{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,19]],"date-time":"2025-10-19T15:45:55Z","timestamp":1760888755457},"reference-count":26,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2012,8,8]],"date-time":"2012-08-08T00:00:00Z","timestamp":1344384000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Oral Pathology Medicine"],"published-print":{"date-parts":[[2013,3]]},"abstract":"<jats:sec><jats:title>Background<\/jats:title><jats:p>Nevoid basal cell carcinoma syndrome (<jats:styled-content style=\"fixed-case\">NBCCS<\/jats:styled-content>) is an autosomal dominant disorder and is characterized by tumorigenesis and physical deformity. Keratocystic odontogenic tumors (<jats:styled-content style=\"fixed-case\">KCOT<\/jats:styled-content>s) of the jaws are a common manifestation of this syndrome. This study involved a pooled analysis of Japanese individuals with <jats:styled-content style=\"fixed-case\">NBCCS<\/jats:styled-content> and was performed with the aim of analyzing the clinical features of <jats:styled-content style=\"fixed-case\">NBCCS<\/jats:styled-content> and the patterns of occurrence and recurrence of <jats:styled-content style=\"fixed-case\">KCOT<\/jats:styled-content>s in Japanese individuals.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>This study included 25 patients. The relative frequencies of the major symptoms in these patients were compared with those reported in the literature. We also investigated 11 patients with <jats:styled-content style=\"fixed-case\">KCOT<\/jats:styled-content>s (40 lesions) initially treated at <jats:styled-content style=\"fixed-case\">T<\/jats:styled-content>okyo <jats:styled-content style=\"fixed-case\">M<\/jats:styled-content>edical and <jats:styled-content style=\"fixed-case\">D<\/jats:styled-content>ental <jats:styled-content style=\"fixed-case\">U<\/jats:styled-content>niversity.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p><jats:styled-content style=\"fixed-case\">KCOT<\/jats:styled-content>s (100%) and palmar and\/or plantar pits (<jats:italic>n<\/jats:italic>\u00a0=\u00a019; 76.0%) were the most frequently observed manifestations. Eleven patients (44.0%) had a radiologically confirmed rib anomaly. Nineteen patients (76.0%) had a family history of the syndrome within first\u2010degree relatives. Japanese patients had a relatively low frequency of basal cell carcinoma (<jats:italic>n<\/jats:italic>\u00a0=\u00a07; 28.0%) and falx calcification (<jats:italic>n<\/jats:italic>\u00a0=\u00a07; 28.0%) compared with that reported in other populations.<\/jats:p><jats:p>Twelve of the total 40 <jats:styled-content style=\"fixed-case\">KCOT<\/jats:styled-content>s (30.0%) that were followed up for 6\u00a0months or more recurred. All recurrent cases had undergone conservative treatment, whereas no recurrences occurred in cases that had undergone radical treatment.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Recurrence of <jats:styled-content style=\"fixed-case\">KCOT<\/jats:styled-content>s associated with <jats:styled-content style=\"fixed-case\">NBCCS<\/jats:styled-content> is frequently encountered, and further investigations are required to confirm the optimal treatment that will ensure a complete cure improving the patient's quality of life.<\/jats:p><\/jats:sec>","DOI":"10.1111\/j.1600-0714.2012.01202.x","type":"journal-article","created":{"date-parts":[[2012,8,8]],"date-time":"2012-08-08T06:25:14Z","timestamp":1344407114000},"page":"275-280","source":"Crossref","is-referenced-by-count":16,"title":["Clinical manifestations and treatment for keratocystic odontogenic tumors associated with nevoid basal cell carcinoma syndrome: a study in 25 <scp>J<\/scp>apanese patients"],"prefix":"10.1111","volume":"42","author":[{"given":"Yasuyuki","family":"Shimada","sequence":"first","affiliation":[{"name":"Oral and Maxillofacial Surgery, Division of Oral Health Sciences, Department of Oral Restitution Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University Tokyo Japan"},{"name":"Global Center of Excellence (GCOE) Program, International Research Center for Molecular Science in Tooth and Bone Diseases, Tokyo Medical and Dental University Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kei\u2010ichi","family":"Morita","sequence":"additional","affiliation":[{"name":"Department of Advanced Molecular Diagnosis and Maxillofacial Surgery Hard Tissue Genome Research Center, Tokyo Medical and Dental University Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuji","family":"Kabasawa","sequence":"additional","affiliation":[{"name":"Oral and Maxillofacial Surgery, Division of Oral Health Sciences, Department of Oral Restitution Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Takahide","family":"Taguchi","sequence":"additional","affiliation":[{"name":"Oral and Maxillofacial Surgery, Division of Oral Health Sciences, Department of Oral Restitution Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University Tokyo Japan"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ken","family":"Omura","sequence":"additional","affiliation":[{"name":"Oral and Maxillofacial Surgery, Division of Oral Health Sciences, Department of Oral Restitution Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University Tokyo Japan"},{"name":"Global Center of Excellence (GCOE) Program, International Research Center for Molecular Science in Tooth and Bone Diseases, Tokyo Medical and Dental University Tokyo 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