title: Metastatic squamous cell carcinoma of the oropharynx in a child with a mutation in the Connexin 26 gene.
abstract: Pediatric oral squamous cell carcinoma is an extremely rare occurence. In our report, we describe a 6-year-old White female with sensorineural hearing loss found to have a Connexin 26 gene mutation who developed a well-differentiated squamous cell carcinoma of the hard palate with metastatic disease to submandibular lymph nodes and the lungs. This association emphasizes the need to consider Connexin 26 gene mutations in children who develop oral squamous cell carcinoma and to monitor for oral squamous cell carcinoma in children with Connexin 26 gene mutations.
authors.affiliation: Department of Pediatrics, Texas Children's Hospital, Baylor College of Medicine, Houston, TX, USA.
authors.forename: Surya
authors.initials: S
authors.lastname: Rednam
authors.forename: John
authors.initials: J
authors.lastname: Hicks
authors.forename: Moise L
authors.initials: ML
authors.lastname: Levy
authors.forename: Alberto S
authors.initials: AS
authors.lastname: Pappo
mesh_terms: D002294:Carcinoma, Squamous Cell / Q000235:genetics* / Q000556:secondary; D002648:Child; D000072259:Connexin 26; D017630:Connexins / Q000235:genetics*; D017809:Fatal Outcome; D005260:Female; D006319:Hearing Loss, Sensorineural / Q000235:genetics; D006801:Humans; D008207:Lymphatic Metastasis; D009154:Mutation; D009959:Oropharyngeal Neoplasms / Q000235:genetics* / Q000556:secondary; D010157:Palatal Neoplasms / Q000235:genetics* / Q000473:pathology
chemical_list: D017630:Connexins; D000072259:Connexin 26