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[Syndrome of multiple nevoid basal cell carcinoma]
D Morais Pérez1, J M Santos Martí, J Fernández Gómez
1Servicio de O.R.L. Hospital del Insalud de Barbastro, Huesca.
Anales Otorrinolaringologicos Ibero-Americanos
|January 1, 1991
Summary
Gorlin's syndrome, a genetic disorder, involves multiple odontogenic keratocysts and basal cell carcinomas. These cysts can cause ear, nose, and throat symptoms, particularly when affecting the maxillary sinus.
Area of Science:
- Dermatology
- Oral and Maxillofacial Surgery
- Genetics
Background:
- Gorlin's syndrome, also known as nevoid basal cell carcinoma syndrome, is a rare genetic disorder.
- It is characterized by a triad of clinical features: multiple nevoid basal cell carcinomata, odontogenic keratocysts, and skeletal anomalies.
Observation:
- Odontogenic keratocysts are a common manifestation, often presenting with symptoms related to the ear, nose, and throat (ENT).
- These cysts frequently occur in the maxillary bone and paranasal sinuses, leading to ENT-related clinical presentations.
Findings:
- The paper reviews the clinical aspects of Gorlin's syndrome, emphasizing the significance of odontogenic keratocysts.
- Approximately 250 cases have been documented since its initial description in 1894.
Implications:
- Understanding the ENT manifestations of odontogenic keratocysts in Gorlin's syndrome is crucial for timely diagnosis and management.
- This review highlights the importance of recognizing the multifaceted clinical presentation of this syndrome.