Management Of Odontogenic Keratocysts In Gorlin-Goltz Syndrome
DOI:
https://doi.org/10.34635/rpc.1025Keywords:
Basal Cell Nevus Syndrome, Nonodontogenic Cysts, Basal Cell CarcinomaAbstract
Introduction: Gorlin-Goltz Syndrome is presented by a dominant genetic alteration that can lead to changes throughout the body. Among the most common changes are keratocysts, intraosseous pathological lesions with a high recurrence rate in the maxillofacial regions. Early diagnosis is extremely important, since individuals with the syndrome are susceptible to developing basal cell carcinomas in epithelial tissue.Case Report: Patient affected by a rare syndrome (Gorlin-Goltz Syndrome) with changes diagnosed in different regions of the body such as: keratocysts, basal cell carcinomas, chest excavatum, calcification of the cerebral falx, among others. His treatment was carried out in a multidisciplinary manner associated with surgical treatment and the use of antineoplastic medication, which resulted in significant improvement in relation to his basal cell carcinomas.
Discussion: GGS is a rare condition, with a complex diagnosis, since the syndrome can lead.
Conclusion: to several clinical and imaging changes, which may or may not be present in the individual. Gorlin-Goltz syndrome presents several clinical manifestations, with keratocyst lesions being one of the most important. Multidisciplinary treatment is indicated, as the syndrome presents several changes, requiring long-term monitoring of these individuals.
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References
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2- Neville, BW.; Allen, CM. et al. Patologia: Oral & Maxilofacial. 4ª ed 2016
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7- Garg, P.; Karjodkar, F. et al. Gorlin-Goltz Syndrome – case report. J Clin Diag Resea., 5:393-395, 2011.
8- Stolz, ASB.; Oliveira, MO. et al. Proservação pós-operatória de um paciente com Síndrome de Gorlin- -Goltz – relato de caso. Rev Sul-Brasileira Odontol., 7:226-230, 2010.
9- Gupta, SR.; Jaetli, V. et al. Nevoid basal cell carcinoma syndrome in Indian patients: a clinical and radiological study of 6 cases and review of literature. Oral Maxillofac Pathol., 113:99-110, 2012.
10. Evans, DGR.; Ladusans, EJ. Et al. Complications of the naevoid basal cell carcinoma syndrome: results of a population based study. J Med Genet., 30: 460–464, 1993.
11. Kimonis, VE.; Goldstein, AM. et al. Clinical manifestations in 105 persons with nevoid basal cell carcinoma syndrome. Am J Med Genet., 59: 299–308, 1997.
12. Gorlin RJ. Nevoid Basal-Cell Carcinoma Syndrome. Medicine. MEDICINE., 66(2):98–113, 1987.
13. Parveen, AL.; Nisar, AW. et al. Topical 5-fluorouracil application in management of odontogenic keratocysts. J Oral Biology Craniof Res., 10, 404-406, 2020.
14. Pogrel, MA. The keratocystic odontogenic tumour (KCOT)--an odyssey.Int. J. Oral Maxillofac. Surg., 44(12):1565-8, 2015.
15. Al-Moraissi, EA.; Dahan, AA. et al. What surgical treatment has the lowest recurrence rate following the management of keratocystic odontogenic tumor? A large systematic review and meta-analysis. J Cranio-Maxillof Surg., 45:131-144, 2017.
16. Chrcanovic, BR.; Gomez, RS. Recurrence probability for keratocystic odontogenic tumors: An analysis of 6,427 cases. J Cranio-Maxillofac Surg, 2016.
17. Caminiti, M.; El-Rabbany, M. et al. 5-Fluorouracil is Associated with a Decreased Recurrence Risk in Odontogenic Keratocyst Management: A Retrospective Cohort Study. J Oral Maxillofac Surg. 2020.
18. Fisher, AD.; Williams, DF. et al. The effect of cryosurgery on the strength of bone. Br J Oral Surg 15: 215, 1978.
19. Dashow, JE.; Mchugh, JB. et al. Significantly decreased recurrence rates in keratocystic odontogenic tumor with simple enucleation and curettage using carnoy’s versus modified carnoy’s solution. J Oral Maxillofac Surg 73: 2132, 2015.
20. Xavier, C.; Lopes, E. et al. Vismodegib for treatment of periocular basal cell carcinoma – 6-year experience from a tertiary cancer center. Anais Brasil Dermato, 2021.
21. Booms, P.; Harth, M. et al. Vismodegib hedgehog-signaling inhibition and treatment of basal cell carcinomas as well as keratocystic odontogenic tumors in Gorlin syndrome. Annals Maxilofac Surg., 2015.
22. N, Tang.; D, Ratner. Implementation of Systemic Hedgehog Inhibitors in Daily Practice as Neoadjuvant Therapy. J Natl Compr Canc Netw., 15:537-543, 2017.
2- Neville, BW.; Allen, CM. et al. Patologia: Oral & Maxilofacial. 4ª ed 2016
3- Muzio, L. Nevoid basal cell carcinoma syndrome (Gorlin syndrome). Orphanet Rare Diseases., 3:32, 2008.
4- Valiati, AA.; Pavelecini, M. et al. Síndrome do nevo basocelular. Arq Catarinenses Med., 38:88-95, 2009.
5- Magro, AK.; Ottoni, R. et al. Síndrome de gorlin-goltz – relato de casos. Rev Fac Odontol – Upf., 19:239-244, 2014.
6- Hilgert, R.; Fonseca, LAM. et al. Síndrome de Gorlin: relato de envolvimento familiar. Rev Cir Traumatol Buco- Maxilo-Fac., 10:39-44, 2010.
7- Garg, P.; Karjodkar, F. et al. Gorlin-Goltz Syndrome – case report. J Clin Diag Resea., 5:393-395, 2011.
8- Stolz, ASB.; Oliveira, MO. et al. Proservação pós-operatória de um paciente com Síndrome de Gorlin- -Goltz – relato de caso. Rev Sul-Brasileira Odontol., 7:226-230, 2010.
9- Gupta, SR.; Jaetli, V. et al. Nevoid basal cell carcinoma syndrome in Indian patients: a clinical and radiological study of 6 cases and review of literature. Oral Maxillofac Pathol., 113:99-110, 2012.
10. Evans, DGR.; Ladusans, EJ. Et al. Complications of the naevoid basal cell carcinoma syndrome: results of a population based study. J Med Genet., 30: 460–464, 1993.
11. Kimonis, VE.; Goldstein, AM. et al. Clinical manifestations in 105 persons with nevoid basal cell carcinoma syndrome. Am J Med Genet., 59: 299–308, 1997.
12. Gorlin RJ. Nevoid Basal-Cell Carcinoma Syndrome. Medicine. MEDICINE., 66(2):98–113, 1987.
13. Parveen, AL.; Nisar, AW. et al. Topical 5-fluorouracil application in management of odontogenic keratocysts. J Oral Biology Craniof Res., 10, 404-406, 2020.
14. Pogrel, MA. The keratocystic odontogenic tumour (KCOT)--an odyssey.Int. J. Oral Maxillofac. Surg., 44(12):1565-8, 2015.
15. Al-Moraissi, EA.; Dahan, AA. et al. What surgical treatment has the lowest recurrence rate following the management of keratocystic odontogenic tumor? A large systematic review and meta-analysis. J Cranio-Maxillof Surg., 45:131-144, 2017.
16. Chrcanovic, BR.; Gomez, RS. Recurrence probability for keratocystic odontogenic tumors: An analysis of 6,427 cases. J Cranio-Maxillofac Surg, 2016.
17. Caminiti, M.; El-Rabbany, M. et al. 5-Fluorouracil is Associated with a Decreased Recurrence Risk in Odontogenic Keratocyst Management: A Retrospective Cohort Study. J Oral Maxillofac Surg. 2020.
18. Fisher, AD.; Williams, DF. et al. The effect of cryosurgery on the strength of bone. Br J Oral Surg 15: 215, 1978.
19. Dashow, JE.; Mchugh, JB. et al. Significantly decreased recurrence rates in keratocystic odontogenic tumor with simple enucleation and curettage using carnoy’s versus modified carnoy’s solution. J Oral Maxillofac Surg 73: 2132, 2015.
20. Xavier, C.; Lopes, E. et al. Vismodegib for treatment of periocular basal cell carcinoma – 6-year experience from a tertiary cancer center. Anais Brasil Dermato, 2021.
21. Booms, P.; Harth, M. et al. Vismodegib hedgehog-signaling inhibition and treatment of basal cell carcinomas as well as keratocystic odontogenic tumors in Gorlin syndrome. Annals Maxilofac Surg., 2015.
22. N, Tang.; D, Ratner. Implementation of Systemic Hedgehog Inhibitors in Daily Practice as Neoadjuvant Therapy. J Natl Compr Canc Netw., 15:537-543, 2017.
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Published
2024-07-29
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