bromochloroacetic-acid and Basal-Cell-Nevus-Syndrome

The odontogenic keratocyst is the third most common cyst of the jaws, after the follicular and radicular cyst. Keratocysts most commonly occur as single lesions in the jaw of otherwise healthy persons. Multiple odontogenic keratocysts are a well-recognized feature of the nevoid basal cell carcinoma syndrome. The mandible, especially the third molar region, the angle of the mandible and the ascending ramus are involved far more frequently than the maxilla. Clinically, the cysts often remain asymptomatic and there are two specific histological entities: the orthokeratinized and the parakeratinized odontogenic keratocyst. Different surgical treatment options like marsupialization, enucleation with curettage or peripheral ostectomy, and osseous resection (marginal or segmental) have been discussed in the literature with variable rates of recurrence. Besides a predilection for recurrence, the keratocysts, in contrast to other odontogenic cysts, show a more aggressive clinical behavior and demonstrate a high mitotic count and higher turnover rate of the epithelium. This led to the tentative suggestion that the keratocyst might be a benign cystic neoplasm rather than simply an odontogenic cyst.

Topics: Basal Cell Nevus Syndrome; Diagnosis, Differential; Humans; Jaw Diseases; Jaw Neoplasms; Keratins; Odontogenic Cysts; Odontogenic Tumors; Recurrence

Basal cell nevus syndrome, also known as Gorlin-Goltz syndrome, is an autosomal dominant inherited disorder which is characterised by the presence of multiple maxillary keratocysts and facial basal cell carcinomas, along with other less frequent clinical characteristics such us musculo-skeletal disturbances (costal and vertebrae malformations), characteristic facies, neurological (calcification of the cerebral falx, schizophrenia, learning difficulties), skin (cysts, lipomas, fibromas), sight, hormonal, etc. On occasions it can be associated with aggressive basal cell carcinomas and malignant neoplasias, for which early diagnosis and treatment is essential, as well as family detection and genetic counselling. Currently there are new lines of investigation based on biomolecular studies, which aim at identifying the molecules responsible for these cysts and thus allowing an early diagnosis of these patients. In its clinical management and follow up, the odonto-stomatologist, the maxillofacial surgeon and several other medical specialists are involved. In this paper a review of the literature, and six cases of patients affected by multi-systemic and varied clinical expression of basal cell nevus syndrome, are presented.

Topics: Adolescent; Adult; Basal Cell Nevus Syndrome; Facial Neoplasms; Female; Humans; Jaw Cysts; Keratins; Male; Skin Neoplasms

The last ten years has revealed some of the key players in the development and differentiation of the hair follicle and the epidermis in general. In this review, we discuss how our current understanding of these processes has been made possible by the elucidation of the molecular basis of human inherited diseases and mouse mutants which display defects in the hair and epidermis. For examples, the study of ectodermal dysplasias and the basal cell carcinoma predisposition disease Gorlin syndrome have allowed the determination of signalling hierarchies critical in the formation of the hair follicle. Epidermolytic diseases and hyperkeratoses have focussed attention on the importance of the programs of keratin expression, while ichthyoses provide insight in the final stage of epidermal development, cornification. Finally, the increasing range of diseases and mouse models exhibiting alopecias are revealing the critical pathways in control of the hair follicle cycle.

Topics: Alopecia; Animals; Basal Cell Nevus Syndrome; Cell Differentiation; Epidermis; Forecasting; Genetic Predisposition to Disease; Humans; Keratins; Keratosis; Mice; Mice, Transgenic; Models, Biological; Mutation; Skin; Skin Diseases; Skin Neoplasms

Topics: Acrodermatitis; Amino Acid Metabolism, Inborn Errors; Basal Cell Nevus Syndrome; Epidermolysis Bullosa; Hair Diseases; Humans; Ichthyosis; Keratins; Keratosis; Neurofibromatosis 1; Psoriasis; Refsum Disease; Skin; Skin Diseases; Skin Neoplasms; Tuberous Sclerosis; Tyrosine; Warts; Xeroderma Pigmentosum

Keratocystic odontogenic tumor (KCOT) is a benign tumor that arises sporadically or associated with nevoid basal cell carcinoma syndrome (NBCCS). Its locally aggressive behavior contrasts with its cystic histological appearance. To better understand the interaction between tumor cells and the stroma, the present study aimed to evaluate and compare the immunohistochemical expression of matrix metalloproteinases (MMP-1, -2, and -9), the cellular proliferation index (Ki-67), and the presence of myofibroblasts (MFs) in KCOTs.. Eleven cases of isolated KCOT (G1) and 12 cases of KCOT associated with NBCCS (G2) were selected for an immunohistochemical investigation of the proteins MMP-1, MMP-2, MMP-9, Ki-67, and α-smooth muscle actin (α-SMA) in MFs. A group of 6 pericoronal follicles (G3) was included as a normal odontogenic tissue control.. Significant differences between the G3 and G1/G2 groups regarding the expression of MMP-1, MMP-9 (in connective tissue), and Ki-67 were observed. In KCOT, there was a positive correlation between the Ki-67 antigen and MMP-1 and between MFs and MMP-1 in the parenchyma. No statistical differences were found between G1 and G2 groups.. MMP-1, MMP-9, and proliferative activity appear to play important roles in KCOT pathogenesis. The increased proliferative activity with KCOT was associated with elevated MMP-1 production in the parenchyma, which influenced the growth of the lesion in association with an increased number of MFs.

Topics: Actins; Basal Cell Nevus Syndrome; Cell Proliferation; Connective Tissue; Dental Sac; Epithelium; Humans; Immunohistochemistry; Keratins; Ki-67 Antigen; Matrix Metalloproteinase 1; Matrix Metalloproteinase 2; Matrix Metalloproteinase 9; Myofibroblasts; Odontogenic Tumors; Stromal Cells

To determine the epithelial expression of β-catenin and survivin in sporadic (primary, and recurrent) and nevoid basal cell carcinoma syndrome (NBCCS) keratocystic odontogenic tumour (KCOT) in order to assess activation of the β-catenin pathway and evidence of apoptotic inhibition, processes that may contribute to the known differences in their biological behaviour.. Sections from 40 cases of KCOT (19 sporadic/primary; 9 sporadic/recurrent and 12 NBCCS-associated) were immunohistochemically stained for β-catenin and survivin. The extent and intensity of immunoreactivity within the lining epithelium was assessed, using semi-quantitative scales, independently by two pathologists who were blinded to the clinical-pathological data. Data were analysed using Kruskal-Wallis test and, for pair-wise comparisons, Mann-Whitney test with Bonferroni correction.. All cystic epithelial linings stained for β-catenin and survivin but there were differences in the pattern and intensity of staining among KCOT types. Sporadic primary KCOT showed weaker staining for β-catenin (P=0.0003) and survivin (P<0.0048) that was restricted to the basal and para-basal layers only, compared to sporadic recurrent and NBCCS-associated KCOT, which showed expression throughout all epithelial layers. There were no differences in β-catenin expression among recurrent and NBCCS-associated KCOT, whereas the intensity of survivin staining was higher in NBCCS-KCOT (P=0.0003). Nuclear staining for β-catenin was found exclusively in recurrent (5/9 cases) and NBCCS-associated (4/12 cases) KCOT.. The data demonstrate β-catenin delocalization and survivin over-expression in recurrent sporadic and NBCCS-associated KCOT suggesting that these pathways related to apoptotic inhibition have a role in KCOT growth and recurrence.

Topics: Adolescent; Adult; Apoptosis; Basal Cell Nevus Syndrome; beta Catenin; Cell Cycle; Female; Gene Expression Regulation, Neoplastic; Humans; Immunohistochemistry; Inhibitor of Apoptosis Proteins; Keratins; Male; Middle Aged; Odontogenic Tumors; Recurrence; Survivin; Young Adult

Keratocystic odontogenic tumor (KOT) is a benign odontogenic neoplasm with locally aggressive behavior and high recurrence rates. It is associated with nevoid basal cell carcinoma syndrome which usually has a more rapid growth. The aim of the study is to report the experience of our service on diagnosis and treatment of KOT.. Twenty-five cases of KOT were diagnosed between the years of 1989 and 2006. Demographic data was collected as well as diagnose and treatment.. Fifty-six percent were female with a mean age of 33 years old. Seventy percent occurred in mandibula and all received surgical treatment, associate or not with adjuvant therapy, such as cryotherapy and Carnoy's solution. Recidive was observed in 48% of cases with a mean period of time of 18 months.. Our data analysis showed the importance of previous diagnosis before enucleation procedure and long-term follow-up for recurrence early detection. Recurrence incidence is more frequent on first year after diagnosis.. KOT is a benign tumor with local aggressive behavior and therefore its treatment must consider the high index of recidive. Reports of protocol treatment should raise new discussion to decrease recurrence rates.

Topics: Acetic Acid; Adolescent; Adult; Aged; Antineoplastic Agents; Basal Cell Nevus Syndrome; Child; Chloroform; Combined Modality Therapy; Cryotherapy; Ethanol; Female; Humans; Jaw Neoplasms; Keratins; Male; Middle Aged; Neoplasm Recurrence, Local; Odontogenic Cysts; Odontogenic Tumors; Retrospective Studies; Young Adult

Nevoid basal cell carcinoma syndrome (NBCCS) is a rare autosomal dominant disorder characterized by developmental abnormalities and a predisposition to cancers. Although multiple jaw tumors, such as keratocystic odontogenic tumors (KCOTs), are one of the most frequent complications in NBCCS, the molecular mechanism for how KCOTs develop in NBCCS is poorly understood. A 15-year-old girl with 2 jaw tumors was diagnosed as NBCCS according to the clinical criteria. The pathologic findings indicated that the 2 tumors were consistent with KCOTs. A PTCH1 mutation, c.1472delT, was detected in her peripheral blood as well as in the 2 tumors. Interestingly, an additional PTCH1 mutation, c.264_265insAATA, that was not present in the peripheral blood, was found in the maxillary tumor but not the mandibular tumor. The Ki-67 labeling index was significantly higher in the maxillary KCOT (17.7%) than in the mandibular KCOT (14.3%). These findings indicate distinct molecular mechanisms of tumorigenesis in these KCOTs.

Topics: Adolescent; Basal Cell Nevus Syndrome; Cyclin D1; DNA Mutational Analysis; Female; Humans; Immunohistochemistry; Keratins; Ki-67 Antigen; Mandibular Neoplasms; Maxillary Neoplasms; Odontogenic Tumors; Patched Receptors; Patched-1 Receptor; Receptors, Cell Surface

Keratocystic odontogenic tumor (KOT) is a benign cystic tumor that affects the jaw bones and may be associated with the nevoid basal cell carcinoma syndrome (NBCCS). Twenty-five cases diagnosed as KOT, including primary and recurrent tumors and those associated with NBCCS, were submitted to immunohistochemical study for analysis of cytokeratins (CKs) 7, 8, 10, 13, 14, 18 and 19. The results showed CK13 immunostained on the intermediate layers and upper cells. CK14 was expressed in all epithelial layers and in those areas where inflammation and subepithelial splits were present; this protein was preserved within the basal cells. CK 18 was expressed mainly in the basal layer, whereas CK19 was expressed mainly on the intermediate and superficial layers. The remaining CKs tested were not immuoreactive. The status of maturation of cytokeratin seems to be altered on KOTs, and this is not distinct when different tumors are compared.

Topics: Adult; Basal Cell Nevus Syndrome; Brazil; Female; Humans; Immunohistochemistry; Jaw Neoplasms; Keratins; Male; Middle Aged; Neoplasm Recurrence, Local; Odontogenic Cysts

The objective of this study was to analyze the expression of matrix metalloproteinases (MMPs) 1, 7, and 26 in odontogenic keratocysts (OKCs) associated with Gorlin syndrome (SOKCs) and nonsyndrome OKCs (NSOKCs).. Twenty-one SOKCs and 20 NSOKCs were evaluated for epithelial expression of MMP-1, MMP-7, and MMP-26 and for mesenchymal expression of MMP-1 by immunohistochemistry.. Strong epithelial positivity to MMP-1 was observed in 76% of SOKCs and in 15% of NSOKCs (P < .05). Strong mesenchymal immunoreactivity to MMP-1 was observed in 38% of SOKCs and in 20% of NSOKCs (P > .05). Epithelial immunoreactivity to MMP-7 was strongly positive in 67% of SOKCs and in 40% of NSOKCs (P > .05). For MMP-26, strong positivity was found in 62% of SOKCs, in contrast to 35% of NSOKCs (P > .05).. MMPs-1, -7 and -26 may play important roles in the biology of OKCs. Furthermore, the presence of these proteases at higher levels in SOKCs may help to explain increased OKC aggressiveness associated with nevoid basal cell carcinoma syndrome.

Topics: Basal Cell Nevus Syndrome; Female; Humans; Immunohistochemistry; Keratins; Male; Matrix Metalloproteinase 1; Matrix Metalloproteinase 7; Matrix Metalloproteinases, Secreted; Odontogenic Cysts; Syndrome

Keratocystic odontogenic tumors (KCOTs, previously known as odontogenic keratocysts) are aggressive jaw lesions that may occur in isolation or in association with nevoid basal cell carcinoma syndrome (NBCCS). Mutations in the PTCH1 (PTCH) gene are responsible for NBCCS and are related in tumors associated with this syndrome. Mutations in the SMO gene have been identified in basal cell carcinoma and in medulloblastoma, both of which are features of NBCCS. To clarify the role of PTCH1 and SMO in KCOTs, we undertook mutational analysis of PTCH1 and SMO in 20 sporadic and 10 NBCCS-associated KCOTs, and for SMO, 20 additional cases of KCOTs with known PTCH1 status were also included. Eleven novel (1 of which occurred twice) and 5 known PTCH1 mutations were identified. However, no pathogenic mutation was detected in SMO. Our findings suggest that mutations are rare in SMO, but frequent in PTCH1 in sporadic and NBCCS-associated KCOTs.. NBCCS, nevoid basal cell carcinoma syndrome; KCOTs, keratocystic odontogenic tumors; BCCs, basal cell carcinomas.

Topics: Adolescent; Adult; Aged; Basal Cell Nevus Syndrome; Child; DNA Mutational Analysis; Female; Humans; Jaw Neoplasms; Keratins; Male; Middle Aged; Mutation; Odontogenic Tumors; Patched Receptors; Patched-1 Receptor; Receptors, Cell Surface; Receptors, G-Protein-Coupled; Smoothened Receptor

Topics: Basal Cell Nevus Syndrome; Diagnosis, Differential; Epithelium; Humans; Keratins; Odontogenic Cysts; Recurrence

Topics: Adolescent; Adult; Aged; Basal Cell Nevus Syndrome; DNA-Binding Proteins; Female; Humans; Immunohistochemistry; Keratins; Male; Middle Aged; Odontogenic Cysts; Receptors, Opioid, delta; Recurrence; Trans-Activators; Transcription Factors; Tumor Suppressor Proteins

Odontogenic keratocysts are relatively common lesions that may occur in isolation or in association with nevoid basal cell carcinoma syndrome (or Gorlin syndrome). The PTCH gene has been reported to be associated with Gorlin syndrome. We investigated 10 cases of non-syndromic keratocysts and two other cases associated with Gorlin syndrome, looking for PTCH mutations. Four novel and 1 known PTCH mutations were identified in five individual patients. Of the 5 mutations identified, 2 were germ-line mutations (2619C>A; 1338_1339insGCG) in 2 cysts associated with Gorlin syndrome, and 3 were somatic mutations (3124_3129dupGTGTGC; 1361_1364delGTCT; 3913G>T) in 3 non-syndromic cysts. This report describes PTCH mutations in both non-syndromic and Gorlin-syndrome-related odontogenic keratocysts in Chinese patients, and suggests that defects of PTCH are associated with the pathogenesis of syndromic as well as a subset of non-syndromic keratocysts.

Topics: Adolescent; Adult; Asian People; Basal Cell Nevus Syndrome; China; DNA Mutational Analysis; Female; Humans; Keratins; Male; Middle Aged; Mutation; Odontogenic Cysts; Patched Receptors; Patched-1 Receptor; Polymorphism, Genetic; Receptors, Cell Surface

Although odontogenic keratocysts are common in clinical practice, the simultaneous occurrence of multiple cysts in both the maxilla and mandible of a patient is rare. We report a case of an otherwise healthy individual who developed 17 cysts over 15 years.

Topics: Adult; Basal Cell Nevus Syndrome; Diagnosis, Differential; Humans; Jaw Neoplasms; Keratins; Male; Mandibular Diseases; Maxillary Diseases; Odontogenic Cysts

Topics: Basal Cell Nevus Syndrome; Diagnosis, Differential; Epidermal Cyst; Humans; Keratins; Leg; Male; Middle Aged; Skin Diseases

This article describes a case of odontogenic keratocyst (OKC) in a 1 year and 7 month old girl who presented such a lesion mimicking an eruption cyst. To date, only one well-documented OKC occurring in a patient under 5 years old has been reported and it was thought to be associated with nevoid basal cell carcinoma syndrome (NBCCS). In our OKC case, the cyst was totally enucleated. No evidence of recurrence and NBCCS was found after a 4-year follow-up. The development of involving tooth in a growing child and the histogenesis of OKC are discussed in this article.

Topics: Basal Cell Nevus Syndrome; Diagnosis, Differential; Female; Humans; Infant; Keratins; Maxillary Diseases; Odontogenic Cysts

The Patched (PTC) gene is responsible for basal cell nevus syndrome (BCNS) accompanied by multiple odontogenic keratocysts (OKCs), and its product plays a role in the Sonic hedgehog (SHH) signaling pathway involving smoothened (SMO) and GLI-1. To clarify the role of SHH signaling in OKCs, the expression of SHH, PTC, SMO, and GLI-1 and mutations of PTC were examined in 18 sporadic, 4 BCNS-associated OKCs and 7 control gingivae. SHH, PTC, SMO, and GLI-1 were detected in all OKC and gingiva samples by reverse transcriptase-polymerase chain reaction (RT-PCR). Immunoreactivity for SHH and GLI-1 was markedly higher in epithelial components than in subepithelial cells, while immunoreactivity for PTC and SMO was similar in epithelial components and subepithelial cells in OKCs. The positive rate of PTC and SMO expression in subepithelial cells of OKCs was significantly higher than that in gingivae. The positive rate of GLI-1 expression in subepithelial cells of BCNS-associated OKCs was significantly higher than that in primary OKCs. These results suggest that the SHH signaling might be involved in the pathophysiologic nature of OKCs. While mutations of the PTC gene could not be detected in 4 BCNS-associated OKCs by direct DNA sequencing, 3 of 5 primary and 4 of 4 recurrent OKCs had several mutations of this gene. These results suggest that PTC mutations are probably related not only to BCNS-associated OKCs but also to sporadic OKCs.

Topics: Basal Cell Nevus Syndrome; DNA Mutational Analysis; Frameshift Mutation; Gene Expression; Gingiva; Hedgehog Proteins; Humans; Immunohistochemistry; Keratins; Membrane Proteins; Mutation, Missense; Odontogenic Cysts; Patched Receptors; Patched-1 Receptor; Point Mutation; Receptors, Cell Surface; Receptors, G-Protein-Coupled; Recurrence; Reverse Transcriptase Polymerase Chain Reaction; Signal Transduction; Smoothened Receptor; Trans-Activators; Transcription Factors; Zinc Finger Protein GLI1

This study attempted to identify differential cytokeratin expression in cystic jaw lesions using immunohistochemical staining.. The charts from selected patients treated between 1983 and 1994 for jaw cysts were evaluated. Twenty-four paraffinized specimens were selected randomly for investigation with 5 immunohistochemical stains. The 4 diagnostic categories included ameloblastoma, dentigerous cyst, odontogenic keratocyst (OKC), and recurrent odontogenic keratocyst in patients with nevoid basal cell carcinoma (NBCC) syndrome. The 5 immunohistochemical stains included antibodies to cytokeratins 13, 17, and 18; CAM 5.2; AE 1/3; and carcinoembryonic antigen (CEA).. Differential staining of OKCs from patients with and without NBCC syndrome was found only with the antibody to cytokeratin 17. Furthermore, staining of OKCs in syndromic patients appeared to be stronger and more uniform than in nonsyndromic patients.. These findings suggest that immunohistochemical staining for cytokeratin 17 may aid in the diagnosis of OKCs and may be used to further subdivide these lesions based on the presence or absence of NBCC syndrome.

Topics: Ameloblastoma; Antibodies, Monoclonal; Basal Cell Nevus Syndrome; Dentigerous Cyst; Diagnosis, Differential; Humans; Immunoenzyme Techniques; Jaw Neoplasms; Keratins; Odontogenic Cysts; Random Allocation; Sampling Studies; Staining and Labeling

The odontogenic keratocyst has been well documented and extensively studied. It is of particular interest because of its high recurrence rate and aggressive nature. The material for this study consisted of 70 cases of odontogenic keratocysts in predominantly ethnic Chinese patients who were treated from 1981 to 1996. The cases were retrospectively studied to compare characteristics of the lesion in this population with those in previous reports. Most of the patients in this series were 21 to 30 years of age. Association with an impacted mandibular third molar was found in more than 50% of the cases. The recurrence rate was 20% for 35 patients with a follow-up period of at least 5 years. The follow-up period for the whole series ranged from 1 to 16 years. Treatment was surgical enucleation with peripheral ostectomy. There were no significant differences in characteristics with respect to presentation and prognosis between this series and those described in previous publications.

Topics: Adolescent; Adult; Aged; Basal Cell Nevus Syndrome; Child; China; Female; Humans; Jaw Cysts; Jaw Neoplasms; Keratins; Malaysia; Male; Middle Aged; Odontogenic Cysts; Recurrence; Retrospective Studies; Sex Ratio; Singapore; Tooth, Impacted

Basal-cell naevus syndrome is characterized by multiple odontogenic keratocysts as well as skeletal, ophthalmologic and neurologic features. It is important that the dental practitioner be aware of this syndrome as it has important ramifications for the developing dentition. A case of Basal-cell naevus syndrome is presented along with a review of the literature regarding the management of this disorder. An argument for conservative surgical management of this syndrome is made.

Topics: Basal Cell Nevus Syndrome; Child; Female; Humans; Keratins; Odontogenic Cysts; Radiography, Panoramic

The aim of the present study was to investigate epithelial cell proliferation in the linings of odontogenic cysts, including three different subtypes of odontogenic keratocyst (OKC), namely simple (non-recurrent), recurrent and basal cell naevus syndrome (BCNS)-associated lesions. Ki67 immunoreactivity in OKC (simple, n = 10; recurrent, n = 8; syndrome, n = 9), dentigerous cysts (DC, n = 5), radicular cysts (RC, n = 5) and normal oral mucosa (n = 7) was studied using a biotin-streptavidin-peroxidase method on paraffin sections after microwave treatment. Ki67+ epithelial cells were counted manually and related to the length of basement membrane (BM) as determined by TV image analysis. Data were analysed by the Mann-Whitney U test. The number of Ki67+ cells in simple OKC linings (53.1 +/- 17.8 cells/mmBM) was similar to that in oral epithelium (42.5 +/- 12.7 cells/mmBM; P > 0.2). However, both contained significantly more Ki67+ cells than DC (3.9 +/- 1.3 cells/mmBM) and RC linings (6.7 +/- 4.8 cells/mmBM; P < 0.006). The epithelial distribution of Ki67+ cells differed between groups, with the percentage of positive cells in basal layers in OKC linings (7.0 +/- 2.1%) being significantly lower than that of oral epithelia (35.9 +/- 5.6%), DC (78.4 +/- 8.4%) and RC (80.6 +/- 7.7%) linings respectively (P < 0.003). Comparison of Ki67 expression within the OKC group revealed no significant difference between simple and recurrent lesions (44.0 +/- 13.8 cells/mmBM; P > 0.3). However, OKC associated with BCNS contained significantly higher numbers of Ki67+ cells (91.8 +/- 35.6 cells/mmBM; P < 0.01).(ABSTRACT TRUNCATED AT 250 WORDS)

Topics: Analysis of Variance; Basal Cell Nevus Syndrome; Biomarkers; Cell Cycle Proteins; Cell Division; Humans; Immunoenzyme Techniques; Keratins; Ki-67 Antigen; Microwaves; Mouth Neoplasms; Neoplasm Proteins; Nuclear Proteins; Odontogenic Cysts; Paraffin Embedding; Proliferating Cell Nuclear Antigen; Recurrence; S Phase; Statistics, Nonparametric

Multiple keratocysts of the jaws are habitual in the nevoid basal cell carcinoma syndrome (Gorlin and Goltz. Syndrome). The authors report 3 cases of multiple keratocysts in a 28 year-old woman and in two men respectively ages of 27 and 21 years. In the third case some anomalies in which calcification of the falx cerebri were noted and suggested the Gorlin's syndrome. The limits of this syndrome with multiple keratocysts of the jaws are discussed.

Topics: Adult; Basal Cell Nevus Syndrome; Calcinosis; Dura Mater; Female; Humans; Keratins; Male; Mandibular Diseases; Maxillary Diseases; Maxillary Sinus; Odontogenic Cysts; Paranasal Sinus Diseases

A survey is given of patients with odontogenic keratocysts in childhood and adolescence which were treated at the Clinic for Maxillofacial Surgery Tübingen in the last 10 years. Odontogenic keratocysts occurring in the growth period are treated in most cases by enucleation or partial marsupialization. To avoid damaging the nerve or dental germs, fixation with Carnoy's solution is not performed. In a second part an experimental study dealing with the effects of Carnoy's solution on peripheral nerves is presented. After incubating the sciatic nerve of rats with Carnoy's solution lumbar SEP, M-response and Hoffmann's reflex were recorded. It was possible to demonstrate the detrimental effect of Carnoy's solution on peripheral nerves. After incubation periods of more than 2 minutes almost complete functional loss was observed. There was a strong correlation between electrophysiological and histological findings.

Topics: Acetates; Acetic Acid; Adolescent; Adult; Animals; Basal Cell Nevus Syndrome; Child; Chloroform; Dentition, Mixed; Ethanol; Female; Humans; Jaw Neoplasms; Keratins; Male; Odontogenic Cysts; Peripheral Nerves; Rats; Rats, Inbred Strains; Tooth, Deciduous

Measurement of colony-forming ability following exposure to gamma-rays was performed on cultured skin keratinocytes and skin fibroblasts obtained from normal individuals, basal cell naevus syndrome patients (BCNS) and ataxia telangiectasia (A-T) patients. The most striking observation was the radiation resistance of 8/8 keratinocyte strains compared with fibroblasts whether from BCNS patients or normals. The single A-T keratinocyte cell strain showed the same radiosensitivity as A-T fibroblast cell strains. The differential survival of keratinocytes and fibroblasts was also observed following exposure to 254 nm UV light. The survival curves of SV40 immortalized keratinocytes and retinoblasts derived from normal individuals or BCNS patients showed large shoulder regions following exposure to gamma-rays or 254 nm UV light. An increased D37 rather than an increased D0 was therefore the feature of such curves. This contrasted with the SV40 immortalized A-T keratinocytes or fibroblasts which showed a minimal shoulder effect and an increased D0. No difference in survival was observed between BCNS and normal cells following exposure to either UV or gamma-rays.

Topics: Ataxia Telangiectasia; Basal Cell Nevus Syndrome; Carcinoma, Basal Cell; Cell Survival; Cobalt Radioisotopes; Epidermal Cells; Epidermis; Fibroblasts; Gamma Rays; Humans; Keratins; Skin; Ultraviolet Rays

164 odontogenic keratocysts from 60 patients with the basal cell naevus syndrome were compared with a similar number of single keratocysts matched for age and site. Significant differences between the two groups were found in the numbers of satellite cysts, solid islands of epithelial proliferation and odontogenic rests within the capsule, and in the numbers of mitotic figures in the epithelium lining the main cavity. An index of activity derived from these parameters suggests a greater growth potential in syndrome cysts; in addition, the patterns of association of the features support the theory that the odontogenic rests give rise to satellite cysts.

Topics: Adolescent; Adult; Aged; Basal Cell Nevus Syndrome; Carcinoma, Basal Cell; Cell Division; Child; Female; Humans; Keratins; Male; Mandibular Neoplasms; Maxillary Neoplasms; Middle Aged; Odontogenic Cysts

Familial odontogenic keratocysts are described in this report. The Case 1 patient, who has 3 sisters, developed odontogenic keratocysts. The 2 younger sisters (Cases 2 and 3) also had odontogenic keratocysts, although the elder sister did not have any odontogenic cysts. The father of the patients had a history of removal of a jaw cyst, and the mother was found later to have malignant ameloblastoma. Besides the odontogenic keratocysts, the Case 1 patient had basal cell nevus, prominent frontal process, and ocular hypertelorism; the Case 2 patient had prominent frontal process; the Case 3 patient had prominent frontal process, ocular hypertelorism, and squint. All 3 sisters are suspected of being patients with the basal cell nevus syndrome. The Japanese dental literature concerning the basal cell nevus syndrome is reviewed.

Topics: Adolescent; Basal Cell Nevus Syndrome; Carcinoma, Basal Cell; Child; Diagnosis, Differential; Female; Humans; Keratins; Odontogenic Cysts

Three hundred nineteen odontogenic keratocysts from 255 patients (167 males and 88 females, a 2:1 ratio) were histologically and clinically examined. The cysts sometimes occurred as multiple or recurrent lesions or in association with Gorlin's syndrome. The rate of recurrence for the total population was 27%. The 116 patients who were tentatively diagnosed as having keratocyst prior to surgery had a recurrence rate of 26%. There was no direct correlation between a large number of different histopathologic parameters and the propensity of a lesion to recur. Based on these data, a theory for the growth mechanism of these lesions is presented, and it is that the odontogenic keratocyst should be regarded as a benign cystic neoplasm and treated accordingly.

Topics: Adolescent; Adult; Aged; Basal Cell Nevus Syndrome; Child; Diagnosis, Differential; Epithelium; Female; Humans; Jaw Diseases; Keratins; Male; Middle Aged; Odontogenic Cysts; Recurrence