Cell Carcinoma (ESCC) and Esophageal Adenocarcinoma (EAC). Using the Kaplan-Meier method and multivariate Cox regression analysis, we investigated the clinicopathological factors affecting the survival of patients with MEC. RESULTS: The incidence of MECE was predominantly higher in males, with a male-to-female ratio of approximately 7:1. The mid-thoracic segment emerged as the most common site of occurrence. A mere 6.3% of cases were correctly diagnosed preoperatively. The lymph node metastasis rate stood at 35.4%. The overall 1-year, 3-year, 5-year, and 10-year survival rates for all patients were 85.4%, 52.1%, 37.0%, and 31.0%, respectively. Post 1:1 propensity score matching, no significant statistical difference was observed in the Overall Survival (OS) between MEC patients and those with Esophageal Squamous Cell Carcinoma (ESCC) and Esophageal Adenocarcinoma (EAC) (P = 0.119, P = 0.669). Univariate analysis indicated that T staging and N staging were the primary factors influencing the prognosis of esophageal MEC. CONCLUSIONS: MECE occurs more frequently in males than females, with the mid-thoracic segment being the most common site of occurrence. The rate of accurate preoperative endoscopic diagnosis is low. The characteristic of having a short lesion length yet exhibiting significant extramural invasion may be a crucial clinicopathological feature of MECE. The OS of patients with MEC does not appear to significantly differ from those with esophageal squamous carcinoma and adenocarcinoma.

Rare but clinically important salivary gland-type tumor of the lung: A review.

Salivary gland-type tumor (SGT) of the lung, which arises from the bronchial glands of the tracheobronchial tree, was first recognized in the 1950s. SGT represents less than 1% of all lung tumors and is generally reported to have a good prognosis. Mucoepidermoid carcinoma (MEC) and adenoid cystic carcinoma (ACC) are the two most common subtypes, comprising more than 90% of all SGTs. The reported 5-year survival rate of patients with SGT is 63.4%. Because this type of tumor develops in major bronchi, patients with SGT commonly present with symptoms of bronchial obstruction, including dyspnea, shortness of breath, wheezing, and coughing; thus, the tumor is usually identified at an early stage. Most patients are treated by lobectomy and pneumonectomy, but bronchoplasty or tracheoplasty is often needed to preserve respiratory function. Lymphadenectomy in the surgical resection of SGT is recommended, given that clinical benefit from lymphadenectomy has been reported in patients with MEC. For advanced tumors, appropriate therapy should be considered according to the subtype because of the varying clinicopathologic features. MEC, but not ACC, is less likely to be treated with radiation therapy because of its low response rate. Although previous researchers have learned much from studying SGT over the years, the diagnosis and treatment of SGT remains a complex and challenging problem for thoracic surgeons. In this article, we review the diagnosis, prognosis, and treatment (surgery, chemotherapy, and radiotherapy) of SGT, mainly focusing on MEC and ACC. We also summarize reports of adjuvant and definitive radiation therapy for ACC in the literature.

Elective nodal dissection for cN0 intermediate-grade parotid mucoepidermoid carcinoma: A NCDB study.

PURPOSE: To determine the occult nodal disease rate and whether elective regional lymph node dissection (RLND) confers any 10-year overall survival (OS) in cN0 intermediate-grade mucoepidermoid carcinoma (MEC) of the parotid gland. MATERIALS & METHODS: The National Cancer Database was reviewed from 2004 to 2016 on adults with cT1-4aN0M0 intermediate-grade parotid MEC undergoing resection with/without RLND. Comparisons between patients with and without RLND were made. Occult nodal rate and 10-year overall survival (OS) were determined. RESULTS: Out of 898 included patients with cN0 intermediate grade parotid MEC undergoing elective RLND, the occult nodal rate was 7.6%. This was significantly different from low-grade (3.9%) and high-grade (25.7%) cN0 disease. When stratified by pT-classification, marginal differences were identified between low-grade and intermediate-grade tumors, whereas high-grade tumors demonstrated increased occult nodal disease with low T-stage (pT1-pT2, 20.4% vs. 5.1%) and high T-stage (pT3-pT4a, 32.1% vs. 17.6%). Patients undergoing elective RLND were more often treated at an academic facility (53.8% vs. 41.2%), had higher pT3-pT4 tumors (19.2% vs. 10.4%), and more frequently underwent total/radical parotidectomy (46.0% vs. 29.9%) with adjuvant radiation therapy (53.8% vs. 41.0%) Cox-proportional hazard modeling did not identify RLND, regardless if stratified by nodal yield or pT-classification, nor nodal positivity as significant predictors of 10-year OS. CONCLUSIONS: The occult nodal disease in intermediate-grade parotid MEC is low and similar to low-grade. Elective RLND may have a limited impact on OS, though its effect on locoregional control remains unknown. LEVEL OF EVIDENCE: III.

Immunotherapy combined with chemotherapy in the treatment for rare primary mucoepidermoid carcinoma of the liver: a case report.

Primary mucoepidermoid carcinoma of the liver (PMCL) is rare in the hepatic system, with no standard treatment and poor prognosis with a median overall survival of only 120 days. PMCL with immunotherapy has not been reported yet. Here, we present a case of PMCL treated by immunotherapy and chemotherapy. A 64-year-old male with PMCL underwent partial right hepatectomy and liver lesion resection on 19 June 2020. Two months later, the chest computed tomography indicated the presence of multiple nodules in both lungs with higher tumor markers. Considering the presence of a tumor metastasis, the patient received four courses of immunotherapy plus mGEMOX chemotherapy from 8 September 2020. The patient tolerated the combined therapy well, with red moles on the face and chest which were considered as grade 1 reactive cutaneous capillary endothelial proliferation. He also had grade 2 thrombocytopenia and leucopenia after the first course of chemotherapy, but no neutropenia, fatigue, vomiting or diarrhea. However, his disease progressed. The patient refused further treatment and died on 20 April 2021. The overall survival time after diagnosis was 301 days. We describe here the first case report on immunotherapy treatment for PMCL. That suggested immunotherapy combined with chemotherapy may be an option after a hepatic lobectomy for PMCL.

Mucoepidermoid Carcinoma Ex Pleomorphic Adenoma of Lacrimal Gland: Case Report and Review of Literature.

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.

Maxillectomy and Flap Reconstruction of Maxillary Defects After Cancer Ablation Through the Lip-Split Parasymphyseal Mandibulotomy Approach in Patients With Advanced-Stage Maxillary Malignant Tumors.

BACKGROUND: The conventional approach for maxillectomy has some common and serious complications. AIMS: The present study evaluated the outcomes of maxillectomy and flap reconstruction after cancer ablation using the lip-split parasymphyseal mandibulotomy (LPM) approach. METHODS: Twenty-eight patients with malignant tumors, including squamous cell carcinoma, adenoid cystic carcinoma, and mucoepidermoid carcinoma, underwent maxillectomy through the LPM approach. Brown classes II and III were reconstructed with the facial-submental artery submental island flap, an extensive segmental pectoralis major myocutaneous flap, and a free anterolateral thigh flap with the use of a titanium mesh, respectively. RESULTS: All proximal margin frozen section specimens showed negative surgical margins. Anterolateral thigh flap failure occurred in 1 patient, whereas ophthalmic and mandibulotomy complications developed in 4 and 7 patients, respectively. In all, 84.6% of the patients had satisfactory or excellent lip esthetic results. Of the patients, 57.1% were alive with no evidence of disease, whereas 28.6% were alive with disease and 14.3% died of local recurrence or distant metastasis. No significant survival difference was evident among the squamous cell carcinoma, adenoid cystic carcinoma, and mucoepidermoid carcinoma groups. CONCLUSIONS: The LPM approach can provide good surgical access, facilitating maxillectomy in advanced-stage malignant tumors with minimal morbidity. Facial-submental artery submental island flap and anterolateral thigh flap or extensive segmental pectoralis major myocutaneous flap with a titanium mesh are ideal techniques for reconstructing Brown classes II and III defects, respectively.

Intraosseous Mucoepidermoid Carcinoma of the Mandible Detected as an Asymptomatic Swelling.

Intraosseous mucoepidermoid carcinoma (IMEC) is a rare neoplasm of the jawbones. Although hypotheses focused on the malignant transformation of the epithelial mucosa of odontogenic cysts or ectopic salivary gland tissue have been suggested, the etiology of the disease is still unclear. It is more frequent in middle-aged individuals, has a slight female predilection, and is more common in the mandible than in the maxilla. Cortical enlargement is the most common symptom, while some lesions are detected by coincidence on radiography. This paper reports an IMEC of the mandible of a 35-year-old female, possibly arising from the remains of an odontogenic cyst associated with an unerupted mandibular molar, which was operated in an external center 5 years ago before IMEC diagnosis.

A rare case of mucoepidermoid carcinoma ex pleomorphic adenoma of the lacrimal gland.

Carcinoma ex pleomorphic adenoma (CEPA) of the lacrimal gland is a rare malignant tumor that arises from a pre-existing pleomorphic adenoma. Lacrimal gland CEPA with mucoepidermoid histological subtype is exceedingly rare. Diagnosis can be aided by radiographic findings, though the gold standard is histopathological analysis following excisional biopsy. Management options include complete surgical excision with or without adjuvant radiation therapy based on tumor grade and invasiveness. We present a 76-year-old woman with 6 months of diplopia and unilateral proptosis. Her initial exam was remarkable for hypoglobus, proptosis, and limited elevation of the right eye. Computed tomography (CT) scan demonstrated a superior, well-circumscribed, extraconal orbital mass. An excisional biopsy was performed, and histopathological findings were consistent with mucoepidermoid carcinoma ex pleomorphic adenoma with positive margins in the tumor capsule. The patient received radiation therapy and remains markedly improved with no disease recurrence at 5 months post-operatively.

[Surgical Case of Bronchial Mucoepidermoid Carcinoma Safely Diagnosed by Enucleation Under the Bronchoscopy After Bronchial Arterial Embolization].

A 28-year-old male presented to our hospital with hemoptysis and his chest computerized tomography (CT) showed the right middle and lower lobe atelectasis due to the tumor of right intermediate bronchial trunk. To reduce the blood flow to the tumor, bronchial arterial embolization was performed and the tumor was resected using Cryoprobe with a flexible endobronchial scope. Thus, we could observe the tumor localization and diagnose before the surgical procedure. We performed the right sleeve middle lobectomy and the right lower lobe was safely preserved.

[Sclerosing mucoepidermoid carcinoma with eosinophilia of thyroid gland]. / Skleroziruyushchii mukoepidermoidnyi rak shchitovidnoi zhelezy s eozinofiliei stromy.

Sclerosing mucoepidermoid carcinoma with stromal eosinophilia of thyroid gland is represented less than 100 cases in the world literature. We present a rare case of sclerosing mucoepidermoid carcinona with stromal eosinophilia in a 69-year-old woman who has been observed for multinodular goiter for more than 3 years. Cytological examination revealed a picture most of all corresponding to a malignant neoplasm (Bethesda V). The patient underwent a thyroidectomy. Pathomorphological examination revealed a neoplasm of mixed structure with foci of cribriform structures and squamous metaplasia with areas of keratinization. According to the IHC study, the expression of TTF-1, p63, cytokeratins 5/6 were positive, while there were no expression of thyroglobulin with its positive reaction in the thyroid tissue. Additional histochemical staining with Alcian blue revealed a positive reaction with a homogeneous acellular substance of the tumor. A comprehensive morphological study with the use of additional stains made it possible to accurately establish the diagnosis, which will determine the further tactics of managing the patient.

Surgical management of pediatric salivary malignant tumors-A single-center cohort study.

BACKGROUND: The purpose of this study was to report incidence, clinicopathologic behavior, management, and outcome of pediatric patients treated surgically for salivary gland (SG) malignancies. METHODS: Patients who underwent surgery for SG malignancies from 1985 to 2015 were identified. Clinical, pathological, treatment and outcomes data were collected. Disease-specific survival (DSS), recurrence-free survival (RFS), and overall survival (OS) were calculated using Kaplan-Meier method. RESULTS: Twenty-eight pediatric patients were included. The most common histopathological types were mucoepidermoid (n = 18, 64.3%), acinic cell (n = 7, 25.0%), adenoid cystic (n = 2, 7.1%), and adenocarcinoma (n = 1, 3.6%). Surgical approach varied and ranged from superficial parotidectomy (n = 11, 39.3%) to partial maxillectomy (n = 6, 21.4%). Nine patients (32%) required postoperative radiotherapy. DSS, OS, and RFS probability at 5 years were 96.4%, 96.4%, and 89.3%, respectively. CONCLUSION: Pediatric SG malignancies are rare and have favorable outcome at 5 years. Larger, multi-institutional studies are required to better understand the natural history of these rare tumors.

Survival Outcome of Salivary Gland Carcinoma: A 50-Year Retrospective Study With Long-Term Follow-up.

PURPOSE: Salivary gland carcinomas (SGCs) can be classified into more than 20 subtypes with various clinical behaviors. The present study aimed to analyze the clinical and pathological features of SGCs and evaluate their long-term prognosis. METHODS: A retrospective cohort study was performed. This study investigated cases of histologically confirmed SGC at the authors' institution from January 1963 to December 2014. Data on sex, age, site, histopathological diagnosis, tumor-node-metastasis classification, postoperative radiotherapy and/or chemotherapy, local and regional recurrence, and distant metastasis (DM) were collected as covariates. The overall survival (OS) rate was analyzed as the outcome. Kaplan-Meier survival analysis and Cox multivariate analysis were used for survival analysis. The cohort was divided into 2 groups-before and after 1989. The clinicopathological characteristics of the 2 groups were compared using the χ2 test. RESULTS: The cohort included 1,637 patients who met the admission criteria and had a male-to-female ratio of 0.9:1. The median age was 47 years (range, 8 months to 86 years). The median follow-up time was 54 months (range, 1-432 months). The majority of the tumors occurred in the parotid gland (35.3%), followed by the palate gland (25.2%). Adenoid cystic carcinoma was the most common tumor type (34.3%), and mucoepidermoid carcinoma (29%) was the second most common type. In the 1,637 patients, the neck lymph node metastasis rate was 8.7% at the first surgery, and the overall DM rate was 14.1%. The 5-, 10-, and 15-year OS rates of the 1,637 cases were 93.1%, 87.2%, and 79.3%, respectively. Comparative analysis before and after 1989 showed statistically significant differences in sex, site, histologic subtype, T classification, local and regional recurrence rate, and radiotherapy (P < .05), while no significant differences were found in age, N classification, M staging, DM, or chemotherapy. CONCLUSIONS: The OS rates of SGC have improved significantly over the past 30 years. This is attributable to an increase in the proportion of patients diagnosed at the early stage and receiving radiotherapy, as this has led to a reduction in the local and regional recurrence rate and, consequently, an improvement in the survival rates.

Clinicopathological Analysis of 907 Major and Minor Salivary Gland Tumors.

PURPOSE: The frequency and types of salivary gland tumors show significant geographical variations. The most common are primary epithelial tumors, with pleomorphic adenoma and mucoepidermoid carcinoma being the most frequent. This study aims to analyze the clinicopathological data of patients with major and minor salivary gland (MiSG) tumors. METHODS: The retrospective study included all patients with major and MiSG tumors diagnosed and treated between January 2000 and January 2019. Files of 907 patients were reviewed and investigated for clinicopathologic features of major and MiSG tumors in Serbia. RESULTS: The majority of tumors were of epithelial origin. Pleomorphic adenoma was the predominant type of tumor, with 35.1% among all tumors on all sites. Adenoid cystic carcinoma and mucoepider-moid carcinoma (with 7.1% and 2.7%, respectively) were the most common malignant ones. The most common localization was the parotid gland. Minor salivary gland tumors comprised 16.43% of all salivary gland tumors in our series, the most common localization being the oral cavity. The results of our study are mostly consistent with the results of other previously published studies. CONCLUSIONS: The most important finding, worth emphasizing, is that the most common malignant major and MiSG tumor in our population is adenoid cystic carcinoma, rather than mucoepidermoid carcinoma, in all investigated localizations. In addition, the nasal cavity is the most common localization among malignant MiSG tumors.

Response to Miles Kiernan Re: a rare case of mucoepidermoid carcinoma ex pleomorphic adenoma of the lacrimal gland.

Our goal is to contribute to the medical literature with our case of CEPA and hope to better help fellow clinicians diagnose and treat these rare tumors.

[Mucoepidermoid Carcinoma Suspectedly Developed from a Bronchogenic Cyst in the Posterior Mediastinum].

Mucoepidermoid carcinoma developing from a bronchogenic cyst is extremely rare. We present a case of a 74-year-old man with a cystic mass in the posterior mediastinum detected by chest computed tomography( CT) and magnetic resonance imaging. A bronchogenic cyst or neurogenic tumor was suspected. He did not accept surgical treatment and was followed up at outpatient. Since the enlargement of the mass was shown by chest CT after seven years, the resection of the mass was performed by thoracoscopic surgery, however the cyst wall remained due to the severe adhesion and the residual mucosa was cauterized. The mass was diagnosed as a mucoepidermoid carcinoma by pathology which was likely to develop from a bronchogenic cyst. After postoperative radiotherapy, the patient is well without recurrence 10 months after surgery.

An eight-year-old girl with tracheal mass treated as a difficult asthma case.

INTRODUCTION: Endobronchial masses such as mucoepidermoid carcinomas or carcinoid tumors are extremely rare in children and they usually originate from large bronchi. These lesions may cause wheezing and dyspnea with poor response to bronchodilators and mimic the airway obstruction caused by asthma. CASE STUDY: We present the case of an 8-year-old girl with tracheal mucoepidermoid carcinoma who was treated as a difficult asthma case with high dose of inhaled corticosteroids. RESULTS: The characteristic stridor, the lack of response to bronchodilators and to inhaled corticosteroid treatment, combined with the characteristic flow loop in spirometry and the hyperinflation seen on the chest radiograph, all raised the clinical suspicion of a tracheal lesion and indicated the need for flexible bronchoscopy. The bronchoscopy revealed a large lesion obstructing totally the trachea lumen. The latter finding was confirmed by chest high resolution CT. The mass was completely excised via sternotomy under cardiopulmonary bypass, and the pathologic examination showed a low-grade mucoepidermoid carcinoma of the trachea. One month after the surgery she was free of symptoms and her spirometry was normal. CONCLUSION: Tracheal lesions mimic the symptoms of airway obstruction caused by asthma and should be always be part of the differential diagnosis in young patients with no response to asthma treatment.

Primary pulmonary mucoepidermoid carcinoma: Cyto-histologic correlation and review of the literature.

Preoperative fine needle aspiration diagnosis and cyto-histologic correlation of primary pulmonary mucoepidermoid carcinoma have rarely been described in detail in the literature. A 26-year old male presented at our institution with cough, bloody sputum, and a 4.3 cm left lower lobe lung mass. He was accurately diagnosed with pulmonary mucoepidermoid carcinoma on preoperative aspiration cytology. The patient subsequently proceeded to left lower lobectomy, confirming the diagnosis. In this article, we present a detailed report of primary pulmonary mucoepidermoid carcinoma describing the cytologic and histologic morphologic features, its differential diagnosis with review of the literature.

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.

[A case of bronchial mucous gland adenoma: A rare diagnosis that should not be mistaken!] / Un cas d'adénome muqueux glandulaire bronchique : un diagnostic rare à ne pas méconnaître !

We report the case of a 32-year-old man, who was admitted for a recurrent pneumopathy. The thoracic computed tomography revealed a small well-circumscribed lesion of the lower right lobe of the lung. Microscopic examination from the biopsy material of the endoscopy concluded a mucoepidermoid carcinoma. A lobectomy was realized. Microscopic examination revealed the presence of a well-delineated lesion composed of glands and cysts containing mucous and limited by mucous and cylindric and ciliated cells without atypia. The proliferation index was very low. A diagnosis of mucous gland adenoma was made. It is an exceptional tumor and is very difficult to diagnose on biopsy material but should be known by pathologists. It is associated with a good prognosis. The aims of our observation are to present the macroscopic and microscopic features of this tumor and data from recent literature review to better diagnose it. This is also the second observation with molecular details for this entity.

Mucoepidermoid carcinoma of parotid gland and membranous nephropathy - differentiation between sclerosing mucoepidermoid carcinoma with eosinophilia and Kimura's disease.

BACKGROUND: When we encounter patients who present with both a neck mass and nephrotic syndrome, both malignancy and Kimura's disease need to be evaluated as the therapeutic strategies differ vastly between them. CASE PRESENTATION: We present the case of a 27-year-old male patient with neck mass and nephrotic syndrome. The presence of both eosinophilia and elevated immunoglobulin E levels were concerning for Kimura's disease, which is an allergic syndrome defined by eosinophilic granulomas of neck soft tissue along with peripheral eosinophilia. The eventual final diagnosis, however, was sclerosing mucoepidermoid carcinoma of parotid gland with both eosinophilia and membranous nephropathy. Following the surgical resection of the mass, the nephrotic syndrome completely resolved. CONCLUSION: Detailed histopathological assessments of both the parotid gland and renal tissue were key aspects of the diagnosis and management to exclude Kimura's disease.