title: Mucoepidermoid Carcinoma in Children: A Single Institutional Experience.
abstract: OBJECTIVE
  To determine the clinicopathologic and molecular features and outcome of children with mucoepidermoid carcinoma (MEC).
  
  
  METHODS
  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.
  
  
  RESULTS
  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).
  
  
  CONCLUSIONS
  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.
authors.affiliation: Texas Children's Cancer and Hematology Centers, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas.|Department of Pediatrics, Division of Hematology and Oncology, Faculty of Medicine, Chulalongkorn University, Bangkok, Thailand.
authors.forename: Piti
authors.initials: P
authors.lastname: Techavichit
authors.affiliation: Department of Pathology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas.
authors.forename: M John
authors.initials: MJ
authors.lastname: Hicks
authors.affiliation: Department of Pathology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas.
authors.forename: Dolores H
authors.initials: DH
authors.lastname: López-Terrada
authors.affiliation: Department of Pathology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas.
authors.forename: Norma M
authors.initials: NM
authors.lastname: Quintanilla
authors.affiliation: Department of Pediatric Radiology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas.
authors.forename: R Paul
authors.initials: RP
authors.lastname: Guillerman
authors.affiliation: Department of Pathology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas.
authors.forename: Stephen F
authors.initials: SF
authors.lastname: Sarabia
authors.affiliation: Department of Pathology, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas.
authors.forename: Hadi
authors.initials: H
authors.lastname: Sayeed
authors.affiliation: Department of Surgery, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas.
authors.forename: Jed G
authors.initials: JG
authors.lastname: Nuchtern
authors.affiliation: Department of Radiation Oncology, University of Texas MD Anderson Cancer Center, Houston, Texas.|Department of Pediatrics, Baylor College of Medicine, Houston, Texas.
authors.forename: Arnold C
authors.initials: AC
authors.lastname: Paulino
authors.affiliation: Texas Children's Cancer and Hematology Centers, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas.
authors.forename: Jodi A
authors.initials: JA
authors.lastname: Muscal
authors.affiliation: Texas Children's Cancer and Hematology Centers, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas.
authors.forename: M Fatih
authors.initials: MF
authors.lastname: Okcu
authors.affiliation: Texas Children's Cancer and Hematology Centers, Texas Children's Hospital, Baylor College of Medicine, Houston, Texas.
authors.forename: Murali
authors.initials: M
authors.lastname: Chintagumpala
mesh_terms: D000293:Adolescent; D018277:Carcinoma, Mucoepidermoid / Q000235:genetics / Q000473:pathology / Q000628:therapy; D002648:Child; D003131:Combined Modality Therapy; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D015514:Oncogene Proteins, Fusion / Q000235:genetics; D060888:Real-Time Polymerase Chain Reaction; D012189:Retrospective Studies; D012468:Salivary Gland Neoplasms / Q000235:genetics / Q000473:pathology / Q000628:therapy; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: C495418:MECT1-MAML2 fusion protein, human; D015514:Oncogene Proteins, Fusion
keywords: MECT1-MAML2 fusion; children; mucoepidermoid carcinoma