BACKGROUND Pulmonary mucoepidermoid carcinoma(PMEC)is a rare malignancy that arises from minor salivary glands within the tracheobronchial tree.The clear cell variant of PMEC is exceptionally uncommon and presents not...BACKGROUND Pulmonary mucoepidermoid carcinoma(PMEC)is a rare malignancy that arises from minor salivary glands within the tracheobronchial tree.The clear cell variant of PMEC is exceptionally uncommon and presents notable diagnostic challenges,primarily attributable to its morphological similarity to other tumors containing clear cells.CASE SUMMARY A 22-year-old male,formerly in good health,came in with a two-month duration of persistent cough and production of sputum.Subsequent imaging and bronchoscopy examinations revealed a 2 cm tumor in the distal left main bronchus,which resulted in complete atelectasis of the left lung.Further assessment via positron emission tomography/computed tomography scans and endoscopic biopsy confirmed the primary malignant nature of the tumor,charac-terized by clear cell morphology in most of the tumor cells.The patient underwent a left lower lobe sleeve resection accompanied by systematic mediastinal lymph node dissection.Molecular pathology analysis subsequently revealed a CRTC3-MAML2 gene fusion,leading to a definitive pathological diagnosis of the clear cell variant of PMEC,staged as T2N0M0.After surgery,the patient experienced a smooth recovery and exhibited no signs of recurrence during the one-and-a-half-year follow-up period.CONCLUSION This article describes an unusual case of a clear cell variant of PMEC characterized by the presence of a CRTC3-MAML2 gene fusion in a 22-year-old male.The patient underwent successful left lower lobe sleeve resection.This case underscores the distinctive challenges associated with diagnosing and treating this uncommon malignancy,underscoring the importance of precise diagnosis and personalized treatment strategies.展开更多
Sclerosing mucoepidermoid thyroid cancer (SMECE) is a rare entity with less than 100 cases reported in the literature. Previously considered to have an indolent course, however, recent evidence has reported an aggress...Sclerosing mucoepidermoid thyroid cancer (SMECE) is a rare entity with less than 100 cases reported in the literature. Previously considered to have an indolent course, however, recent evidence has reported an aggressive nature ranging from local invasion to distant metastasis. We present a 66-year-old Caucasian female with SMECE who initially presented neck compressive symptoms. A thyroid ultrasound (US) revealed a solid hypoechoic mass replacing the left thyroid lobe. Fine needle aspiration cytology (FNAC) of the nodule resulted in suspicion of Papillary Thyroid Cancer, Bethesda category 5. The patient underwent total thyroidectomy and surgical pathology showed SMECE. Post-therapy whole-body scan following treatment with 150 mCi I-131 showed no residual or metastatic disease. SMECE is more common in females, between the third to eighth decade of life. Preoperative diagnosis may not be accurate given variable cytopathologic features. Differential diagnoses include primary squamous cell carcinoma of the thyroid, squamous differentiation of other thyroid malignancies, anaplastic thyroid cancer and nodular sclerosing variety of Hodgkin’s lymphoma. Due to its rarity, treatment of SMECE has ranged from thyroid surgery without or with radioactive iodine therapy, to surgery and external beam radiation and even chemotherapy.展开更多
BACKGROUND Mucoepidermoid carcinoma of the lung is a rare malignant tumor,accounting for 0.1%–0.2%of all lung malignancies.It is a primary salivary gland tumor of the lung.Surgical resection is the primary treatment ...BACKGROUND Mucoepidermoid carcinoma of the lung is a rare malignant tumor,accounting for 0.1%–0.2%of all lung malignancies.It is a primary salivary gland tumor of the lung.Surgical resection is the primary treatment for pulmonary mucoepidermoid carcinoma,for which there has been no standardized treatment strategy.This article reports a case of a young woman with pulmonary mucoepidermoid carcinoma with hemoptysis as the first symptom.CASE SUMMARY A 24-year-old female patient presented with"4 d of hemoptysis"as the chief complaint.She had no special history and denied any smoking or drinking history.Physical examination revealed that the vital signs were stable and scattered small wet rales were heard in the left lung.After admission,the lung tumor markers were checked,and no abnormalities were found.After completing the bronchoscopy,a spherical lesion was observed at the main bronchus 1.5 cm away from the protubercle,with obvious pulsation and little blood seepage on the surface,and histopathological biopsy results showed acute and chronic inflammation.She was transferred to the Department of Thoracic Surgery for surgical treatment on the 16th day after admission.After exclusion of surgical conjunctures,the patient underwent resection of the tumor in the left main bronchus with single-pore video-assisted thoracic surgery on the 19th day after admission.The postoperative histopathological biopsy results showed mucoepidermoid carcinoma of the lung.The patient and her family refused to complete genetic testing and she was discharged from the hospital on the 8th day after surgery.During the follow-up period,the patient experienced shortness of breath after feeling active and had no special discomfort.CONCLUSION We have documented a case of moderately differentiated mucoepidermoid lung cancer with hemoptysis as the first symptom to improve clinicians'understanding of the disease and provide a new dimension of thinking for its future diagnosis and treatment.展开更多
Objective: Mucoepidermoid carcinoma(MEC) is the most common primary malignancy of the salivary glands. Insulin-like growth factor-II m RNA-binding protein-3(IMP3) is an important prognostic factor in some cancers and ...Objective: Mucoepidermoid carcinoma(MEC) is the most common primary malignancy of the salivary glands. Insulin-like growth factor-II m RNA-binding protein-3(IMP3) is an important prognostic factor in some cancers and a tool that differentiates between benign and malignant pancreatic lesions. This study aimed to identify a relationship between the expression of IMP3 and the outcome of salivary gland MEC, as well as to differentiate MEC from pleomorphic adenoma(PA).Methods: Tissue specimens from 70 cases of salivary gland MEC, 40 cases of PA, and 10 cases with normal salivary gland were examined immunohistochemically for IMP3. The association among the expression of IMP3, clinicopathological characteristics and patient's survival was assessed.Results: IMP3 was present in 51.4% of MEC but absent in PA and normal salivary gland tissues. IMP3 expression was associated with age > 60 years, submandibular gland tumors, tumor size > 4 cm, high-grade tumors, lymph node metastasis, involvement of surgical margins, perineural invasion, distant metastasis, advanced TNM stage, tumor relapse, and death(P<0.05). Increased expression of IMP3, tumors of the submandibular gland, and lymph node metastasis were independent prognostic factors of disease-free survival(DFS). In addition, IMP3 was a strong predictor of overall survival(OS) together with distant metastasis and intermediate and high-grade tumors.Conclusions: IMP3 expression is highly important in evaluating the outcome of MEC. IMP3 can be used to differentiate MEC from PA of salivary glands.展开更多
Mucoepidermoid carcinoma (MEC) is a rare primary esophageal malignancy. It is characterized by poor clinical recognition, pre-operative diagnostic challenges and a lack of standardized therapeutic guidelines. We repor...Mucoepidermoid carcinoma (MEC) is a rare primary esophageal malignancy. It is characterized by poor clinical recognition, pre-operative diagnostic challenges and a lack of standardized therapeutic guidelines. We report the clinicopathological features of a hitherto unreported variant of esophageal MEC, sclerosing MEC with “tissue eosinophilia”, in a mid-esophageal location in a 51-year-old female. The diagnosis of the initial biopsy was challenging, because of the small size, poor orientation and inadequate representation of the MEC components. Recognition of the resectability of the tumor prompted surgical resection and enabled a demonstration of the low grade foci containing intermediate cells, mucin pools and the hitherto undescribed presence of stromal sclerosis and tissue eosinophils in esophageal MEC. Heightened clinicopathological awareness of esophageal MEC facilitated a definitive diagnosis and patient management. Increased recognition and global documentation of esophageal sclerosing MEC with “tissue eosinophilia” is necessary to improve the understanding and diagnosis of this malignancy in this location and to improve management guidelines.展开更多
Background: Mucoepidermoid carcinoma ex-pleomorphic adenoma (MECxPA) is an extremely rare salivary gland malignancy. With only nine prior reported cases, this entity represents a challenging histopathological diagnosi...Background: Mucoepidermoid carcinoma ex-pleomorphic adenoma (MECxPA) is an extremely rare salivary gland malignancy. With only nine prior reported cases, this entity represents a challenging histopathological diagnosis. Methods: We present a case of a 71-year-old male with an enlarging left neck mass over several months. CT showed both a parapharyngeal space mass and a separate level II neck mass. Results: The patient underwent resection of the left parapharyngeal mass and ipsilateral selective level II-IV lymphadenectomy. The final pathologic diagnosis was metastatic high-grade mucoepidermoid carcinoma ex-pleomorphic adenoma. Conclusions: We describe a novel presentation of high-grade mucoepidermoid carcinoma ex-pleomorphic adenoma as a metastatic parapharyngeal mass.展开更多
BACKGROUND Primary tracheobronchial mucoepidermoid carcinoma(MEC),derived from salivary mucus glands,is an uncommon neoplasm in adults.At present,surgery is still the preferred treatment for adult bronchial MEC,althou...BACKGROUND Primary tracheobronchial mucoepidermoid carcinoma(MEC),derived from salivary mucus glands,is an uncommon neoplasm in adults.At present,surgery is still the preferred treatment for adult bronchial MEC,although it may cause significant trauma and loss of lung function.Here,we report a patient with endobronchial MEC who received the interventional bronchoscopic therapy to remove the neoplasm and no recurrence occurred during follow-up.CASE SUMMARY A 28-year-old man was admitted to our unit with mild hemoptysis for 3 d.Physical examination did not show any abnormal signs,and the serological indexes were all in the normal range.Chest computed tomography(CT)indicated an intraluminal nodule in the bronchus intermedius with homogeneous density and a well-defined margin.Upon fiberoptic bronchoscopy,an endobronchial pedunculated polypoid was discovered without submucosal involvement.As the neoplasm was confined to the bronchus,interventional bronchoscopy was performed to remove the mass by high-frequency electric knife and laser resection.Tissue was sampled and histopathological examination confirmed the diagnosis of low-grade MEC.As the proliferation index was low,no further treatment was given.During 2 years of follow-up,the patient’s condition was good and no relapse was discovered under fluorescence bronchoscopy or CT scan.CONCLUSION Interventional bronchoscopy can be considered for treatment of low-grade bronchial MEC,with few complications and preserved lung function.展开更多
BACKGROUND Mucoepidermoid carcinoma is the most common primary epithelial salivary gland malignancy.It mostly occurs in the major or intraoral minor salivary glands but rarely in the infratemporal fossa.Here,we presen...BACKGROUND Mucoepidermoid carcinoma is the most common primary epithelial salivary gland malignancy.It mostly occurs in the major or intraoral minor salivary glands but rarely in the infratemporal fossa.Here,we present a case of aggressive mucoepidermoid carcinoma in the infratemporal fossa with neck lymph node metastasis and also discuss diagnostic and treatment strategies.CASE SUMMARY A 39-year-old woman with a mass located in the right submandibular area presented to our department.Physical examination revealed lymphadenopathy on the right submandibular side measuring 2.5 cm×3 cm that was hard and had poor mobility.Results of nasal endoscopy were unremarkable.Ultrasound examination revealed an enlarged lymph node at level II of the right side.Fine needle aspiration cytology of the metastatic lymph node revealed malignant cells with infection.Contrast-enhanced computed tomography revealed an enhancing ill-defined soft tissue mass in the right infratemporal region.Positron emission tomography/computed tomography revealed hyperintensity in the right infratemporal fossa along with lymphadenopathy at level II of the right-side lymph node.The patient underwent extended resection of the primary tumor,and ipsilateral radical neck dissection was also completed.Hematoxylin-eosin staining and immunohistochemistry revealed a high-grade mucoepidermoid carcinoma.No signs and symptoms of recurrence of the neoplasm were present after 20 mo of follow-up.CONCLUSION Positron emission tomography/computed tomography play a key role in primary tumor localization.Furthermore,histopathology and immunohistochemistry play pivotal roles in disease diagnosis.展开更多
BACKGROUND Mucoepidermoid carcinoma(MEC)is a rare malignancy of the head and neck;however,it accounts for a majority of the tumors of the salivary glands.This study used a national population-based registry to describ...BACKGROUND Mucoepidermoid carcinoma(MEC)is a rare malignancy of the head and neck;however,it accounts for a majority of the tumors of the salivary glands.This study used a national population-based registry to describe the pre-treatment and treatment-related prognostic factors that influence survival in patients with MEC of the major salivary glands.To our knowledge,this is the largest populationbased study examining predictors of both overall and cause-specific survival of MEC of the major salivary glands.AIM To identify prognostic factors influencing overall survival(OS)and cause-specific survival(CSS)of patients with MEC of the major salivary glands.METHODS We used the Surveillance,Epidemiology and End-Results Database of the National Cancer Institute to investigate a variety of factors that could influence survival of patients diagnosed with mucoepidermoid carcinoma of the major salivary glands.A total of 2210 patients diagnosed with MEC of the major salivary glands during the years of 1975-2016 were studied.The primary endpoints were OS and CSS.Cox regression analysis was used to perform univariate and multivariate analyses of clinical variables such as age at diagnosis,diagnosis year,sex,race,tumor size,stage,grade,treatment with or without surgical excision,and adjuvant radiotherapy treatment.RESULTS A total of 2210 patients diagnosed with MEC of the major salivary glands met inclusion criteria.In this study,95%of patients underwent surgical excision and 41%received adjuvant radiation therapy.Median OS time for Grade I,II,and III/IV was 401 mo(±48.25,95%CI),340 mo(±33.68,95%CI)and 55 mo(±11.05,95%CI),respectively.Univariate analysis revealed that lack of surgical excision was associated with decreased OS[hazard ratio(HR)4.26,P<0.0001]and that patients with localized disease had improved OS compared to both regional and distant disease(HR 3.07 and 6.96,respectively,P<0.0001).Additionally,univariate analysis demonstrated that male sex,age over 50 at diagnosis,Grade III tumors,and increasing tumor size were associated with worsened OS(P<0.0006).Univariate analysis of CSS similarly revealed that lack of surgical excision and Grade III carcinoma conferred decreased CSS(HR 4.37 and 5.44,respectively,P<0.0001).Multivariate analysis confirmed that increasing age,in 10-year age bands,advanced tumor stage,increasing tumor size,Grade III carcinoma,male sex,and lack of surgical excision were associated with a statistically significant decrease in OS and CSS(P<0.04).Of note,multivariate analysis revealed that the use of adjuvant radiation therapy was not associated with improved OS or CSS.CONCLUSION Multivariate analysis demonstrated increasing age,advanced tumor stage,increasing tumor size,Grade III carcinoma,male sex,and lack of surgical excision were associated with decreased OS and CSS(P<0.04).展开更多
Purpose: To report the clinical features, therapeutic method, and histopathological findings of a case of mucoepidermoid carcinoma in the lower eyelid and review the literature about the mucoepidermoid carcinoma arisi...Purpose: To report the clinical features, therapeutic method, and histopathological findings of a case of mucoepidermoid carcinoma in the lower eyelid and review the literature about the mucoepidermoid carcinoma arising from the eye.Methods: Case report and review of the literature.Results: An 88-year-old man developed a painless, indurated nodule in the left lower eyelid for two years and ulceration of the skin existed for a year. He underwent tumor resection and reconstruction of the eyelid. By histopathology, tumor cells showed an admixture of epidermoid and mucus-secreting cells, which was consistent with mucoepidermoid carcinoma. Mucoepidermoid carcinoma is a common malignant tumor of the salivary glands, but rare in the eye tissues among which conjunctiva and lacrimal gland are most commonly involved. It has a higher degree of malignancy than basal cell carcinoma and squamous cell carcinoma. It displays an unusual capacity of aggressive local invasion and recurs easily after simple excision and tumors may require enucleation or exenteration because of the involvement of the intraocular structures and/or orbit.Frequent follow-up is necessary for the patient after operation.Conclusions: Mucoepidermoid carcinoma arising from the eye is rare and has a high degree of malignancy. It should be differentiated from other neoplasms such as basal cell carcinoma and squamous cell carcinoma.展开更多
Background:The differential diagnosis of mucoepidermoid carcinoma(MEC)from neoplasm undergoing mucinous features brings more pitfalls to pathologists.Combining specific MAML2 gene rearrangement and histological charac...Background:The differential diagnosis of mucoepidermoid carcinoma(MEC)from neoplasm undergoing mucinous features brings more pitfalls to pathologists.Combining specific MAML2 gene rearrangement and histological characteristics may be the solution.Methods:Twenty-five tumors with mucinous components were selected for differential diagnosis of MEC.All the cases were detected for MAML2 gene rearrangement.The cases diagnosed as MEC were classified into four variants:classic,oncocytic,Warthin-like,and nonclassified,and they were graded using the Brandwein system.The histological characteristics of non-MECs were summarized for differential diagnosis.Univariate survival analysis was performed on MECs.Results:There were 16 MECs;62.5%were MAML2 rearranged.For the low-,intermediate-,and high-grade MECs,the rate of rearrangement was 83.3%,100%,and 28.6%,respectively.Both the oncocytic and Warthinlike MECs were MAML2 rearranged.For the classic and nonclassified MECs without MAML2 rearrangement,non-keratinized squamoid cells and distinctive mucinous cells were essential diagnostic criteria.On survival analysis,all the disease progression occurred in high-grade MECs(p=0.038).Nine cases were diagnosed as non-MECs:pleomorphic adenoma with mucinous metaplasia showed no ex-capsular involvement;metaplastic Warthin tumor appeared with overt keratinization and residual oncocytic bilayered epithelium;mix squamous cell and glandular papilloma showed an endobronchial papillary growing pattern;adenosquamous carcinoma was accompanied by squamous carcinoma in situ of the overlying mucosa.All the non-MECs were negative for MAML2 rearrangement.Conclusion:The application of combining MAML2 rearrangement and histological characteristics is helpful in the differential diagnosis between MEC and other tumors with mucinous components.展开更多
Background： Tracheobronchial mucoepidermoid carcinoma （MEC） is a rare airway tumor in adults for which surgery is considered a first-line treatment. However, some patients already lost the best opportunity of a sur...Background： Tracheobronchial mucoepidermoid carcinoma （MEC） is a rare airway tumor in adults for which surgery is considered a first-line treatment. However, some patients already lost the best opportunity of a surgical intervention when diagnoses are confirmed, and surgery causes considerable trauma resulting in partial loss of pulmonary function. Moreover, the tumor is resistant to radiotherapy and chemotherapy. These factors make the treatment of tracheobronchial MEC challenging. This study aimed to evaluate the safety and et^cacy ofinterventional bronchoscopic therapy in adult patients with tracheobronchial MEC. Methods： We retrospectively analyzed the clinical manifestations, bronchoscopic interventions, complications, and outcomes of 11 adult patients with tracheobronchial MEC. Paired t-test was used to analyze the parameters of the American Thoracic Society Dyspnea Index and the Karnofsky Score before and after the first interventional bronchoscopic therapy. Results： All tumors occurred in the main bronchus and were easily visualized by bronchoscopy. After interventional bronchoscopic therapy, the symptoms of all patients showed significant improvement. The American Thoracic Society Dyspnea Index decreased from 1.91 ± 1.22 to 0.27 ± 0.47 （t = 6.708, P 〈 0.001） and the Kamofsky Score increased from 78.18 ±16.62 to 95.46 ± 8.20 （t =-5.190, P 〈 0.001 ）. Bronchoscopic intervention did not result in serious complications or mortality. During the follow-up period between 3 and 96 months after the first therapy, the following results were noted： （ 1 ） among the eight patients with low-grade tracheobronchial MEC, only one patient had a relapse and agreed to surgical treatment; （2） among the three patients with high-grade tracheobronchial MEC, one patient required repeated bronchoscopic interventions, one patient died of pulmonary infection, and one patient died of systemic failure owing to tumor metastasis. Conclusions： Interventional bronchoscopic therapy, as an alternative treatment, shows promise in some adult patients with low-grade tracheobronchial MEC confined to the bronchus. However, for adult patients with high-grade tracheobronchial MEC, early diagnosis and surgical treatment are still strongly recommended.展开更多
Background Researchers have recently demonstrated that thrombospondin-1 （TSP-1） has an important function in regulating neovascularization. Whether it inhibits or accelerates neovascularization, however, is still co...Background Researchers have recently demonstrated that thrombospondin-1 （TSP-1） has an important function in regulating neovascularization. Whether it inhibits or accelerates neovascularization, however, is still controversial. We found few reports about the correlation between TSP-1 and vascularization in mucoepidermoid carcinoma. In this research, the distribution and expression of TSP-1 in mucoepidermoid carcinoma were investigated. We also analyzed （1） the correlation between the expression of TSP-1 and microvessel density （MVD）, as an indicator of neovascularization activity, and （2） the effect of TSP-1 on neovascularization and tumor growth in the subcutaneous xenotransplanted model of mucoepidermoid carcinoma. Method （1） The sites and intensity of expression of TSP-1 and the MVD were analyzed in 45 cases of mucoepidermoid carcinoma after surgery by the method of streptavidin-peroxidase （SP） immunohistochemistry; and （2） recombinant human thrombospondin-1 （rhTSP-1） was injected twice a week for five consecutive weeks around the tumor in the subcutaneous xenotransplanted tumor model of mucoepidermoid carcinoma in nude mice. Each week, the tumor size was measured, in order to draw the growth curve of the xenotransplanted tumor model of mucoepidermoid carcinoma, and MVD was measured. Results （1） The positive expression of TSP-1 protein was 57.78% （26/45）. Most positive staining for TSP-1 was found in the cytoplasm of the cancer cells, while some staining occurred in the extracellular matrix. The mean MVD in 45 cases of m ucoepidermoid carcinoma was 58.17±19.77 per 100 visual fields. Tumors with a high expression of TSP-1 showed a low MVD value, and the TSP-1 immunocompetence and microvessel density showed a significant negative correlation （rs=-0.947, P 〈0.001）. （2） The xenotransplanted tumors with the injection doses of 1.25, 0.75 and 0.25 ug/ml respectively were 36.97%, 53.36% and 73.61% of the size of the control group （（451±92）, （651±113）, （898±86） and （1220±157） mm^3 respectively, F=-53.167, P 〈0.001）, and their weights were respectively 35.14%, 51.35% and 70.27% of the control group （（1.3±0.5）, （1.9±0.5）, （2.6±0.3）, and （3.7±0.7） g respectively, F=-62.669, P 〈0.001）. Their MVDs were 25.00%, 45.93%, and 72.20% respectively of the control group and concentration dependent （15.43±3.45, 28.35±4.24, 44.57±3.35 and 61.73±5.43 per 100 visual fields respectively, F=54.582, P 〈0.001）. Conclusions The TSP-1 has a higher expression in mucoepidermoid carcinoma and the expression has a significant negative correlation with neovascularization. The TSP-1 inhibits neovascularization and tumor growth, and it might be a new biological therapy for treatment of patients with mucoepidermoid carcinoma.展开更多
Mucoepidermoid carcinomas （MECs） had always been recognized as salivary gland tumors mainly steming from parotid and submandibular salivary glands. Eater, studies showed that MECs can occur in bronchus, esophagus, l...Mucoepidermoid carcinomas （MECs） had always been recognized as salivary gland tumors mainly steming from parotid and submandibular salivary glands. Eater, studies showed that MECs can occur in bronchus, esophagus, lacrimal glands, pancreas, thymus, and thyroid gland. MECs in bronchus are usually centrally located and are proposed to originate from submucosal minor salivary-type glands in large airways.展开更多
基金Hunan Provincial Natural Science Foundation of China,No.2022JJ40246The Hunan Cancer Hospital Climb Plan,No.2021NSFC-B005.
文摘BACKGROUND Pulmonary mucoepidermoid carcinoma(PMEC)is a rare malignancy that arises from minor salivary glands within the tracheobronchial tree.The clear cell variant of PMEC is exceptionally uncommon and presents notable diagnostic challenges,primarily attributable to its morphological similarity to other tumors containing clear cells.CASE SUMMARY A 22-year-old male,formerly in good health,came in with a two-month duration of persistent cough and production of sputum.Subsequent imaging and bronchoscopy examinations revealed a 2 cm tumor in the distal left main bronchus,which resulted in complete atelectasis of the left lung.Further assessment via positron emission tomography/computed tomography scans and endoscopic biopsy confirmed the primary malignant nature of the tumor,charac-terized by clear cell morphology in most of the tumor cells.The patient underwent a left lower lobe sleeve resection accompanied by systematic mediastinal lymph node dissection.Molecular pathology analysis subsequently revealed a CRTC3-MAML2 gene fusion,leading to a definitive pathological diagnosis of the clear cell variant of PMEC,staged as T2N0M0.After surgery,the patient experienced a smooth recovery and exhibited no signs of recurrence during the one-and-a-half-year follow-up period.CONCLUSION This article describes an unusual case of a clear cell variant of PMEC characterized by the presence of a CRTC3-MAML2 gene fusion in a 22-year-old male.The patient underwent successful left lower lobe sleeve resection.This case underscores the distinctive challenges associated with diagnosing and treating this uncommon malignancy,underscoring the importance of precise diagnosis and personalized treatment strategies.
文摘Sclerosing mucoepidermoid thyroid cancer (SMECE) is a rare entity with less than 100 cases reported in the literature. Previously considered to have an indolent course, however, recent evidence has reported an aggressive nature ranging from local invasion to distant metastasis. We present a 66-year-old Caucasian female with SMECE who initially presented neck compressive symptoms. A thyroid ultrasound (US) revealed a solid hypoechoic mass replacing the left thyroid lobe. Fine needle aspiration cytology (FNAC) of the nodule resulted in suspicion of Papillary Thyroid Cancer, Bethesda category 5. The patient underwent total thyroidectomy and surgical pathology showed SMECE. Post-therapy whole-body scan following treatment with 150 mCi I-131 showed no residual or metastatic disease. SMECE is more common in females, between the third to eighth decade of life. Preoperative diagnosis may not be accurate given variable cytopathologic features. Differential diagnoses include primary squamous cell carcinoma of the thyroid, squamous differentiation of other thyroid malignancies, anaplastic thyroid cancer and nodular sclerosing variety of Hodgkin’s lymphoma. Due to its rarity, treatment of SMECE has ranged from thyroid surgery without or with radioactive iodine therapy, to surgery and external beam radiation and even chemotherapy.
文摘BACKGROUND Mucoepidermoid carcinoma of the lung is a rare malignant tumor,accounting for 0.1%–0.2%of all lung malignancies.It is a primary salivary gland tumor of the lung.Surgical resection is the primary treatment for pulmonary mucoepidermoid carcinoma,for which there has been no standardized treatment strategy.This article reports a case of a young woman with pulmonary mucoepidermoid carcinoma with hemoptysis as the first symptom.CASE SUMMARY A 24-year-old female patient presented with"4 d of hemoptysis"as the chief complaint.She had no special history and denied any smoking or drinking history.Physical examination revealed that the vital signs were stable and scattered small wet rales were heard in the left lung.After admission,the lung tumor markers were checked,and no abnormalities were found.After completing the bronchoscopy,a spherical lesion was observed at the main bronchus 1.5 cm away from the protubercle,with obvious pulsation and little blood seepage on the surface,and histopathological biopsy results showed acute and chronic inflammation.She was transferred to the Department of Thoracic Surgery for surgical treatment on the 16th day after admission.After exclusion of surgical conjunctures,the patient underwent resection of the tumor in the left main bronchus with single-pore video-assisted thoracic surgery on the 19th day after admission.The postoperative histopathological biopsy results showed mucoepidermoid carcinoma of the lung.The patient and her family refused to complete genetic testing and she was discharged from the hospital on the 8th day after surgery.During the follow-up period,the patient experienced shortness of breath after feeling active and had no special discomfort.CONCLUSION We have documented a case of moderately differentiated mucoepidermoid lung cancer with hemoptysis as the first symptom to improve clinicians'understanding of the disease and provide a new dimension of thinking for its future diagnosis and treatment.
文摘Objective: Mucoepidermoid carcinoma(MEC) is the most common primary malignancy of the salivary glands. Insulin-like growth factor-II m RNA-binding protein-3(IMP3) is an important prognostic factor in some cancers and a tool that differentiates between benign and malignant pancreatic lesions. This study aimed to identify a relationship between the expression of IMP3 and the outcome of salivary gland MEC, as well as to differentiate MEC from pleomorphic adenoma(PA).Methods: Tissue specimens from 70 cases of salivary gland MEC, 40 cases of PA, and 10 cases with normal salivary gland were examined immunohistochemically for IMP3. The association among the expression of IMP3, clinicopathological characteristics and patient's survival was assessed.Results: IMP3 was present in 51.4% of MEC but absent in PA and normal salivary gland tissues. IMP3 expression was associated with age > 60 years, submandibular gland tumors, tumor size > 4 cm, high-grade tumors, lymph node metastasis, involvement of surgical margins, perineural invasion, distant metastasis, advanced TNM stage, tumor relapse, and death(P<0.05). Increased expression of IMP3, tumors of the submandibular gland, and lymph node metastasis were independent prognostic factors of disease-free survival(DFS). In addition, IMP3 was a strong predictor of overall survival(OS) together with distant metastasis and intermediate and high-grade tumors.Conclusions: IMP3 expression is highly important in evaluating the outcome of MEC. IMP3 can be used to differentiate MEC from PA of salivary glands.
文摘Mucoepidermoid carcinoma (MEC) is a rare primary esophageal malignancy. It is characterized by poor clinical recognition, pre-operative diagnostic challenges and a lack of standardized therapeutic guidelines. We report the clinicopathological features of a hitherto unreported variant of esophageal MEC, sclerosing MEC with “tissue eosinophilia”, in a mid-esophageal location in a 51-year-old female. The diagnosis of the initial biopsy was challenging, because of the small size, poor orientation and inadequate representation of the MEC components. Recognition of the resectability of the tumor prompted surgical resection and enabled a demonstration of the low grade foci containing intermediate cells, mucin pools and the hitherto undescribed presence of stromal sclerosis and tissue eosinophils in esophageal MEC. Heightened clinicopathological awareness of esophageal MEC facilitated a definitive diagnosis and patient management. Increased recognition and global documentation of esophageal sclerosing MEC with “tissue eosinophilia” is necessary to improve the understanding and diagnosis of this malignancy in this location and to improve management guidelines.
文摘Background: Mucoepidermoid carcinoma ex-pleomorphic adenoma (MECxPA) is an extremely rare salivary gland malignancy. With only nine prior reported cases, this entity represents a challenging histopathological diagnosis. Methods: We present a case of a 71-year-old male with an enlarging left neck mass over several months. CT showed both a parapharyngeal space mass and a separate level II neck mass. Results: The patient underwent resection of the left parapharyngeal mass and ipsilateral selective level II-IV lymphadenectomy. The final pathologic diagnosis was metastatic high-grade mucoepidermoid carcinoma ex-pleomorphic adenoma. Conclusions: We describe a novel presentation of high-grade mucoepidermoid carcinoma ex-pleomorphic adenoma as a metastatic parapharyngeal mass.
基金Supported by the National Natural Science Foundation of China,No.81870018.
文摘BACKGROUND Primary tracheobronchial mucoepidermoid carcinoma(MEC),derived from salivary mucus glands,is an uncommon neoplasm in adults.At present,surgery is still the preferred treatment for adult bronchial MEC,although it may cause significant trauma and loss of lung function.Here,we report a patient with endobronchial MEC who received the interventional bronchoscopic therapy to remove the neoplasm and no recurrence occurred during follow-up.CASE SUMMARY A 28-year-old man was admitted to our unit with mild hemoptysis for 3 d.Physical examination did not show any abnormal signs,and the serological indexes were all in the normal range.Chest computed tomography(CT)indicated an intraluminal nodule in the bronchus intermedius with homogeneous density and a well-defined margin.Upon fiberoptic bronchoscopy,an endobronchial pedunculated polypoid was discovered without submucosal involvement.As the neoplasm was confined to the bronchus,interventional bronchoscopy was performed to remove the mass by high-frequency electric knife and laser resection.Tissue was sampled and histopathological examination confirmed the diagnosis of low-grade MEC.As the proliferation index was low,no further treatment was given.During 2 years of follow-up,the patient’s condition was good and no relapse was discovered under fluorescence bronchoscopy or CT scan.CONCLUSION Interventional bronchoscopy can be considered for treatment of low-grade bronchial MEC,with few complications and preserved lung function.
基金the Basic Research Program of Shenzhen Innovation Council of China,No.JCYJ 20180228175511141 and No.SZBC2017023Sanming Project of Medicine in Shenzhen,No.SZSM 201512036(Oral and Maxillofacial Surgery Team,Professor Yu Guangyan,Stomatology Hospital Peking University).
文摘BACKGROUND Mucoepidermoid carcinoma is the most common primary epithelial salivary gland malignancy.It mostly occurs in the major or intraoral minor salivary glands but rarely in the infratemporal fossa.Here,we present a case of aggressive mucoepidermoid carcinoma in the infratemporal fossa with neck lymph node metastasis and also discuss diagnostic and treatment strategies.CASE SUMMARY A 39-year-old woman with a mass located in the right submandibular area presented to our department.Physical examination revealed lymphadenopathy on the right submandibular side measuring 2.5 cm×3 cm that was hard and had poor mobility.Results of nasal endoscopy were unremarkable.Ultrasound examination revealed an enlarged lymph node at level II of the right side.Fine needle aspiration cytology of the metastatic lymph node revealed malignant cells with infection.Contrast-enhanced computed tomography revealed an enhancing ill-defined soft tissue mass in the right infratemporal region.Positron emission tomography/computed tomography revealed hyperintensity in the right infratemporal fossa along with lymphadenopathy at level II of the right-side lymph node.The patient underwent extended resection of the primary tumor,and ipsilateral radical neck dissection was also completed.Hematoxylin-eosin staining and immunohistochemistry revealed a high-grade mucoepidermoid carcinoma.No signs and symptoms of recurrence of the neoplasm were present after 20 mo of follow-up.CONCLUSION Positron emission tomography/computed tomography play a key role in primary tumor localization.Furthermore,histopathology and immunohistochemistry play pivotal roles in disease diagnosis.
基金Supported by Community Cancer Fund in Spokane,Washington,United States.
文摘BACKGROUND Mucoepidermoid carcinoma(MEC)is a rare malignancy of the head and neck;however,it accounts for a majority of the tumors of the salivary glands.This study used a national population-based registry to describe the pre-treatment and treatment-related prognostic factors that influence survival in patients with MEC of the major salivary glands.To our knowledge,this is the largest populationbased study examining predictors of both overall and cause-specific survival of MEC of the major salivary glands.AIM To identify prognostic factors influencing overall survival(OS)and cause-specific survival(CSS)of patients with MEC of the major salivary glands.METHODS We used the Surveillance,Epidemiology and End-Results Database of the National Cancer Institute to investigate a variety of factors that could influence survival of patients diagnosed with mucoepidermoid carcinoma of the major salivary glands.A total of 2210 patients diagnosed with MEC of the major salivary glands during the years of 1975-2016 were studied.The primary endpoints were OS and CSS.Cox regression analysis was used to perform univariate and multivariate analyses of clinical variables such as age at diagnosis,diagnosis year,sex,race,tumor size,stage,grade,treatment with or without surgical excision,and adjuvant radiotherapy treatment.RESULTS A total of 2210 patients diagnosed with MEC of the major salivary glands met inclusion criteria.In this study,95%of patients underwent surgical excision and 41%received adjuvant radiation therapy.Median OS time for Grade I,II,and III/IV was 401 mo(±48.25,95%CI),340 mo(±33.68,95%CI)and 55 mo(±11.05,95%CI),respectively.Univariate analysis revealed that lack of surgical excision was associated with decreased OS[hazard ratio(HR)4.26,P<0.0001]and that patients with localized disease had improved OS compared to both regional and distant disease(HR 3.07 and 6.96,respectively,P<0.0001).Additionally,univariate analysis demonstrated that male sex,age over 50 at diagnosis,Grade III tumors,and increasing tumor size were associated with worsened OS(P<0.0006).Univariate analysis of CSS similarly revealed that lack of surgical excision and Grade III carcinoma conferred decreased CSS(HR 4.37 and 5.44,respectively,P<0.0001).Multivariate analysis confirmed that increasing age,in 10-year age bands,advanced tumor stage,increasing tumor size,Grade III carcinoma,male sex,and lack of surgical excision were associated with a statistically significant decrease in OS and CSS(P<0.04).Of note,multivariate analysis revealed that the use of adjuvant radiation therapy was not associated with improved OS or CSS.CONCLUSION Multivariate analysis demonstrated increasing age,advanced tumor stage,increasing tumor size,Grade III carcinoma,male sex,and lack of surgical excision were associated with decreased OS and CSS(P<0.04).
文摘Purpose: To report the clinical features, therapeutic method, and histopathological findings of a case of mucoepidermoid carcinoma in the lower eyelid and review the literature about the mucoepidermoid carcinoma arising from the eye.Methods: Case report and review of the literature.Results: An 88-year-old man developed a painless, indurated nodule in the left lower eyelid for two years and ulceration of the skin existed for a year. He underwent tumor resection and reconstruction of the eyelid. By histopathology, tumor cells showed an admixture of epidermoid and mucus-secreting cells, which was consistent with mucoepidermoid carcinoma. Mucoepidermoid carcinoma is a common malignant tumor of the salivary glands, but rare in the eye tissues among which conjunctiva and lacrimal gland are most commonly involved. It has a higher degree of malignancy than basal cell carcinoma and squamous cell carcinoma. It displays an unusual capacity of aggressive local invasion and recurs easily after simple excision and tumors may require enucleation or exenteration because of the involvement of the intraocular structures and/or orbit.Frequent follow-up is necessary for the patient after operation.Conclusions: Mucoepidermoid carcinoma arising from the eye is rare and has a high degree of malignancy. It should be differentiated from other neoplasms such as basal cell carcinoma and squamous cell carcinoma.
基金Beijing Hope Run Special Fund of Cancer Foundation of China(Grant/Award Number: LC2018A19)
文摘Background:The differential diagnosis of mucoepidermoid carcinoma(MEC)from neoplasm undergoing mucinous features brings more pitfalls to pathologists.Combining specific MAML2 gene rearrangement and histological characteristics may be the solution.Methods:Twenty-five tumors with mucinous components were selected for differential diagnosis of MEC.All the cases were detected for MAML2 gene rearrangement.The cases diagnosed as MEC were classified into four variants:classic,oncocytic,Warthin-like,and nonclassified,and they were graded using the Brandwein system.The histological characteristics of non-MECs were summarized for differential diagnosis.Univariate survival analysis was performed on MECs.Results:There were 16 MECs;62.5%were MAML2 rearranged.For the low-,intermediate-,and high-grade MECs,the rate of rearrangement was 83.3%,100%,and 28.6%,respectively.Both the oncocytic and Warthinlike MECs were MAML2 rearranged.For the classic and nonclassified MECs without MAML2 rearrangement,non-keratinized squamoid cells and distinctive mucinous cells were essential diagnostic criteria.On survival analysis,all the disease progression occurred in high-grade MECs(p=0.038).Nine cases were diagnosed as non-MECs:pleomorphic adenoma with mucinous metaplasia showed no ex-capsular involvement;metaplastic Warthin tumor appeared with overt keratinization and residual oncocytic bilayered epithelium;mix squamous cell and glandular papilloma showed an endobronchial papillary growing pattern;adenosquamous carcinoma was accompanied by squamous carcinoma in situ of the overlying mucosa.All the non-MECs were negative for MAML2 rearrangement.Conclusion:The application of combining MAML2 rearrangement and histological characteristics is helpful in the differential diagnosis between MEC and other tumors with mucinous components.
文摘Background： Tracheobronchial mucoepidermoid carcinoma （MEC） is a rare airway tumor in adults for which surgery is considered a first-line treatment. However, some patients already lost the best opportunity of a surgical intervention when diagnoses are confirmed, and surgery causes considerable trauma resulting in partial loss of pulmonary function. Moreover, the tumor is resistant to radiotherapy and chemotherapy. These factors make the treatment of tracheobronchial MEC challenging. This study aimed to evaluate the safety and et^cacy ofinterventional bronchoscopic therapy in adult patients with tracheobronchial MEC. Methods： We retrospectively analyzed the clinical manifestations, bronchoscopic interventions, complications, and outcomes of 11 adult patients with tracheobronchial MEC. Paired t-test was used to analyze the parameters of the American Thoracic Society Dyspnea Index and the Karnofsky Score before and after the first interventional bronchoscopic therapy. Results： All tumors occurred in the main bronchus and were easily visualized by bronchoscopy. After interventional bronchoscopic therapy, the symptoms of all patients showed significant improvement. The American Thoracic Society Dyspnea Index decreased from 1.91 ± 1.22 to 0.27 ± 0.47 （t = 6.708, P 〈 0.001） and the Kamofsky Score increased from 78.18 ±16.62 to 95.46 ± 8.20 （t =-5.190, P 〈 0.001 ）. Bronchoscopic intervention did not result in serious complications or mortality. During the follow-up period between 3 and 96 months after the first therapy, the following results were noted： （ 1 ） among the eight patients with low-grade tracheobronchial MEC, only one patient had a relapse and agreed to surgical treatment; （2） among the three patients with high-grade tracheobronchial MEC, one patient required repeated bronchoscopic interventions, one patient died of pulmonary infection, and one patient died of systemic failure owing to tumor metastasis. Conclusions： Interventional bronchoscopic therapy, as an alternative treatment, shows promise in some adult patients with low-grade tracheobronchial MEC confined to the bronchus. However, for adult patients with high-grade tracheobronchial MEC, early diagnosis and surgical treatment are still strongly recommended.
基金This study was supported by the NatiOnal Natural Science Foundation of China （No. 0040305401042）.Acknowledgements： We would like to thank Steven Pan and Mary Meyer for the assistance in polishing the paper. Their innovative suggestions and advice contributed significantly to the final version of the paper.
文摘Background Researchers have recently demonstrated that thrombospondin-1 （TSP-1） has an important function in regulating neovascularization. Whether it inhibits or accelerates neovascularization, however, is still controversial. We found few reports about the correlation between TSP-1 and vascularization in mucoepidermoid carcinoma. In this research, the distribution and expression of TSP-1 in mucoepidermoid carcinoma were investigated. We also analyzed （1） the correlation between the expression of TSP-1 and microvessel density （MVD）, as an indicator of neovascularization activity, and （2） the effect of TSP-1 on neovascularization and tumor growth in the subcutaneous xenotransplanted model of mucoepidermoid carcinoma. Method （1） The sites and intensity of expression of TSP-1 and the MVD were analyzed in 45 cases of mucoepidermoid carcinoma after surgery by the method of streptavidin-peroxidase （SP） immunohistochemistry; and （2） recombinant human thrombospondin-1 （rhTSP-1） was injected twice a week for five consecutive weeks around the tumor in the subcutaneous xenotransplanted tumor model of mucoepidermoid carcinoma in nude mice. Each week, the tumor size was measured, in order to draw the growth curve of the xenotransplanted tumor model of mucoepidermoid carcinoma, and MVD was measured. Results （1） The positive expression of TSP-1 protein was 57.78% （26/45）. Most positive staining for TSP-1 was found in the cytoplasm of the cancer cells, while some staining occurred in the extracellular matrix. The mean MVD in 45 cases of m ucoepidermoid carcinoma was 58.17±19.77 per 100 visual fields. Tumors with a high expression of TSP-1 showed a low MVD value, and the TSP-1 immunocompetence and microvessel density showed a significant negative correlation （rs=-0.947, P 〈0.001）. （2） The xenotransplanted tumors with the injection doses of 1.25, 0.75 and 0.25 ug/ml respectively were 36.97%, 53.36% and 73.61% of the size of the control group （（451±92）, （651±113）, （898±86） and （1220±157） mm^3 respectively, F=-53.167, P 〈0.001）, and their weights were respectively 35.14%, 51.35% and 70.27% of the control group （（1.3±0.5）, （1.9±0.5）, （2.6±0.3）, and （3.7±0.7） g respectively, F=-62.669, P 〈0.001）. Their MVDs were 25.00%, 45.93%, and 72.20% respectively of the control group and concentration dependent （15.43±3.45, 28.35±4.24, 44.57±3.35 and 61.73±5.43 per 100 visual fields respectively, F=54.582, P 〈0.001）. Conclusions The TSP-1 has a higher expression in mucoepidermoid carcinoma and the expression has a significant negative correlation with neovascularization. The TSP-1 inhibits neovascularization and tumor growth, and it might be a new biological therapy for treatment of patients with mucoepidermoid carcinoma.
文摘Mucoepidermoid carcinomas （MECs） had always been recognized as salivary gland tumors mainly steming from parotid and submandibular salivary glands. Eater, studies showed that MECs can occur in bronchus, esophagus, lacrimal glands, pancreas, thymus, and thyroid gland. MECs in bronchus are usually centrally located and are proposed to originate from submucosal minor salivary-type glands in large airways.
