Neurofibromatosis type 1 associated with bilateral central giant cell granuloma of the mandible Bruno Ramos Chrcanovic * , Ricardo Santiago Gomez, Belini Freire-Maia Department of Oral and Maxillofacial Surgery, School of Dentistry, (Head: Belini Freire-Maia), Pontifícia Universidade Católica de Minas Gerais, Av. Dom José Gaspar, 500 Prédio 45, Coração Eucarístico-30535-610, Belo Horizonte, MG, Brazil article info Article history: Paper received 17 October 2009 Accepted 4 October 2010 Keywords: Neurofibromatosis type 1 Central giant cell granuloma Mandible Treatment abstract Neurofibromatosis type 1, or von Recklinghausen disease, is one of the most common hereditary neu- rocutaneous disorders in humans. Clinically, Neurofibromatosis type 1 is characterized by café-au-lait spots, freckling, skin neurofibroma, plexiform neurofibroma, bony defects, Lisch nodules and tumors of the central nervous system. Central giant cell granuloma is a benign central lesion of bone, primarily involving the jaws, of variably aggressive nature characterized by aggregates of multinucleated giant cells in a background of cellular vascular fibrous connective tissue and spindle-shaped mononuclear stromal cells. The association between neurofibromatosis and central giant cell granuloma has been reported in the literature. A case of mandibular bilateral central giant cell granuloma in a patient with Neurofibro- matosis type 1 was conservatively but successfully treated by adequate surgical curettage of mandibular bone lesions. Ó 2010 European Association for Cranio-Maxillo-Facial Surgery. 1. Introduction Neurofibromatosis type 1 (NF1) is an autosomal dominant, multisystem disorder affecting approximately 1 in 3500 people (Boyd et al., 2009), caused by a spectrum of mutations affecting the neurofibromatosis 1 (Nf1) gene. The central giant cell granuloma (CGCG) of the jaws is usuallya non-neoplastic bone lesion accounting for fewer than 7% of all benign tumors of the jaws (Regezi, 2002), with an incidence rate of 1.1/million population annually (de Lange et al., 2004). The classification of the World Health Organization (Barnes et al., 2005) defined this entity as localized benign but sometimes aggressive osteolytic proliferation. Reports of CGCG in patients with NF1 have been described in the literature (Kerl and Schroll, 1971; Davis and Tideman, 1977; Van Damme and Mooren, 1994; Ardekian et al., 1999; Ruggieri et al., 1999; Krammer et al., 2003; de Lange and Van den Akker, 2005; Edwards et al., 2006). In the present report, a case of mandibular bilateral CGCG in a patient with NF1 was conservatively but successfully treated by adequate surgical curettage of mandibular bone lesions. It is also discussed in the differential diagnosis and treatment options. 2. Case report An 18-year-old female presented at his dentist in order to make a orthodontic treatment. A panoramic radiograph was requested as part of the complementary exams. The radiograph revealed a small radiolucent lesion between the right second mandibular molar and the impacted third molar, and a larger radiolucent lesion at the left side, covering the impacted third molar and reaching the ascending mandibular ramus, and the distal root of the second molar was totally involved (Fig. 1a). However, the orthodontist decided only to follow the case. The orthodontic device was mounted and 2 months later the patient was referred to another dentist, in order to perform the removal of the impacted third molars. This second professional saw the lesions and decided to request a more recent radiograph, to verify the evolution of the lesions. The second panoramic radio- graph showed that the right radiolucent lesion was larger (Fig. 1b). A right enlarged mandibular foramen, an inferiorly bilateral displaced coronoid notch, and a bilateral pseudoelongation of condylar process were seen in these radiographs (Fig. 1a and 1b). There were no signs of a possible diffusely invasive plexiform neurofibroma extending to the mandibular angle. The patient was then referred to the Department of Oral and Maxillofacial Surgery of the Pontifícia Universidade Católica de Minas Gerais, Belo Horizonte, Brazil. The patient’s examination showed prominent and posteriorly angulated ears but no swelling of bilateral cheeks, peripheral cutaneous neurofibromas mainly in the back and belly, café-au-lait * Corresponding author. Av. Raja Gabaglia 1000/1209, Gutierrez, Belo Horizonte, Minas Gerais, CEP 30441-070, Brazil. Tel.: þ55 31 32920997 (office)/33356892 (home)/91625090 (mobile); fax: þ55 31 25151579. E-mail address: brunochrcanovic@hotmail.com (B.R. Chrcanovic). Contents lists available at ScienceDirect Journal of Cranio-Maxillo-Facial Surgery journal homepage: www.jcmfs.com 1010-5182/$ e see front matter Ó 2010 European Association for Cranio-Maxillo-Facial Surgery. doi:10.1016/j.jcms.2010.10.014 Journal of Cranio-Maxillo-Facial Surgery xxx (2010) 1e6 Please cite this article in press as: Chrcanovic BR, et al., Neurofibromatosis type 1 associated with bilateral central giant cell granuloma of the mandible, Journal of Cranio-Maxillo-Facial Surgery (2010), doi:10.1016/j.jcms.2010.10.014