Sturge-Weber syndrome with cemento-ossifying fibroma in the maxilla and giant odontoma in the mandible: A case report
Aki Sato,
Hisako Furusho,
Tatsushi Matsumura,
Makoto Nakano,
Koichi Sawaki,
Yohsuke Yoshioka,
Sho Akashi,
Mutsumi Miyauchi,
Nobuyoshi Mizukawa,
Seiji Iida
Affiliations
Aki Sato
Department of Dentistry and Oral Surgery, Hiroshima City Hiroshima Citizens Hospital, Hiroshima, Japan; Corresponding author. 7-33 Motomachi, Naka-ku, Hiroshima, Hiroshima, p/o 730-8518,Japan.
Hisako Furusho
Department of Pathology, Hiroshima City Hiroshima Citizens Hospital, Hiroshima, Japan; Department of Oral and Maxillofacial Pathobiology, Hiroshima University Graduate School of Biomedical and Health Sciences, Hiroshima, Japan
Tatsushi Matsumura
Department of Oral and Maxillofacial Surgery, Wakayama Medical University, Wakayama, Japan
Makoto Nakano
Department of Dentistry and Oral Surgery, Hiroshima City Hiroshima Citizens Hospital, Hiroshima, Japan
Koichi Sawaki
Department of Dentistry and Oral Surgery, Hiroshima City Hiroshima Citizens Hospital, Hiroshima, Japan
Yohsuke Yoshioka
Department of Dentistry and Oral Surgery, Hiroshima City Hiroshima Citizens Hospital, Hiroshima, Japan
Sho Akashi
Department of Dentistry and Oral Surgery, Hiroshima City Hiroshima Citizens Hospital, Hiroshima, Japan
Mutsumi Miyauchi
Department of Oral and Maxillofacial Pathobiology, Hiroshima University Graduate School of Biomedical and Health Sciences, Hiroshima, Japan
Nobuyoshi Mizukawa
Department of Dentistry, Kurashiki Central Hospital, Okayama, Japan
Seiji Iida
Department of Dentistry and Oral Surgery, Hiroshima City Hiroshima Citizens Hospital, Hiroshima, Japan; Department of Oral and Maxillofacial Reconstructive Surgery, Okayama University Hospital, Okayama, Japan; Department of Oral and Maxillofacial Reconstructive Surgery, Okayama University Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama, Japan
Sturge-Weber syndrome (SWS) is a neurocutaneous syndrome with vascular lesions of the cerebral meninges, port wine spots on the face, and glaucoma of the eyes; it is a congenital, non-genetic disease whose etiology and mechanisms are unknown. In this report, we describe a rare case of SWS with unilateral large odontogenic tumors in the maxilla and mandible. The histopathological diagnosis of the maxillary bone lesion on biopsy was juvenile psammomatoid ossifying fibroma, which is considered a type of ossifying fibroma of craniofacial bone origin. However, the final pathological diagnosis of the excision was cemento-ossifying fibroma derived from periodontal ligament cells, and we discuss the histopathology in detail. In addition, the mandibular lesion was one of the largest odontomas reported to date. Furthermore, in this case, we suggest the possibility that the maxillary and mandibular bone lesions are not separate lesions, but a series of lesions related to SWS.
