Mucoepidermoid Carcinoma In Children Research Articles

Comparison Between Primary and Secondary Pediatric Mucoepidermoid Carcinoma of the Head and Neck.

Objectives: Secondary mucoepidermoid carcinoma (MEC) of the head and neck is occasionally observed in childhood cancer survivors. The goal of this research was to compare the demographic and pathologic characteristics, as well as survival between primary and secondary MEC in children and adolescent patients.Methods: Pediatric patients (younger than 19 years old) with surgically treated MEC of the head and neck were retrospectively enrolled at the Affiliated Children's Hospital of Zhengzhou University and divided into two groups based on their cancer history. Demographic, pathologic, and survival characteristics between the two groups were compared. The main study interests were recurrence-free survival (RFS), overall survival (OS), and disease-specific survival (DSS).Results: The primary and secondary groups consisted of 63 and 15 patients, respectively. The two groups had similar distributions in terms of age, sex, tumor stage, neck lymph node stage, perineural invasion, lymphovascular invasion, p53, Bcl-2, proliferating cell nuclear antigen, carcinoembryonic antigen, and Ki-67 index. The 10-year RFS rates for the primary group and secondary group were 80 and 71%, respectively, and this difference was not significant (p = 0.464). The 10-year DSS rates for the primary group and secondary group were 83 and 82%, respectively, and this difference was also not significant (p = 0.649). The 10-year OS rates for the primary group and secondary group were 74 and 51%, respectively; this difference was significant (p = 0.023). Further Cox model analysis confirmed the independence of a previous cancer history (p = 0.043) in decreasing OS.Conclusions: Pediatric patients with secondary MEC exhibit similar demographic, pathologic, and molecular characteristics as primary patients but worse OS. These findings indicate that special disease management approaches might be needed for secondary patients.

Application of bronchoscope in diagnosis and treatment of bronchial mucoepidermoid carcinoma in children

Objective To explore the application of bronchoscope in diagnosis and treatment of bronchial mucoepidermoid carcinoma in children. Methods The clinical data of 4 cases of bronchial mucoepidermoid carcinoma treated in Department of Interventional Pulmonology, Beijing Children's Hospital Affiliated to Capital Medical University from April 2013 to June 2015, were retrospectively analyzed, and their clinical manifestations, chest computed tomography(CT), bronchoscopic manifestations were analyzed. Results Among the 4 cases of bronchial mucoepidermoid carcinoma, 3 cases were low-grade bronchial mucoepidermoid carcinoma, and 1 case was intermediate grade malignant. Onset of clinical manifestations occurred from the ages of 4 years and 11 months old to 10 years and 9 months old.All patients had no characteristic clinical manifestations, only 2 cases had hemoptysis, while the main symptoms were cough, wheezing, and exercise tolerance decreased in 4 the patients, and they were initially misdiagnosed as pneumonia, asthma or bronchial foreign body.Chest CT prompted that there was circular mass in respiratory tract, the lesions located in left main bronchus in 2 patients, in right bronchus intermedius in 1 patient, and in right middle bronchus in 1 case, and atelectases or emphysema occurred in bronchus.The biopsy by bronchoscope under general anesthesia was performed for more enough specimen in all the 4 patients.The lesions were completely removed in 3 patients by interventional bronchoscopy, and 1 patient by lobectomy. Conclusions The children with bronchial mucoepidermoid carcinoma were presented with cough and wheezing without specific manifestations. Biopsy by bronchoscope is a good choice for the diagnosis of bronchial mucoepidermoid carcinoma. Bronchoscopic intervention was effective in the treatment of non-operative carcinoma. Key words: Mucoepidermoid carcinoma; Bronchus; Child; Bronchoscope

Mucoepidermoid Carcinoma in Children: A Single Institutional Experience.

To determine the clinicopathologic and molecular features and outcome of children with mucoepidermoid carcinoma (MEC). A retrospective analysis of clinical and histopathologic findings was performed in patients with MEC diagnosed at Texas Children's Cancer Center between 2000 and 2014. Ten female and four male patients with median age 12 years (range 7-19 years) were included in the study. Tumors involved major salivary glands, minor salivary glands of the palate, and the tracheobronchial tree. Nine of 11 patients with salivary MEC underwent more than one surgical resection at the time of initial diagnosis to achieve a gross total resection. Three patients with tracheobronchial tumors underwent pulmonary lobectomy. Three patients received postoperative radiation therapy. No patient was treated with chemotherapy. Histopathologic grades were classified as low (n = 2), intermediate (n = 9), and high (n = 3). All 12 patients with tumor tissue available for testing were positive for MECT1/MAML2 fusion transcripts. There were no deaths, metastases, or recurrences in this series, with a median follow-up of 24 months (range 5-96 months). Low to intermediate histopathologic grade MECs are more common than high grade MEC in children. In contrast to adults, MECT1/MAML2 fusion transcripts occur with a frequency of 100% in our pediatric MEC series. Complete excision is the treatment of choice and is associated with excellent outcome. The role of radiotherapy is unclear, but may be indicated in patients with high grade tumors with positive surgical margins.

Efficacy of bronchoscopic therapies for bronchial mucoepidermoid carcinoma in children: results from six patients.

Bronchial mucoepidermoid carcinoma is a rare disease in children, and lobectomy is traditionally considered as a first-line treatment. As the tumor is characterized by intraluminal growth, low malignancy and superficial infiltration of bronchial walls, bronchoscopic interventional therapy may provide an alternative treatment with favorable results. The aim of the study was to evaluate the efficacy and safety of bronchoscopic therapies for bronchial mucoepidermoid carcinoma in children. Clinical manifestations, multiple bronchoscopic interventions and outcomes in 6 children with bronchial mucoepidermoid carcinoma were retrospectively analyzed. The median age was 8.7 years (range 4 to 12 years). All the tumors were located in the lobar bronchus and were detected by computerized tomography. Diagnoses of low-grade mucoepidermoid carcinoma were made based on biopsies obtained via bronchoscopy. Five tumors were classified as intraluminal type and successfully eradicated by carbon dioxide cryotherapy and argon plasma coagulation under bronchoscopy. The other tumor in patient 3 was classified as the mixture type and could not be removed by a bronchoscopic, so left upper lobectomy was performed. For each patient, interventional procedures were conducted on the average for 6 times. No recurrence was detected by bronchoscopic inspections or computed tomography scans during follow-ups for 16-72 months. Multiple bronchoscopic procedures, as alternative treatments, are effective in removing bronchial mucoepidermoid carcinoma mucoepidermoid carcinoma in children without any major complications.

Minor salivary gland mucoepidermoid carcinoma in children and adolescents: a case series and review of the literature

IntroductionBecause well-documented cases of mucoepidermoid carcinomas that are of minor salivary gland origin and occur in children and adolescents have rarely been reported, little information regarding their clinical features and biologic behavior is available. This case report represents a retrospective clinical analysis of five minor salivary gland mucoepidermoid carcinomas accessioned from a 35-year period at the Louisiana State University School of Dentistry and combines the data with 15 well-documented cases from the English language literature.Case presentationThe five mucoepidermoid carcinomas in patients from birth to 19 years of age accounted for 1.3% of the accessioned minor salivary gland neoplasms. There were an additional 15 well-documented cases in the literature. Combining the data for the 20 mucoepidermoid carcinomas resulted in a mean age of 13.5 years and a 2.3:1 female-to-male ratio. Collectively, the hard palate, soft palate, and hard palate/soft palate junction accounted for 85% of the cases. Thirty-five percent of the cases presented as a fluctuant submucosal swelling with surface color alterations. The average duration was five months, and bone involvement occurred in seven cases. A histologic grade of low to intermediate predominated (95%). Surgical removal was the treatment in all cases. Thirteen cases had adequate follow-up of three years or more, and recurrence was documented in only one case. There were no cases of death or metastasis in this series.ConclusionsIn children and adolescents, mucoepidermoid carcinomas have a female predilection and occur most commonly on the hard or soft palate or both. A fluctuant submucosal lump with a bluish color is a helpful diagnostic clue. The histologic grades of most mucoepidermoid carcinomas in the first and second decades of life are low and, to a lesser degree, intermediate. Complete surgical excision is the treatment of choice and results in a recurrence rate of less than 10%.

Bronchial Mucoepidermoid Carcinoma in Children

Three cases of children, aged 7, 12 and 13 years, respectively, with bronchial mucoepidermoid carcinoma are presented. All patients were primarily misdiagnosed as pneumonitis following common symptoms of prolonged fever, cough, expectoration, and indirect signs of bronchial obstruction by chest roentgenogram, before the tumours in the main bronchi were revealed by chest computed tomography and bronchoscopy. After undergoing lobectomy and systematic lymph node dissection, all three patients recovered uneventfully. Histologically, all the cases were low-grade mucoepidermoid carcinoma; one case additionally had mediastinal lymph node metastasis. The patients have remained in good health during a follow-up of between 1.5 and 6 years. Despite its extremely rare occurrence in childhood, the possibility of bronchial mucoepidermoid carcinoma should be kept in mind when encountering a child presenting with symptoms of recurrent pneumonia.

Primacy of surgery in the management of mucoepidermoid carcinoma in children.

Epithelial salivary gland neoplasms are rare in children. Malignant tumors account for 30% to 50% of cases in the pediatric age group, with mucoepidermoid carcinoma as the most common histology. A retrospective medical record review was conducted from 1953 to 2007 to identify patients with mucoepidermoid carcinoma at the age of 18 years or younger at the time of diagnosis. Forty-nine patients were identified. Their medical records were examined for presentation, treatment, pathologic features, and outcomes. Forty-nine pediatric patients with mucoepidermoid carcinoma were identified. The parotid gland (49%) and oral cavity (35%) were the most common subsites. Nodal metastasis was seen in 24% of patients. All patients underwent surgery, and 11 patients (22%) were treated with radiation therapy. The 5-year overall survival was 98%, the 10-year overall survival was 94%, and 10% of patients developed recurrence. Mucoepidermoid carcinoma in children carries a favorable prognosis and can be successfully treated with surgery alone in most cases.

Mucoepidermoid carcinoma of bronchus in a pediatric patient: 18F-FDG PET findings

In children, primary neoplasms of the tracheobronchial tree and lungs are rare; most are malignant. Of the primary malignant pulmonary neoplasms arising in childhood, mucoepidermoid carcinoma accounts for approximately 10%. Due to its well-confined local growth within the airway, mucoepidermoid carcinoma commonly produces respiratory symptoms from progressive tracheal or bronchial obstruction. Mucoepidermoid tumor has minimal metastatic potential in children, and local resection alone is the current treatment of choice. Early detection, diagnosis, and surgical resection of mucoepidermoid tumor are especially important in pediatric patients since the bulk of the remaining pulmonary parenchyma can be preserved, thereby decreasing the thoracic deformity and pulmonary functional morbidity. Radiographic and CT imaging findings of bronchial mucoepidermoid carcinoma in children have been described in several case reports. However, to the best of our knowledge, imaging findings of 2-((18)F)-fluoro-2-deoxy-D: -glucose positron emission tomography ((18)F-FDG PET) of mucoepidermoid carcinoma of the bronchus in pediatric patients have not been well established. We report a mucoepidermoid carcinoma arising from the right upper lobe bronchus in a 15-year-old girl with an emphasis on the (18)F-FDG PET findings.

Mucoepidermoid Carcinoma in Children: Report of a case and review of literature.

A rare case of mucoepidermoid carcinoma arising in a 15-year-old boy is reported. The patient had palatal swelling with tenderness for two years. After a diagnosis of mucoepidermoid carcinoma was obtained by biopsy and CT scan examinations, the patient underwent a partial maxillectomy. Histopathologically, the tumor arising in the palatal mucosa involved the maxillary bone and the base of the right maxillary sinus. The tumor consisted not only of predominantly clear cells and squamous cells in solid nests but also of mucous cells forming various sized cystic cavities. From a review of the literature, mucoepidermoid carcinoma was shown to be the most frequent salivary tumor in children, comprising 3% to 8% of the total numbers of patients with mucoepidermoid carcinoma. When compared with adult cases, childhood cases more frequently involve the parotid gland and less frequently the minor salivary glands.

Mucoepidermoid carcinoma of salivary glands in children and adolescents: assessment of proliferation markers.

Malignant neoplasms represent one-third of all pediatric salivary gland tumors. Mucoepidermoid carcinoma (MEC) composes 51% of malignant tumors and 16% of all salivary gland neoplasms in pediatrics. Prognostic factors in MEC in pediatric patients have not been well defined. Histopathologic features, clinical outcomes and proliferation markers in 26 pediatric patients (median age 11 years; 19F:7M) with salivary gland MECs were evaluated retrospectively. MEC histocytologic grading used a three-tiered system. Proliferation was assessed by determining the percentage of tumor cells immunoreactive for PCNA and Ki-67. Tumor site was 16 parotid, eight submandibular, one base of tongue and one maxillary lip. Median tumor size was 2.5 cm (range 1.5-5 cm). MEC grade was nine low grade (LG), 15 intermediate grade (IG) and two high grade (HG). Metastatic disease and capsular invasion occurred in five cases, while perineural invasion was noted in three cases. Mean percentage of tumor cells immunoreactive for proliferation markers is as follows: PCNA: LG 9%, IG 17%, HG 32%; and Ki-67: LG 7%, IG 12%, HG 26%. Treatment was surgical in 21 cases, and surgery with chemotherapy and radiotherapy in five cases. Two patients with high grade MECs died of disease (21, 44 months). Twenty-four patients had no evidence of disease at a median follow-up of 104 months (range 30-298 months). MECs were second malignancies in two children with prior radiotherapy and chemotherapy for leukemia and histiocytosis. Low and intermediate grade salivary gland MECS in a pediatric population may have a favorable outcome when compared with high grade MECs. Proliferation markers appear to be linked to histocytologic MEC grade and may provide information regarding biologic behavior of salivary gland MECs in children and adolescents.

小児の気管支に発生した粘表皮癌の１症例

小児の気管支に発生した粘表皮癌の１症例

Endobronchial carcinoid and mucoepidermoid carcinoma in children

Endobronchial neoplastic disease is rarely encountered in patients under 20 years of age. The great majority of these lesions are carcinoids or mucoepidermoid carcinoma. Symptoms are secondary to bronchial irritation and manifest as recurrent pneumonitis, hemoptysis, persistent cough, reactive airway disease, and chest pain. Early bronchoscopy reliably enables identification and may prevent harmful sequelae resulting from delay in diagnosis. Retrospective data from four cases collected from the Tumor Registry in the Southern California Kaiser Permanente Medical Group is presented and a review of the literature is discussed.