Gorlin's Basal Cell Nevus Syndrome

Naevoid Basal Cell Carcinoma (Gorlin's) Syndrome

“…'.^ In affected individuals BCC usually begin to appear around puberty, and are invariably multiple. 6 The regions most frequently affected are the face (particularly around the nose, mouth, eyes and ears), the upper trunk and the arms, although the BCC may occur on any part of the body.' The majority behave in a relatively benign fashion, but with increasing age of the patient they tend to become more aggressive, and may then cause significant morbidity.…”

Diagnosis and Treatment of Basal Cell Nevus Syndrome

“…Other less common manifestations include a host of benign and malignant noncutane‐ous neoplasms, ocular abnormalities, ovarian calcifications, cleft lip and palate, and characteristic facies 1 . The disorder is inherited in an autosomal dominant manner with a gene on chromosome 9q that regulates cell growth; however, one third of patients do not have a positive family history, suggesting that incomplete penetrance, variable expression of genes, or new mutations may occur 3,4 . The syndrome occurs with equal frequency in both sexes and usually becomes apparent between 5 and 30 years of age.…”

“…The syndrome occurs with equal frequency in both sexes and usually becomes apparent between 5 and 30 years of age. The incidence is estimated to be 0.5% of all basal cell carcinoma patients 4 …”

“…1 The disorder is inherited in an autosomal dominant manner with a gene on chromosome 9q that regulates cell growth; however, one third of patients do not have a positive family history, suggesting that incomplete penetrance, variable expression of genes, or new mutations may occur. 3,4 The syndrome occurs with equal frequency in both sexes and usually becomes apparent between 5 and 30 years of age. The incidence is estimated to be 0.5% of all basal cell carcinoma patients.…”