Gorlin-Goltz Syndrome: A Case Report and Literature Review

Abstract

Gorlin-Goltz syndrome (GGS) is an infrequent multisystemic disease with an autosomal dominant inherited disorder characterized by the presence of multiple keratocystic odontogenic tumors (KCOT) in the jaws, multiple basal cell nevi carcinomas, and skeletal abnormalities. Early diagnosis of Gorlin-Goltz syndrome is essential as it may progress to aggressive basal cell carcinomas and neoplasias. Gorlin-Goltz syndrome has rarely been reported in Saudi Arabia. This article reports a case of a 13-year-old Saudi female patient with Gorlin-Goltz syndrome and includes an extensive literature review of the syndrome. To the extent of our knowledge, this is the first case reported by dentists in the Kingdom of Saudi Arabia.

Keywords: basal cell carcinoma; gorlin goltz syndrome; keratocystic odontogenic tumors.

Figure 1. Multiple skin lesions (red arrows) on the patient's arms.

Figure 2. An examination of the patient revealed multiple palmar pits (red arrows).

Figure 3. An examination of the patient revealed multiple plantar pits (red arrow).

Figure 4. An orthopantomogram (OPG) shows the maxillary and mandibular lesions before the surgery.

An orthopantomogram (OPG) shows a lesion associated with a partially erupted mandibular second molar tooth. The left mandibular lesion extended up to the ramus of the mandible (the gray arrows pointing down in the left side); moreover, the patient had another lesion associated with a horizontally impacted second premolar in the maxilla (the white arrows pointing up in the right side).

Figure 5. Cone beam computed tomography (CBCT) of another large lesion in the maxilla.

Cone beam computed tomography (CBCT) revealed another large lesion associated with the impacted right maxillary second molar, which extended up to the right maxillary sinus (white arrow).

Figure 6. Anteroposterior view of chest X-ray.

The anteroposterior view of a chest X-ray showed the presence of a bifid fifth rib on the left side (white box).

Figure 7. Orthopantomogram (OPG) after two months of follow-up.

An orthopantomogram (OPG) after two months of follow-up shows improvement and bone deposition (white arrows).