Clinicopathological and molecular insights into odontogenic tumors associated with syndromes: A comprehensive review

Table 1 Summarize findings of syndromic conditions and odontogenic tumors

Odontogenic tumor	Syndrome	Genetic alteration	General clinical condition
Odontoma	Gardner syndrome	Mutations in the APC gene	Multiple colorectal polyps and various types of tumors, both benign and malignant
	Otodental syndrome	Not yet established (chromosome 11q13 deletion syndrome)	Globodontia, sensorineural high-frequency hearing loss and ocular coloboma
Adenomatoid odontogenic tumor	Schimmelpenning syndrome	Postzygotic mutations in RAS genes	One or several nevus sebaceous with abnormalities of ocular, cardiac, skeletal, and nervous systems
	Attenuated familial adenomatosis polyposis	Mutations in the APC gene, with less common occurrences linked to mutations in the MUTYH gene	Hundreds or thousands of adenomatous polyps in the large bowel
Ameloblastoma	Gardner syndrome	Mutations in the APC gene	Multiple colorectal polyps and various types of tumors, both benign and malignant
	Gorlin syndrome	Mutations in the patched (PTCH) gene	Numerous basal cell carcinomas and accompanying skeletal, ophthalmological, and neurological abnormalities
	Schimmelpenning syndrome	Postzygotic mutations in RAS genes	One or several nevus sebaceous with abnormalities of ocular, cardiac, skeletal, and nervous systems
	Simpson-Golabi-Behmel syndrome	Mutations in a semi-dominant X-linked gene encoding Glypican 3	Pre- and postnatal overgrowth, distinctive facial anomalies, and abnormalities affecting internal organs, the skeleton, and occasionally, varying degrees of intellectual disability
	Williams syndrome	Deletion of genes on chromosome 7q11.23	Developmental delay, intellectual disability, a specific cognitive profile, unique personality characteristics, cardiovascular disease, connective tissue abnormalities, growth deficiency, endocrine abnormalities, and distinctive facies
Odontogenic myxoma	Gardner syndrome	Mutations in the APC gene	Multiple colorectal polyps and various types of tumors, both benign and malignant

APC: Adenomatous polyposis coli.