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Nevoid basal cell carcinoma syndrome
Synonym:
Gorlin-Goltz syndrome
Nevoid basal cell carcinoma syndrome, is a syndrome consisting of multiple nevoid basal cell carcinomas, jaw cysts, skeletal abnormalities, a broad nasal ridge, hypertelorism, palmar pits, and a tendency to malignant disease.
Both benign and malignant systemic tumors have been described in association with nevoid basal cell carcinoma syndrome.
Benign tumors have included leiomyomas and fibromas of the ovary. The most common malignant tumor is medulloblastoma.
The CNS tumors can occur at a very early age, sometimes in the first year of life.
Treatment:
- As patients with nevoid basal cell carcinoma syndrome are more sensitive to x-ray damage than normals, modalities other than radiation are best used to treat the skin cancers.
- Systemic retinoids have been shown to be useful in causing the regression of some skin cancers and preventing the development of others. Often high doses are necessary for suppression.
- The role of retinoids in preventing systemic cancers has not yet been defined.
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