Basal cell carcinoma arising in association with maxillary odontogenic keratocyst in a patient with Gorlin-Goltz syndrome

• Published in: Journal of oral and maxillofacial surgery, medicine, and pathology Vol. 34; no. 3; pp. 333 - 337
• Main Authors: Kawabe, Mutsuki, Tsukamoto, Yoshitane, Matuo, Shohei, Kanda, Shuji, Hashitani, Susumu
• Format: Journal Article
• Language: English
• Published: Elsevier Ltd 01.05.2022
• Subjects: Basal cell carcinoma; Gorlin-Goltz syndrome; Maxilla; Odontogenic keratocyst; Basal cell carcinoma; Gorlin-Goltz syndrome; Odontogenic keratocyst; Maxilla
• ISSN: 2212-5558; 2212-5566
• DOI: 10.1016/j.ajoms.2021.10.015

We report a case of Gorlin-Goltz syndrome in a patient who developed odontogenic keratocyst (OKC) and basal cell carcinoma (BCC) arising in the maxillary bone. A 39-year-old Japanese man was referred to our hospital because of gingival swelling surrounding the right upper teeth. He had undergone OKC enucleation twice at 9 and 17 years old. We observed gingival swelling and pus discharge in the right maxillary region on the first consultation. Panoramic radiography and computed tomography revealed two radiolucent lesions. We predicted that one lesion was recurrent and the other lesion was de novo. Furthermore, he had a fused rib, a bifid rib, and evident calcification of the falx cerebri. He had multiple BCCs in the skin simultaneously. Based on these findings, he was diagnosed with Gorlin-Goltz syndrome. We performed enucleation of the two maxillary cysts under general anesthesia. Pathological findings revealed that the recurring lesion was typical OKC, whereas the other de novo lesion was BCC, which is similar to the skin lesion in hematoxylin and eosin staining and immunohistochemical staining of p40. This indicated that the BCC arose from the maxillary OKC.