GORLIN SYNDROME SYNCHRONIC WITH ORAL SQUAMOUS CELL CARCINOMA: A CASE REPORT

• Published in: Oral surgery, oral medicine, oral pathology and oral radiology Vol. 130; no. 3; p. e119
• Main Authors: PANJWANI, CAMILA MARIA BEDER RIBEIRO GIRISH, FERREIRA, KELLY DE MOURA, VASCONCELOS, MARIANA DE LYRA, MELO, ISABELLE DE ARGOLO, PAIVA, JANAINA MARIA DOS SANTOS, LISBOA-NETO, JOSÉ DE AMORIM, COSTA, MARCELO DE ALMEIDA
• Format: Journal Article
• Language: English
• Published: Elsevier Inc 01-09-2020
• ISSN: 2212-4403; 2212-4411
• DOI: 10.1016/j.oooo.2020.04.047

The nevoid basal cell carcinoma syndrome (NBCCS) is a genetic disorder that affects the PTCH gene, which is highly associated with tumor suppression. It presents a case of NBCCS diagnosed in a 49-year-old Caucasian male. His medical history has shown a great number of surgical procedures for the removal of lesions that are common to the syndrome. One of the lesions was removed from the mandible and sent for biopsy. The patient was diagnosed by means of a jaw biopsy conducted by a buccomaxillofacial surgeon, and histopathologic sections showed a diagnosis of odontogenic keratocyst (OKC) and squamous cell carcinoma (SCC). Also, in the same section, some calcified materials were found that refer to psammoma bodies. The patient is still under care with an oncologic surgeon for future surgeries given the nature of the disorder. This case illustrates the importance of a multidisciplinary team in the diagnosis and treatment of patients with NBCCS.