Epidemiology, prognostic factors, and management of malignant odontogenic tumors: an analysis of 295 cases.

OBJECTIVE: To determine the demographic characteristics, prognostic factors, and management for patients diagnosed with a malignant odontogenic tumor (MOT). STUDY DESIGN: The Surveillance, Epidemiology, and End Results (SEER) registry was reviewed for patients diagnosed with MOT from 1973 to 2011. Kaplan-Meier and multivariate Cox regression analyses were performed on patient demographic characteristics and pathologic variables. RESULTS: The SEER database identified 295 MOT patients. The mean age at diagnosis was 50.5 years (range 5-89 years). Of these patients, 61.7% were male and 38.3% were female. The racial composition was 66.4% White, 22% Black, 6.1% Asian, 3.1% Pacific Islander, 0.3% Native American, and 2.1% Other/Unknown. Kaplan-Meier analysis found an overall survival (OS) and disease-specific survival (DSS) at 5 years of 54% and 67%, respectively. Multivariate analysis of the entire cohort found that age and stage were predictors of OS and that age was a predictor for DSS. For stage I/II MOTs, age and surgical therapy were predictors of OS and DSS, respectively. CONCLUSIONS: Here we report the largest study to date investigating demographic characteristics, prognostic factors, and management of MOT patients. Determinants of survival for OS and DSS include age, stage, and surgical therapy.

A prospective epidemiological study on odontogenic tumours in a black African population, with emphasis on the relative frequency of ameloblastoma.

The persistent view in the literature is that the relative frequency of ameloblastomas is higher in the black population than in Caucasians. The aim of this study was to determine the relative frequency of all odontogenic tumours (OT) in a 100% black population and to compare our findings with those of previous studies. A prospective study was undertaken of all patients presenting with OT to all 16 Nigerian departments of oral and maxillofacial surgery over a 4-year period. The following data were obtained: patient demographics, delay to presentation, extent of the lesion, and histological diagnosis. Six hundred and twenty-two cases were studied. A slight male preponderance was observed (male to female ratio 1.17:1). Patients ranged in age from 5 to 89 years, with a peak incidence in the third decade. The relative frequency of OT was 0.99 per million and that of ameloblastoma was 0.76 per million. Ameloblastoma was the most prevalent OT (76.5%), followed by adenomatoid odontogenic tumours (5.6%), odontogenic myxoma (4.5%), and keratocystic odontogenic tumours (KCOT) (3.1%). The relative frequency of ameloblastoma among Nigerians was not different from frequencies reported previously among Caucasian and Tanzanian black populations. KCOTs were, however, rarely diagnosed in Nigerians as compared to the white population in the Western world.

PTCH1 gene mutations in Keratocystic odontogenic tumors: a study of 43 Chinese patients and a systematic review.

BACKGROUND: The keratocystic odontogenic tumor (KCOT) is a locally aggressive cystic jaw lesion that occurs sporadically or in association with nevoid basal cell carcinoma syndrome (NBCCS). PTCH1, the gene responsible for NBCCS, may play an important role in sporadic KCOTs. In this study, we analyzed and compared the distribution pattern of PTCH1 mutations in patients with sporadic and NBCCS-associated KCOTs. METHODS: We detected PTCH1 mutations in 14 patients with NBCCS-associated KCOTs and 29 patients with sporadic KCOTs by direct sequencing. In addition, five electronic databases were searched for studies detecting PTCH1 mutations in individuals with NBCCS-associated or sporadic KCOTs, published between January 1996 and June 2013 in English language. RESULTS: We identified 15 mutations in 11 cases with NBCCS-associated KCOTs and 19 mutations in 13 cases with sporadic KCOTs. In addition, a total of 204 PTCH1 mutations (187 mutations from 210 cases with NBCCS-associated and 17 mutations from 57 cases with sporadic KCOTs) were compiled from 78 published papers. CONCLUSIONS: Our study indicates that mutations in transmembrane 2 (TM2) are closely related to the development of sporadic KCOTs. Moreover, for the early diagnosis of NBCCS, a genetic analysis of the PTCH1 gene should be included in the new diagnostic criteria.

Keratocystic odontogenic tumour: systematic review.

OBJECTIVES: The aim of this review is to evaluate the principal clinical and conventional radiographic features of non-syndromic keratocystic odontogenic tumour (KCOT) by systematic review (SR), and to compare the frequencies between four global groups. METHODS: The databases searched were the PubMed interface of Medline and LILACS. Only those reports of KCOTs that occurred in a series of consecutive cases, in the reporting authors' caseload, were considered. RESULTS: 51 reports, of 49 series of cases, were included in the SR. 11 SR-included series were in languages other than English. KCOTs affected males more frequently and were three times more prevalent in the mandible. Although the mean age at first presentation was 37 years, the largest proportion of cases first presented in the third decade. The main symptom was swelling. Over a third were found incidentally. Nearly two-thirds displayed buccolingual expansion. Over a quarter of cases recurred. Only a quarter of all SR-included reported series of cases included details of at least one radiological feature. The East Asian global group presented significantly as well-defined, even corticated, multilocular radiolucencies with buccolingual expansion. The KCOTs affecting the Western global group significantly displayed an association with unerupted teeth. CONCLUSIONS: Long-term follow-up of large series that would have revealed detailed radiographic description and long-term outcomes of non-syndromic KCOT was lacking.

Adenomatoid odontogenic tumour: review of the literature and an analysis of 33 cases from South Africa.

The adenomatoid odontogenic tumour (AOT) is a benign lesion of odontogenic origin. It is a slow growing tumour that results in a painless expansion of the jaws. This is a retrospective review of the demographic, clinical and radiographic features of AOTs diagnosed in a black South African population over 20 years. Of the 746 odontogenic tumours diagnosed, 4% were AOTs. The patients' ages ranged from 9 to 37 years with a mean age of 15 years. The highest incidence was in the second decade of life (85%). The female to male ratio was 5.6:1. The maxilla was more commonly affected than the mandible in a ratio of 1.5:1. The sizes of the lesions ranged from 2 to 7cm, with 60% involving an entire quadrant. All were of the central follicular type and appeared as well-demarcated radiolucent lesions. The canine was the most common impacted tooth. The treatment of choice was enucleation of the lesion, with no recurrences being reported.

Distribution pattern of the adenomatoid odontogenic tumor: a retrospective study from the files of a government teaching hospital, Tamil Nadu, Chennai, India.

AIM: To determine the distribution and clinicopathologic characteristics of adenomatoid odontogenic tumors from Tamil Nadu, India, to provide meaningful data for comparison among different population groups, mainly in the Asian context. METHODS: Forty-three adenomatoid odontogenic tumors were reviewed from 493 odontogenic tumors recorded between 1970 and 2008. The data pertaining to age, sex, anatomic site, associated impacted teeth and radiographic details, provisional diagnoses, and macroscopic and microscopic details of the 43 odontogenic tumors were reviewed. RESULTS: The relative frequency of adenomatoid tumors comprised 8.72% of the total odontogenic tumors. The age range was 9-30 years, with a mean age of 16.88 years for combined adenomatoid odontogenic tumors. The mean age for other variants of adenomatoid odontogenic tumors assessed in this study differed slightly between sexes. Forty occurred within the bone and three peripherally. In general, the maxilla was the predominant site of involvement (72.5%), and the anterior region of jaw was affected in 92.5%. For combined adenomatoid odontogenic tumors, the male-to-female ratio was 1:1.26, and the maxilla-to-mandible ratio was 2.9:1. Microscopically, cystic lesions comprised 37.5% and occurred more often in males with a lower mean age. CONCLUSION: The present study shows variations in the distribution of adenomatoid odontogenic tumors within the population assessed, but no definite conclusion emerged with regard to regional or ethnic effect.

The adenomatoid odontogenic tumor: an analysis of 57 cases in a black African population.

PURPOSE: This study analyzed the findings in a large series of adenomatoid odontogenic tumors (AOT) in Nigerians. PATIENTS AND METHODS: Hospital records of all cases of AOT diagnosed at three teaching hospitals were reviewed and analyzed. RESULTS: Most of the tumors were intraosseous (central) (98.3%) and of the follicular type (75%). Females were more frequently affected than males (1.4:1), and patients in their second decade of life were most frequently affected (75%). Patients with follicular AOT were relatively younger (15.2 +/- 5.6 years) than those with extrafollicular tumors (20.9 +/- 13.8 years). However, males who presented with extrafollicular tumors (14.6 +/- 3.9 years) were relatively younger than their female counterparts (24.9 +/- 16 years). The maxilla was affected nearly twice as often as the mandible (1.8:1), and the canine tooth was frequently embedded in the tumor (76.9%). Although follicular tumors were most frequently located in the maxilla (76.3%), extrafollicular tumors were more commonly found in the mandible (69.2%). CONCLUSION: The distribution of this tumor in black Africans does not appear to be substantially different from that reported in caucasians.

Pigmented lateral periodontal cyst and other pigmented odontogenic lesions.

OBJECTIVE: To report a case of lateral periodontal cyst (LPC) with marked melanin pigmentation in a 38-year-old Black male and to discuss the phenomenon of melanin pigmentation in odontogenic cysts and tumors. RESULTS: Histologically, the epithelial lining of the LPC contained an abundant amount of melanin granules throughout the entire epithelium. Ultrastructurally, epithelial cells contained mature melanosomes (stage IV melanosomes). Melanophages containing aggregates of melanosomes were identified in the connective tissue cyst wall. Perusal of the literature revealed that melanin pigmentation in odontogenic lesions is uncommon. Melanin has been reported in calcifying odontogenic cyst (18 cases), odontogenic keratocyst (8 cases), adenomatoid odontogenic tumor (3 cases), ameloblastic fibroma (3 cases), odontoma (2 cases), and ameloblastic fibro-odontoma, odonto-ameloblastoma, and odontogenic fibroma (1 case each). CONCLUSIONS: Almost all pigmented odontogenic lesions; occurred in Blacks and Asians; they are almost non-existent in Whites. Thus, racial pigmentation probably plays an important role in such lesions.