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J Pediatr Hematol Oncol. 2011 Jul;33(5):387-9. doi: 10.1097/MPH.0b013e3181e65c1c.

Metastatic squamous cell carcinoma of the oropharynx in a child with a mutation in the Connexin 26 gene.

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Department of Pediatrics, Texas Children's Hospital, Baylor College of Medicine, Houston, TX, USA.


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.

[Indexed for MEDLINE]

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