Management of Odontogenic Keratocysts Tumors Associated with Gorlin-Goltz Syndrome. A Literature Review Based on a Case Report
PDF (Spanish)
PDF (Inglés) (Spanish)

Keywords

Gorlin‐Goltz Syndrome
Basal cell Nevus Syndrome
Odontogenic Keratocyst
Vismodegib
Sonidegib

How to Cite

Management of Odontogenic Keratocysts Tumors Associated with Gorlin-Goltz Syndrome. A Literature Review Based on a Case Report. (2025). Odontoestomatología, 27(45). https://doi.org/10.22592/ode2025n45e423

Abstract

The aim of this article is to describe and discuss, based on the 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 presented again with recurrent OKCs, and a new approach was chosen: enucleation and cavity treatment with MCS for the smaller OKC, while decompression was performed for the larger OKC. Currently, the clinical management of OKCs does not differ between individuals with and without GGS, despite their clinical and molecular differences. Therefore, we highlight the need to establish a specific treatment for OKCs in patients with GGS, which could consider Sonic Hedgehog (SHH) pathway inhibitors as an adjuvant therapy.

PDF (Spanish)
PDF (Inglés) (Spanish)

References

Murgia G, Valtellini L, Denaro N, Nazzaro G, Bortoluzzi P, Benzecry V, et al. Gorlin syndrome-associated basal cell carcinomas treated with vismodegib or sonidegib: A retrospective study. Cancers (Basel) 2024;16:2166.

Spadari F, Pulicari F, Pellegrini M, Scribante A, Garagiola U. Multidisciplinary approach to Gorlin-Goltz syndrome: from diagnosis to surgical treatment of jawbones. Maxillofac Plast Reconstr Surg 2022;44:25.

Fernández LT, Ocampo-Garza SS, Elizondo-Riojas G, Ocampo-Candiani J. Basal cell nevus syndrome: an update on clinical findings. Int J Dermatol 2022;61:1047–55. (6)

Kesireddy M, Mendiola VL, Jana B, Patel S. Long-term response to vismodegib in a patient with Gorlin-Goltz syndrome: A case report and review of pathological mechanisms involved. Cureus 2019;11:e5383.

Akbari M, Chen H, Guo G, Legan Z, Ghali G. Basal cell nevus syndrome (Gorlin syndrome): genetic insights, diagnostic challenges, and unmet milestones. Pathophysiology 2018;25:77–82.

Booms P, Harth M, Sader R, Ghanaati S. Vismodegib hedgehog-signaling inhibition and treatment of basal cell carcinomas as well as keratocystic odontogenic tumors in Gorlin syndrome. Ann Maxillofac Surg 2015;5:14–9.

Ambele MA, Robinson L, van Heerden MB, Pepper MS, van Heerden WFP. Comparative molecular genetics of odontogenic keratocysts in sporadic and syndromic patients. Mod Pathol 2023;36:100002.

Schuch LF, de Arruda JAA, Mosconi C, Kirschnick LB, Pinho RFC, Viveiros SK, Abreu LG, do Amaral-Silva GK, da Silva LP, Martins-Chaves RR, Vasconcelos ACU, Gomes APN, Rivero ERC, Martins MD, Rados PV, de Almeida OP, de Andrade BAB, Romañach MJ, Agostini M, de Mendonça EF, Pontes HAR, de Souza LB, Fonseca FP, de Aguiar MCF, de Sousa SCOM, Mesquita RA. A Brazilian multicentre study of 2,497 isolated cases of odontogenic keratocysts. Oral Dis. 2020 Apr;26(3):711-715. doi: 10.1111/odi.13278. Epub 2020 Feb 14. PMID: 31917876.

Titinchi, F., & Nortje, C. J. (2012). Keratocystic odontogenic tumor: a recurrence analysis of clinical and radiographic parameters. Oral surgery, oral medicine, oral pathology and oral radiology, 114(1), 136-142.

Al-Moraissi EA, Kaur A, Gomez RS, Ellis E 3rd. Effectiveness of different treatments for odontogenic keratocyst: a network meta-analysis. Int J Oral Maxillofac Surg 2023;52:32–43.

Bree AF, Shah MR, BCNS Colloquium Group. Consensus statement from the first international colloquium on basal cell nevus syndrome (BCNS). Am J Med Genet A 2011;155A:2091–7.

Tang JY, Ally MS, Chanana AM, Mackay-Wiggan JM, Aszterbaum M, Lindgren JA, et al. Inhibition of the hedgehog pathway in patients with basal-cell nevus syndrome: final results from the multicentre, randomised, double-blind, placebo-controlled, phase 2 trial. Lancet Oncol 2016;17:1720–31.

Winters R, Garip M, Meeus J, Coropciuc R, Politis C. Safety and efficacy of adjunctive therapy in the treatment of odontogenic keratocyst: a systematic review. Br J Oral Maxillofac Surg. 2023 Jun;61(5):331-336. doi: 10.1016/j.bjoms.2023.04.006. Epub 2023 Apr 25. PMID: 37248124.

Mehta D, Raval N, Patadiya H, Tarsariya V. Gorlin-goltz syndrome. Ann Med Health Sci Res. 2014;4(2):279-282. doi:10.4103/2141-9248.129064

Singh AK, Khanal N, Chaulagain R, Bhujel N, Singh RP. How effective is 5-Fluorouracil as an adjuvant in the management of odontogenic keratocyst? A systematic review and meta-analysis. Br J Oral Maxillofac Surg. 2022;60(6):746-754. doi:10.1016/j.bjoms.2022.02.001

Palmeiro AG, Carvalho M, Gonçalves Castro C, Pimentel B, Catorze G. Vismodegib in Gorlin-Goltz syndrome: A systematic review. Australas J Dermatol 2024;65.

Villani, Alessia, et al. Sonidegib: safety and efficacy in treatment of advanced basal cell carcinoma. Dermatology and therapy, 2020, vol. 10, p. 401-412.

Creative Commons License

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

Copyright (c) 2025 Arturo Javier Henriquez Avendaño, Sebastián Alberto Galleguillos Conejeros, Jose Fernandez, Mauricio Lobos Esparza, Marcelo Mardones Muñoz, Luis Alberto Córdova Jara