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Nevoid basal cell carcinoma syndrome is a genetic abnormality that often manifests with skin abnormalities and odontogenic pathology at a relatively young age. Whenever basal cell carcinoma is discovered, a more detailed history must be taken that goes back to the patient's youth. A suspicious history should lead to further follow-up. The diagnosis of NBCCS should not deter the clinician from treating the underlying skin carcinoma; rather, it should educate both the patient and the clinician that a more complex pathology exists that requires more aggressive and frequent oversight by a dermatologist.

Gorlin-Goltz syndrome, also known as basal cell nevus syndrome, is an uncommon, autosomal dominant inherited disorder, which is characterized by numerous basal cell carcinomas, maxillary keratocysts, and musculoskeletal malformations. Occasionally, it is associated with aggressive basal cell carcinomas and internal malignancies. Early diagnosis and treatment are essential, as well as genetic counseling. A patient with characteristic symptoms of nevoid basal cell carcinomas and a review of the literature are presented.

Background: Nevoid basal cell carcinoma syndrome, or NBCCS, is a hereditary condition characterized by basal cell carcinomas, or BCCs; odontogenic keratocysts, or OKCs; and skeletal abnormalities. The authors conducted this study to determine the early signs of NBCCS., Methods: The authors reviewed files from two Italian dental schools from January 1980 to January 1995 to determine the early signs of NBCCS and the age at which patients were first examined. They re-examined all of the patients, using the diagnostic criteria for NBCCS., Results: The authors found 14 patients who fulfilled the criteria for diagnosis of NBCCS in five families. All of the patients were 16 years of age or younger. In 11 cases (78 percent), the first sign of NBCCS in the patients was an OKC. The OKCs diagnosed in patients older than 13 years of age were large and characterized by widespread bone resorption. One 11-year-old patient had six large OKCs. The authors also found a case of multiple OKCs in an 8-year-old patient. Only one patient showed BCCs., Conclusions: OKCs are often the first signs of NBCCS and can be detected in patients younger than 10 years of age. Our data suggest that OKCs arise earlier in patients who have NBCCS than in patients who do not have NBCCS. The patients' young ages explain the low incidence of BCCs in this study., Clinical Implications: The presence of multiple OKCs in a child or onset of BCC in a patient younger than 20 years of age should alert dentists to the possibility of the patient's having NBCCS.

Gorlin's syndrome is a complex hamartomatous/neoplastic syndrome with multisystemic manifestations involving the skin, central nervous system, and bony skeleton. The purpose of this article is to describe how a patient with Gorlin's syndrome was managed with a multipronged approach that included exposure osteotomies for keratocyst removal combined with orthognathic surgery. This case demonstrates the advantages of standard maxillofacial osteotomies to gain access for the removal of odontogenic cysts and benign tumors.

A 14-year-old girl with nevoid basal cell carcinoma syndrome presented with intermittent headache and nausea. Magnetic resonance imaging and a computed tomographic scan of the head revealed a colloid cyst of the third ventricle and mild dilatation of the lateral ventricle. The cyst was successfully removed by stereotaxic surgery. The occurrence of two rare disorders in the same patient could be a coincidence. Other similar patients must be found before it is established that the colloid cyst is part of the nevoid basal cell carcinoma syndrome.

We report on a 27-year-old male patient suffering from multiple trichoepitheliomas, which first appeared on his face at the age of 15 and later spread to his upper extremities and back. Multiple trichoepitheliomas are benign tumors of the skin appendages with autosomally dominant heredity. Clinically, these lesions have to be differentiated from other adnexal tumors, adenoma sebaceum, as well as basal cell nevus syndrome (Gorlin-Goltz's disease).

Dentists should be aware of the multiple jaw cysts of NBCS that may develop in patients as young as 7 to 8 years. As the jaw cysts frequently are the initial symptom, dentists have the opportunity to detect the syndrome early, before the patients seek medical treatment. Any young patient with multiple odontogenic keratocysts in the jaws can develop other conditions characteristic of the disease. A careful family history should be taken, and the patient should be monitored closely for subsequent clinical manifestations of the disease.