Abstract
Gorlin-Goltz Syndrome (GGS) also known as Nevoid Basal Cell Carcinoma Syndrome is a rare autosomal dominant disorder. Is characterized for presenting some clinical features such as palmar pits, fused eyebrows, multiple odontogenic keratocyst, which are considered some of the most common signs. In our case, a rare familial case highlights awareness when a member of the family is diagnosed with this condition, and the probability to find in one the first degree relative. Once again it is confirmed that is impossible to determine without the radiographical analysis such as Cone Beam Computed Tomography (CBCT), ortophantomography, magnetic resonance imaging, to corroborate with clinical aspects, advising that, this could be the best way for the sake of patients benefit. Many treatments less aggressive have been proposed along the years, with a lower percentage of success against the traditional way, specifically surgical enucleation/marsupialization combined with peripheral osteotomy, in order to approach the variable behavior of nevoid basal cell carcinoma syndrome. The early diagnosis is considered the key to well manage this kind of disease, due to its aggressiveness, potential to become malignant, and in some cases, with a silent destructive pattern.
Highlights
A rare familial case highlights awareness when a member of the family is diagnosed with this condition, and the probability to find in one the first degree relative
Once again it is confirmed that is impossible to determine without the radiographical analysis such as Cone Beam Computed Tomography (CBCT), ortophantomography, magnetic resonance imaging, to corroborate with clinical aspects, advising that, this could be the best way for the sake of patients benefit
Many treatments less aggressive have been proposed along the years, with a lower percentage of success against the traditional way, surgical enucleation/marsupialization combined with peripheral osteotomy, in order to approach the variable behavior of nevoid basal cell carcinoma syndrome
Summary
Gorlin-Goltz syndrome known as Nevoid Basal Cell Carcinoma Syndrome was described for the first time in 1894 from Jarisch and White [1]. Bettley and Ward were the first to relate the presence of palmar and plantar pits with this syndrome It was not until 1960 when Robert James Gorlin and William Goltz established classical triad that characterizes the diagnosis of this syndrome The CBCT (Figure 3) and the orthopantomogram (Figure 4) was advised which revealed three cystic lesions in mandibular bone, one in the symphysis and one on each side of the mandibular angle, and two involving the maxillary canine region on both sides with the displaced permanent teeths. Do to the genetical component and aggressive behavior of Gorlin Goltz Syndrome, the decision to advise a control orthopantogram for his sister, revealed one cyst in left base of the mandible (Figure 17)
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