Multiple odontogenic tumors and other anomalies. An autosomal dominantly inherited syndrome.
The authors report the occurrence of multiple odontogenic tumors in a father, his two sons, and his daughter. These occurred soon after birth and recurred upon occasion, with imcomplete removal. Associated with the multiple odontomas is esophageal stenosis. The occurrence of hepatopathy of unknown origin and an increased susceptibility to infection appear to be other components in this syndrome. A similar case reported in the literature has been cited. Autosomal dominant inheritance is suggested.
D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D002869:Chromosome Aberrations / Q000235:genetics; D025063:Chromosome Disorders; D004940:Esophageal Stenosis / Q000150:complications / Q000175:diagnosis; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D008107:Liver Diseases / Q000150:complications; D008297:Male; D008339:Mandibular Neoplasms / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery; D008441:Maxillary Neoplasms / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery; D009364:Neoplasm Recurrence, Local; D009808:Odontogenic Tumors / Q000150:complications / Q000175:diagnosis / Q000235:genetics* / Q000601:surgery; D013577:Syndrome