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Clinical manifestations and treatment for keratocystic odontogenic tumors associated with nevoid basal cell carcinoma syndrome: a study in 25 J apanese patients
Author(s) -
Shimada Yasuyuki,
Morita Keiichi,
Kabasawa Yuji,
Taguchi Takahide,
Omura Ken
Publication year - 2013
Publication title -
journal of oral pathology and medicine
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.887
H-Index - 83
eISSN - 1600-0714
pISSN - 0904-2512
DOI - 10.1111/j.1600-0714.2012.01202.x
Subject(s) - nevoid basal cell carcinoma syndrome , keratocystic odontogenic tumor , basal cell nevus syndrome , medicine , calcification , falx cerebri , basal cell carcinoma , pathology , dermatology , odontogenic , basal cell
Background Nevoid basal cell carcinoma syndrome ( NBCCS ) is an autosomal dominant disorder and is characterized by tumorigenesis and physical deformity. Keratocystic odontogenic tumors ( KCOT s) of the jaws are a common manifestation of this syndrome. This study involved a pooled analysis of Japanese individuals with NBCCS and was performed with the aim of analyzing the clinical features of NBCCS and the patterns of occurrence and recurrence of KCOT s in Japanese individuals. Methods 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 KCOT s (40 lesions) initially treated at T okyo M edical and D ental U niversity. Results KCOT s (100%) and palmar and/or plantar pits ( n = 19; 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‐degree relatives. Japanese patients had a relatively low frequency of basal cell carcinoma ( n = 7; 28.0%) and falx calcification ( n = 7; 28.0%) compared with that reported in other populations. Twelve of the total 40 KCOT s (30.0%) that were followed up for 6 months or more recurred. All recurrent cases had undergone conservative treatment, whereas no recurrences occurred in cases that had undergone radical treatment. Conclusions Recurrence of KCOT s associated with NBCCS 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.