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MULTIPLE ODONTOGENIC CYSTS ASSOCIATED WITH NEVOID BASAL CELL CARCINOMA SYNDROME

2017; Elsevier BV; Volume: 124; Issue: 2 Linguagem: Inglês

10.1016/j.oooo.2017.05.093

2212-4411

Patrícia Guerra Peixe Gonçalves, HUGO COSTA NETO, ANDRÉIA FERREIRA DO CARMO, Rodrigo Rodrigues Rodrigues, Adriano Rocha Germano, Bruno César de Vasconcelos Gurgel, Hébel Cavalcanti Galvão,

Hedgehog Signaling Pathway Studies

Nevoid basal cell carcinoma syndrome (NBCCS) is an autosomal dominant disorder characterized by wide variety of developmental abnormalities. Common symptoms include multiple basal cell carcinomas, recurrent keratocystic odontogenic tumors (KCOT), pits of the palms and soles, and skeletal malformations. Herein we report a case of NBCCS in a 22-year-old male patient with multiple cysts, 2 of them were diagnosed as odontogenic cysts and the others as KCOT. Clinical examination revealed ocular hypertelorism and facial asymmetry. Imaging tests showed calcifications in the falx cerebri, 3 osteolytic lesions in the anterior mandible region, and 1 in maxillar sinus. Treatment of choice was enucleation and the patient is being monitored. Based on clinical, radiologic, and histopathologic findings, the diagnosis of NBCCS was confirmed. Recurrence of KCOT associated with NBCCS is frequently encountered. This case leads us to believe in the possible association between NBCCS and other cystic lesions, in addition to KCOT.