Resumo

AVENDANO, Arturo Javier Henríquez et al. Management of Odontogenic Keratocysts Tumors Associated with Gorlin-Goltz Syndrome. A Literature Review Based on a Case Report. Odontoestomatología [online]. 2025, vol.27, n.45, e423.  Epub 01-Jun-2025. ISSN 0797-0374.  https://doi.org/10.22592/ode2025n45e423.

This article aims to describe and discuss, based on current literature, a case of a young patient with Gorlin-Goltz syndrome (GGS), highlighting the therapeutic approach and the challenge posed by the high recurrence rate of odontogenic keratocysts (OKCs) in GGS. We present a 19-year-old patient with GGS and multiple OKCs treated through decompression, enucleation, and cavity treatment with modified Carnoy’s solution (MCS). Three years later, the patient returned with recurrent OKCs, and a new approach was taken: enucleation and cavity treatment with MCS for the smaller OKC, while decompression was performed for the larger OKC. Currently, OKC clinical management does not differ between individuals with and without GGS, despite their clinical and molecular differences. Therefore, we highlight the need for a specific treatment for OKCs in GGS patients, which could include Sonic Hedgehog (SHH) pathway inhibitors as adjuvant therapy.

Palavras-chave : Gorlin‐Goltz Syndrome; Basal Cell Nevus Syndrome; Odontogenic Keratocyst; Vismodegib; Sonidegib.

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