Central giant cell lesions with an unusual behavior in patient with Noonan syndrome: A case report with 8-year follow-up

Authors

DOI:

https://doi.org/10.33448/rsd-v10i1.11563

Keywords:

Giant cell granuloma; Noonan syndrome; Multimodal treatment; Calcitonin.

Abstract

This study describes a patient with Noonan syndrome affected by multiple Central Giant Cell Lesions (CGCL) in jaws. The lesions presented an unusual behavior since there was no regression size after puberty. The syndrome was diagnosed by collecting clinical information, represented by ocular hypertelorism, low insertion of ears, pulmonary stenosis, cryptorchidism, cardiac abnormalities, short stature, multiple CGCL in the jaws, and blood analysis that found a mutation of the PTPN11 gene. The treatment consisted of systemic calcitonin for a period of 14 months and three surgical procedures at distinct moments. The patient is currently with 20 years and in the eighth-year of follow-up. Although he presented an improvement in deformity, radiological findings showed remodeling without resolution of mandibular injuries, making it clear that injuries will did not always regress after puberty and not confirm previously publications in the literature. We therefore advocate a larger time of follow-up before patient discharge in these cases.

Author Biographies

Gleysson Matias de Assis, Federal University of Rio Grande do Norte

DDS, Ms, Oral and Maxillofacial Surgeon and postgraduate student Program in Health Public/ Department of Dentistry-UFRN

Marcelo Leite Machado da Silveira, Federal University of Rio Grande do Norte

DDS, Ms, Oral and Maxillofacial Surgeon and postgraduate student Program in Health Public/ Department of Dentistry-UFRN

José Wittor de Macêdo Santos, Federal University of Rio Grande do Norte

DDS, Oral and Maxillofacial Surgeon Resident from the Oral and Maxillofacial Surgery Residency Program, Department of Dentistry-UFRN

Humberto Pereira Chaves Neto, Federal University of Rio Grande do Norte

DDS, Oral and Maxillofacial Surgeon Resident from the Oral and Maxillofacial Surgery Residency Program, Department of Dentistry-UFRN

Lucas Melo da Costa, Federal University of Rio Grande do Norte

DDS, Postgraduate student of Dental Science Program - Oral Pathology/Department of Dentistry-UFRN

Sérgio Ricardo Fernandes de Araújo, Federal University of Rio Grande do Norte

Ms, PhD. Professor of Pharmacology, Department of Pharmacy-UFRN

Márcia Cristina da Costa Miguel, Federal University of Rio Grande do Norte

DDS, Ms, PhD. Professor of Oral Pathology, Department of Dentistry -UFRN

Adriano Rocha Germano, Federal University of Rio Grande do Norte

DDS, Ms, PhD. Professor of Oral and Maxillofacial Surgery, Department of Dentistry -UFRN. Corresponding author.

References

Aditya, A., & Aditya, P. (2016). Central Giant Cell Granuloma of Jaw with Multiple, Multifocal Recurrences. J Clin Diagn Res, 10(8), ZJ03-ZJ04. https://doi.org/10.7860/jcdr/2016/20357.8263.

Bertola, D. R., Pereira, A. C., Albano, L. M., De Oliveira, P. S., Kim, C. A., & Krieger, J. E. (2006). PTPN11 gene analysis in 74 Brazilian patients with Noonan syndrome or Noonan-like phenotype. Genet Test, 10(3), 186-191. https://doi.org/10.1089/gte.2006.10.186.

Chrcanovic, B. R., Gomes, C. C., dos Santos, T. R., Abreu, M. H. N. G., & Gomez, R. S. (2019). Clinical factors associated with the recurrence of central giant cell lesions. J Oral Pathol Med, 48(9), 799-802. https://doi.org/10.1111/jop.12937.

Chrcanovic, B. R., Gomes, C. C., & Gomez, R. S. (2018). Central giant cell lesion of the jaws: An updated analysis of 2270 cases reported in the literature. J Oral Pathol Med, 47(8), 731-739. https://doi.org/10.1111/jop.12730.

Cohen, M. M., Gorlin, R. J. (1991). Noonan-like/multiple giant cell lesion syndrome. Am J Med Genet, 40(2), 159-166. https://doi.org/10.1002/ajmg.1320400208.

Edwards, P. C., Fox, J., Fantasia, J. E., Goldberg, J., & Kelsch, R. D. (2005). Bilateral central giant cell granulomas of the mandible in an 8-year-old girl with Noonan syndrome (Noonan-like/multiple giant cell lesion syndrome). Oral Surg Oral Med Oral Pathol Oral Radiol Endod, 99(3), 334-340. https://doi.org/10.1016/j.tripleo.2004.08.021.

Feng, G. S. (1999). Shp-2 tyrosine phosphatase: signaling one cell or many. Exp Cell Res, 253(1), 47-54. https://doi.org/10.1006/excr.1999.4668.

Ferretti, C., & Muthray, E. (2011). Management of Central Giant Cell Granuloma of Mandible Using Intralesional Corticosteroids: Case Report and Review of Literature. J Oral Maxillofac Surg, 69(11), 2824-2829. https://doi.org/10.1016/j.joms.2010.11.020.

Jamieson, C. R., Van Der Burgt, I., Brady, A. F., Van Reen, M., Elsawi, M. M., Hol, F., Jeffery, S., Patton, M. A., Mariman, E. (1994). Mapping a gene for Noonan syndrome to the long arm of chromosome 12. Nat Genet, 8(4), 357-360. https://doi.org/10.1038/ng1294-357.

Jerkins, D., Malotky, M., Miremadi, R., & Dole, M. (2016). Central Giant Cell Granuloma of the Mandible Requiring Multiple Treatment Modalities: A Case Report. J Oral Maxillofac Surg, 74(8), 1596-1607. https://doi.org/10.1016/j.joms.2016.02.019.

Lange, J., Akker, H. P., & Berg, H. (2007). Central giant cell granuloma of the jaw: a review of the literature with emphasis on therapy options. Oral Surg Oral Med Oral Pathol Oral Radiol Endod, 104(5), 603-615. https://doi.org/10.1016/j.tripleo.2007.04.003.

Losler, B. K., Diallo, R., Gaertner, C., Mischke, K. L., Joos, U., & Kleinheinz, J. (2006). Central giant cell granuloma of the jaws: a clinical, radiologic, and histopathologic study of 26 cases. Oral Surg Oral Med Oral Pathol Oral Radiol Endod, 101(3), 346-354. https://doi.org/10.1016/j.tripleo.2005.02.060.

Lotfi, A., Moghadam, S. A., & Moghadam, F. A. (2013). Multiple Central Giant Cell Granuloma of the Mandible: A Case Report. Journal of Dentomaxillofacial Radiology, Pathology and Surgery, 1(2), 28-31. http://dx.doi.org/10.18869/acadpub.3dj.2.1.5.

Noonan, J. A., & Ehmke, D. A. (1963). Associated noncardiac malformations in children with congenital heart disease. Journal of Pediatrics, 63(3), 468-470.

Pereira, A. S., Shitsuka, D. M., Parreira, F. J., & Shitsuka, R. (2018). Metodologia do trabalho científico. [e-Book]. Santa Maria. Ed. UAB/NTE/UFSM. https://repositorio. ufsm. br/bitstream/handle/1/15824/Lic_Computacao_MetodologiaPesquisa-Cientifica. pdf.

Sandhya, T., Avinash, T., Snehal, D., Neha, T., & Uma, M. (2016). Multifocal Central Giant Cell Granuloma - A Case Report. Iran J Pathol, 11(3), 276-280.

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Published

05/01/2021

How to Cite

ASSIS, G. M. de; SILVEIRA, M. L. M. da; SANTOS, J. W. de M.; CHAVES NETO, H. P. .; COSTA, L. M. da; ARAÚJO, S. R. F. de; MIGUEL, M. C. da C.; GERMANO, A. R. Central giant cell lesions with an unusual behavior in patient with Noonan syndrome: A case report with 8-year follow-up . Research, Society and Development, [S. l.], v. 10, n. 1, p. e13510111563, 2021. DOI: 10.33448/rsd-v10i1.11563. Disponível em: https://rsdjournal.org/index.php/rsd/article/view/11563. Acesso em: 18 jan. 2021.

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Section

Health Sciences