Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2021 Oct 6;9(3):e073.
doi: 10.21142/2523-2754-0903-2021-073. eCollection 2021 Jul-Sep.

[Clinical and imaging characteristics of familial gigantiform cementoma. A review of the literature]

[Article in Spanish]
Grizel Karem Rodríguez-Cuentas  1
Affiliations
Review

[Clinical and imaging characteristics of familial gigantiform cementoma. A review of the literature]

[Article in Spanish]
Grizel Karem Rodríguez-Cuentas. Rev Cient Odontol (Lima). .

Abstract
in English, Spanish

Familial gigantiform cementoma (FGC) is a rare benign fibro-cementum lesion, which follows an autosomal dominant inheritance pattern and presents during childhood. It is limited to the bones of the face, with a predilection for the jaw, is fast growing and painless and expands considerably over time. It is considered among the seven disorders that affect the physiognomy of the craniofacial skeleton. Radiographically, FGC occurs in three stages of maturation similar to bone dysplasia, being radiolucent, mixed and radiopaque and is described as a mixed lobular well delimited mass, which can occur in both maxillae, causing expansion of the buccal and palatal / lingual bone cortices. displacement and retention of teeth. The aim of this study was to perform a review of the literature to identify the clinical, radiographic and histopathological characteristics of FGC in the jaws and describe the imaging tools that are useful for the diagnosis and follow-up of this lesion.

El cementoma gigantiforme familiar es una lesión fibro-ósea benigna poco común, que sigue un patrón de herencia autosómico dominante. Se presenta durante la infancia y se limita a los huesos de la cara, especialmente la mandíbula. Presenta crecimiento rápido e indoloro, y se expande considerablemente con el paso de los años. Está considerada entre los siete trastornos que afectan la fisonomía del esqueleto craneofacial. Radiográficamente, se produce en tres etapas de maduración, al igual que las displasias óseas: radiolúcida, mixta y radiopaca, y se describe como una masa lobular mixta, bien delimitada, que puede aparecer en ambos maxilares y provocar la expansión de corticales óseas vestibular y palatina/lingual, el desplazamiento y la retención de piezas dentarias. Este artículo tuvo el objetivo de realizar una revisión de la literatura más relevante para identificar las características clínicas, radiográficas e histopatológicas del cementoma gigantiforme familiar en los maxilares, además de mostrar las herramientas imagenológicas útiles para el diagnóstico y seguimiento de la lesión.

Keywords: Computed Tomography Cone Beam; Familial Gigantiform Cementoma; fibro-osseous injury.

PubMed Disclaimer

Conflict of interest statement

Potenciales conflictos de intereses: La autora declara no tener ningún conflicto de intereses

Similar articles

See all similar articles

References

    1. Wang HW, Ma CY, Qin XJ, Zhang CP. Management strategy in patient with familial gigantiform cementoma A case report and analysis of the literature. Medicine (Baltimore) 2017;96(50):e9138. doi: 10.1097/MD.0000000000009138. - DOI - PMC - PubMed
    1. Wang HW, Yu M, Qin XJ, Zhang CP. Familial gigantiform cementoma distinctive clinical features of a large Chinese pedigree. Br J Oral Maxillofac Surg. 2015;53(1):83–85. doi: 10.1016/j.bjoms.2014.09.013. - DOI - PubMed
    1. Shah S, Huh KH, Yi WJ, Heo MS, Lee SS, Choi SC. Follow-up CT findings of recurrent familial gigantiform cementoma of a female child. Skeletal Radiol. 2012;41(3):341–346. doi: 10.1007/s00256-011-1245-9. - DOI - PubMed
    1. Rossbach HC, Letson D, Lacson A, Ruas E, Salazar P. Familial gigantiform cementoma with brittle bone disease, pathologic fractures, and osteosarcoma a possible explanation of an ancient mystery. Pediatr Blood Cancer. 2005;44(4):390–396. doi: 10.1002/pbc.20253. - DOI - PubMed
    1. Mainville GN, Turgeon DP, Kauzman A. Diagnosis and management of benign fibro-osseous lesions of the jaws a current review for the dental clinician. Oral Dis. 2017;23(4):440–450. doi: 10.1111/odi.12531. - DOI - PubMed

LinkOut - more resources