Noonan-like Syndrome with Multiple Giant Cell Lesions in the Jaws: A Case Report
International Journal of Research and Reports in Dentistry,
Aims: To present a case report of a patient with Noonan syndrome who presented with multiple giant cell lesions in the mandible and describe the management and follow-up of this pathology, discussing the different treatment modalities and our experience.
Presentation of case: We present the case of a 7-year-old boy with Noonan phenotype and genetic SOS1 mutation who presented with bilateral giant cell lesions of the mandible. He underwent curettage and steroid injection, presenting with recurrence in the follow-up. Corticosteroid injections were decided given the small size of the lesion with good results. However, 2 years later, he presented with a new recurrence that required a new curettage. He is now free of recurrence, with satisfactory bone regeneration and occlusion.
Discussion: Giant cell lesions are frequently found in Noonan syndrome patients. They are usually multiple and indolent but can show an aggressive behaviour.
Different treatment modalities have been described in the management of giant cell lesions. Curettage is the most frequently used. Nonetheless, reports of high recurrence with this technique have been made in the literature and several authors have applied more aggressive surgical resections, with the high morbidity it entails.
Moreover, several adjunctive treatments have also been described, such as corticosteroid injections, interpheron alpha-2a, calcitonin, etc. with variable and unclear results.
Conclusion: We believe the combination of corticosteroids and surgical curettage is an adequate treatment since it can lower recurrence rates and minimizes severity and morbidity while preserving the dentition and mandibular morphology.
- Noonan syndrome
- giant cell lesions
- giant cell tumours
How to Cite
Ferriero K, Shah B, Yan Y, Khatri S, Caccamese J, Napoli JA, Bober MB and Crane JL. Case report: Safety and efficacy of denosumab in four children with noonan syndrome with multiple giant cell lesions of the jaw. Front. Pediatr. 2020;8:515.
Roberts AE, Allanson JE, Tartaglia M, Gelb BD. Noonan syndrome. Lancet. 2013;381:333–342.
Sinnott BP, Patel M. Giant cell lesion of the jaws as a presenting feature of Noonan syndrome. BMJ Case Rep; 2018.
Lee SM, Cooper JC. Noonan syndrome with giant cell lesions. Int J Paediatr Dent. 2005;15:140–5.
Cohen M, Gorlin RJ. Noonan-like/multiple giant cell lesion syndrome. American Journal of Medical Genetics. 1991;40:159–166.
De Lange J, van den Akker H, van den Berg H. Central giant cell granuloma of the jaw: a review of the literaturaewith empohasis on therapy options. Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2007;104:603-15.
Chuong R, Kaban LB, Kozakewich H, Perez-Atayde A. Central giant cell lesions of the jaws; a clinicopathologic study. J Oral Maxillofac Surg. 1986;44:708-13.
Ficarra G, Kaban LB, Hansen LS. Central giant cell lesions of the mandible and maxilla: a clinicopathologic and cytometric study. Oral Surg Oral Med Oral Pathol. 1987;64:44-9.
Pogrel MA. The diagnosis and management of giant cell lesions of the jaws. Annals of Maxillofacial Surgery. 2012;2:102–106.
Rodriguez FA, Castellon L, Moreno R, Paez E, Alvarez MI. Noonan syndrome with multiple Giant cell lesions, management and treatment with surgery and interferon alpha-2a therapy: case report. Birth Defects Research. 2020;1–8.
Kaban LB, Troulis MJ, Ebb D, August M, Hornicek FJ, Dodson TB. Antiangiogenic therapy with interferon alpha for giant cell lesions of the jaws. Journal of Oral and Maxillofacial Surgery. 2002;60:1103–1111.
Bataineh AB, Al-Khateeb T, Rawashdeh MA. The surgical treatment of central giant cell granuloma of the mandible. J Oral Maxillofac Surg. 2002;60:756-61.
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