Ameloblastoma associated with syndromes: A systematic review

Area of Science:

• Oral Pathology
• Genetics
• Oncology

Background:

• Odontogenic lesions, including ameloblastoma, are observed in various genetic syndromes.
• Despite reported associations, ameloblastoma is not a diagnostic criterion for any syndrome.

Purpose of the Study:

• To systematically review and compile data on ameloblastoma cases associated with genetic syndromes.
• To analyze demographic, clinical, and radiographic features of these associated cases.

Main Methods:

• Systematic electronic search of PubMed Central database.
• Inclusion of human case reports and case series published in English from 1975 to 2018.
• Data extraction included associated syndrome, patient demographics, lesion location, radiographic features, and treatment.

Main Results:

• Ameloblastoma was associated with Gorlin syndrome (6 cases), epidermal nevus syndrome (2 cases), Gardner syndrome (2 cases), Simpson-Golabi-Behmel syndrome (1 case), and Williams syndrome (1 case).
• Gorlin syndrome was the most frequent association (50%), with a predilection for the maxilla and female patients.
• Epidermal nevus syndrome cases involved the mandible and occurred in female patients.

Conclusions:

• Ameloblastoma is associated with multiple genetic syndromes, with Gorlin syndrome being the most common.
• Identifying ameloblastic changes is crucial in syndromes featuring odontogenic lesions.
• Further research into these associations may elucidate ameloblastoma pathogenesis.