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Related Experiment Videos

Treating nevoid basal cell carcinoma syndrome

K R Reisner1, R D Riva, R J Cobb

  • 1Department of Orthodontics, School of Dental Medicine, University of Pennsylvania, Philadelphia 19104-6003.

Journal of the American Dental Association (1939)
|July 1, 1994
PubMed
Summary

This case report details an odontogenic keratocyst found in a 15-year-old male, highlighting its association with a syndrome known for over 100 anomalies. The report reviews the syndrome's history and related complications.

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Area of Science:

  • Oral pathology
  • Genetics
  • Dermatology

Background:

  • Nevoid basal cell carcinoma syndrome (Gorlin syndrome) is an autosomal dominant disorder.
  • Characterized by a wide spectrum of developmental abnormalities, including skeletal, ophthalmologic, endocrine, and skin manifestations.
  • Odontogenic keratocysts are a frequent and early feature of the syndrome.

Observation:

  • A 15-year-old male presented with a cyst.
  • The cyst, surgically removed, was histopathologically diagnosed as an odontogenic keratocyst.
  • The patient's clinical history was evaluated in the context of known syndrome-related anomalies.

Findings:

  • The diagnosis of odontogenic keratocyst in this patient is consistent with the known prevalence in Nevoid basal cell carcinoma syndrome.

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  • The case underscores the importance of recognizing the syndrome given its numerous associated anomalies.
  • Detailed description of the syndrome's long-standing history and associated health problems is provided.

    • Implications:

    • Early diagnosis and management of odontogenic keratocysts can be crucial for patients with Nevoid basal cell carcinoma syndrome.
    • Awareness of the syndrome's broad clinical spectrum is essential for comprehensive patient care.
    • This case contributes to the understanding of the clinical presentation and management of this rare genetic disorder.