Anatomy

Maxilla

Internal medicine

Pathology

Odontogenic tumor

Lesion

Biology

CD34

Cancer

Vimentin

Immunohistochemistry

Proliferative index

Survivin

Stem cell

Cell biology

Medicine

Medicina oral, patología oral y cirugía bucal

Background: The primordial odontogenic tumor (POT) is a recently described benign entity with histopathological and immunohistochemical features suggesting its origin during early odontogenesis.Aim: To integrate the available data published on POT into a comprehensive analysis to better define its clinicopathological and molecular features.Material and Methods: An electronic systematic review was performed up to September 2019 in multiple databases.Results: A total of 13 publications were included, representing 16 reported cases and 3 molecular studies.The mean age of the affected patients was 11.6 years (range 2-19), with a slight predominance in males (56.25%).The posterior mandible was the main location (87.5%), with only two cases affecting the posterior maxilla.All cases appeared as a radiolucent lesion in close relationship to an unerupted tooth.Recurrences have not been reported to date.Microscopically, POT comprises fibromyxoid tissue with variable cellularity surrounded by a cuboidal to columnar odontogenic epithelium but without unequivocal dental hard tissue formation.A delicate fibrous capsule surrounds (at least partially) the tumor.The epithelial component shows immunohistochemical positivity for amelogenin, CK19, and CK14, and variable expression of Glut-1, Galectin-3 and Caveolin-1, Vimentin, p-53, PITX2, Bcl-2, Bax and Survivin; the mesenchymal tissue is positive for Vimentin, CD90, p-53, PITX2, Bcl-2, Bax, and Survivin, and the subepithelial region exhibits the strong expression of Syndecan-1 and CD34.The Ki-67 index is low (<5%).The negative or weak expression of dentinogenesis-associated genes could explain the inhibition of dentin and subsequent enamel formation in this neoplasm.Conclusion: POT is an entity with a well-defined clinicopathological, immunohistochemical and molecular profile that must be properly diagnosed and differentiated from other odontogenic lesions and treated consequently.

Primordial odontogenic tumor: A systematic review