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Neurofibromatosis type 1 (NF1) is an autosomal dominant tumor predisposition syndrome that is also characterized by skeletal abnormalities. In the cranial region, skeletal dysplasia is observed that is associated with a characteristic peripheral nerve sheath tumor, the plexiform neurofibroma (PNF). The aim of the study was to determine PNF-associated skeletal asymmetries of the mid-skull and skull base as an indicator of local tumor development.The distances of the zygomatic arch, mastoid, and juga measurement points from the interorbital horizontal plane and median sagittal plane were examined on anterior-posterior cephalograms of 168 NF1 patients (females: 82, males: 86) and compared with the findings of a control group. A distinction was made as to whether the patients had developed a facial PNF.The distances of the measurement points from the reference planes differed between the patient and control group (p0.05). Within the NF1 patients, differences between certain distances were noted when comparing patients with PNF and without PNF (p0.05). In PNF patients, discrete changes in the skull contour were also detectable on the non-affected side (p0.05). The caudal measurement point of the skull base showed no changes in the group comparison.The individually very variable facial PNF in NF1 is associated with deformations and misalignments of midfacial bones that follow a pattern quantifiable with cephalometric analysis.

Purpose The purpose of the study was the investigation of supernumerary teeth (ST) in the permanent dentition of patients with neurofibromatosis type 1 (NF1). Material and methods The panoramic radiographs of 200 NF1 patients were analysed for ST. The potential impact of certain neurogenic facial tumours on the measured variable was investigated separately. The results were compared to dental findings in panoramic radiographs of 200 age- and sex-matched controls. Results The total number of ST in all individuals was 23 in 12 patients (3% of total population). However, all ST had developed in the group of NF1 patients (6%), (p = 0.0004). ST were particularly common distal to the molar region. Although ST occur in the region of facial plexiform neurofibroma, the numerical aberrations of the dentition are independent of specific type of facial nerve sheath tumour. Conclusion The unusual accumulation of ST in the terminal sections of molar development could be an indicator of preferred points of developmental disorder, in which the genetic predisposition of the patient is involved. Reviewing the results of other NF1 patient collectives is desirable.

BACKGROUND/AIM Neurofibromatosis type 1 (NF1) is a tumor predisposition syndrome. Bone findings make a significant contribution to the clinical diagnosis of NF1. It has been suggested that there are characteristic skeletal features of the NF1 patients' skull that cause a specific 'NF1 facies'. To test this thesis, skull examinations were carried out on NF1 patients. PATIENTS AND METHODS The posteroanterior (PA) cephalograms of 76 patients with NF1 were analyzed using defined measuring points. Patients with confirmed facial plexiform neurofibromas (PNF) were excluded from the study. A special interest of the investigation was the symmetry of the measuring points defined as the distance to the median sagittal plane. RESULTS NF1 patients have a slightly larger distance to the Z-plane than controls at the zygomatic arch and mastoid measurement points (p=0.027 and 0.028, respectively). In contrast, the distances of the juga and antegonion measurement points from the horizontal reference plane are larger in the control group (p=0.002 and 0.480, respectively). The transverse development of the midface at the level of the zygomatic arch showed no differences from the control group (p=0.841). The transverse diameter of the skull at the mastoid and juga measurement points is smaller in the NF1 group compared to the control group (p=0.010 and 0.002, respectively). There is a statistically significant left-right (LR) asymmetry of the distances to the median sagittal plane in favor of the left side in the patient group (p=0.002 to 0.037). However, the numerical deviations from the control group are small overall. CONCLUSION Considering the natural, biological deviations of cephalometric measurements of the individual from idealized geometric norms, the facial skeleton of NF1 patients is symmetrically developed. It is unlikely that the calculated LR asymmetry of the patients has a visible effect. In comparison to cephalometric values of a normal population, no characteristic facial skeleton of the NF1 patient in the PA projection of the skull can be derived from these findings. Clear asymmetries of the facial skeleton should give rise to further diagnosis to clarify the suspicion of facial PNF.

Background Radiological cephalometry is an important diagnostic tool for analyzing the shape and proportions of the skull. Standardized teleradiography of the skull in posterior-anterior (PA) projection provides orientation data on the symmetry and vertical relations of the skull. The comparison of individual findings with normal values places high demands on the selection of a control group. The aim of this study was to characterize a group to be used as a standard for cephalometric comparisons. Patients and methods PA teleradiographs of 23 healthy young adults were analyzed. Distances from reference measuring points to the median sagittal plane and the orbital horizontal plane were made. All individuals showed ideal occlusion. None of the participants had been subjected to orthodontic therapy or craniomaxillofacial surgery. Results The measurement results showed a high degree of lateral symmetry of the skeletal reference points and planes. Comparison of the vertical reference lines confirmed the symmetrical constitution of the facial skeleton. Conclusion The study group is suitable for comparison with the cephalometric evaluations of other study groups.

Background/aim Numerical aberrations of permanent dentition and dystopic tooth eruption are part of the phenotype of the tumor predisposition syndrome neurofibromatosis type 1 (NF1). In these cases, surplus tooth germs usually develop in the alveolar processes of the jaw. This report attests to the dystopic development of a dysplastic supernumerary tooth in NF1 arising outside the jaw. Case report The 8-year-old male patient developed a microdont outside the bone and above the occlusal plane of the retained maxillary right second molar. The supernumerary tooth was completely embedded in oral soft tissue. Hyperplastic oral soft tissue in the molar region and microdont were excised. Specimen of the mucosa surrounding the teeth was interspersed with diffuse and plexiform neurofibroma. The retained upper right first molar emerged spontaneously within a few months after surgery. The upper right second molar did not change position. Conclusion Odontogenesis can take place within tumorous oral mucosa in NF1. Surgical removal of the tumorous mucous membrane facilitates tooth eruption in some cases.

Neurofibromatosis type (NF1) is an autosomal dominant inherited tumor-suppressor gene syndrome of significant phenotypic variability with probable complete penetrance of the disease. Skeletal malformations of the skull belong to the phenotype of NF1. In the skull, defects of the calvaria and the sphenoid bone are diagnostically groundbreaking findings in NF1. Malformations of the facial skull are usually diagnosed in patients with NF1 in a topographical context with a plexiform neurofibroma (PNF). This report describes the rare occurrence of slowly advancing, unilateral destruction of proportions of the mandible in NF1, with the affected bone segment completely surrounded by a PNF. A malignant process was ruled out as a cause of partial organ loss. Various hypotheses on the pathogenesis of the rare finding are presented.

The aim of this study was to measure line segments and areas of sella turcica on lateral cephalograms with respect to the clinical diagnosis of facial phenotype of patients with neurofibromatosis type 1 (NF1). Special attention was given to correlate the measured values with certain tumour types that are typical for this disease. Material and methods: Lateral cephalograms of 194 individuals were investigated. Patients with NF1 were further divided according to the detection and topography of facial plexiform neurofibromas (PNF) taking into account the distribution pattern of the trigeminal nerve. All patients with PNF showed unilateral tumour localisation. Patients without any facial PNF constituted a separate group. Healthy volunteers with ideal occlusion and no history of any intervention in the maxillofacial region served as a control group. The following items were determined on the radiographs: sella entrance, sella width, sella depths, sella diagonal, and sella area. Results: Patients with PNF of the first and second trigeminal nerve branch or affected in all branches showed highly statistically significant enlarged sella tucica measurement values. On the other hand, patients with PNF restricted to one branch only or simultaneously in the second and third branches showed measurement values that were not different to those obtained in NF1 patients devoid of facial PNF. The latter group also showed no difference of sella turcica parameters obtained in the control group. Conclusion: This study provides evidence for the association of a certain NF1 phenotype with distinct skeletal alterations of the skull base, shown here using the example of the representation of the sella turcica in the lateral radiograph. These findings are also relevant in the discussion of NF1 as a disease of bones and in the assessment of brain development in NF1. Both items are discussed in relationship to a facial plexiform neurofibroma. Furthermore, the knowledge of this association of findings provides the clinician with relevant information in the planning of skull base procedures in these patients., Das Ziel dieser Untersuchungen war die Vermessung von Strecken und Flächen der Sella turcica auf seitlichen Fernröntgenbildern unter besonderer Berücksichtigung des fazialen Phänotyps von Patienten, die an einer Neurofibromatose Typ 1 (NF1) erkrankt waren. Besondere Aufmerksamkeit wurde der Korrelation von definierten Tumortypen, die für diese Erkrankung typisch sind, und den kephalometrischen Messwerten gewidmet. Material und Methode: Laterale Kephalogramme von 194 Individuen wurden untersucht. Patienten mit NF1 wurden weiter differenziert nach dem Nachweis und der Topographie fazialer plexiformer Neurofibrome (PNF), wobei das Verteilungsmuster des N. trigeminus im Gesicht als Orientierung diente. Alle Patienten mit PNF hatten eine einseitige Manifestation des Tumors. Patienten ohne faziales PNF stellten eine gesondert analysierte Gruppe dar. Gesunde Probanden mit Idealokklusion und ohne jede Vorgeschichte einer Intervention im kraniomaxillofazialen Bereich dienten als Kontrollgruppe. Die folgenden Messungen wurden durchgeführt: Sella-Eingang, Sella-Weite, Sella-Tiefe, Sella-Diagonale und Sella-Fläche. Ergebnisse: Patienten mit einem PNF des ersten und zweiten oder solche, die in allen drei Ästen des N. trigeminus von diesem Tumor betroffen sind, weisen statistisch signifikant vergrößerte Sella-Messwerte auf. Andererseits sind die Messwerte der Patienten, die nur in einem einzelnen Ast vom PNF betroffen sind oder deren Tumorausbreitung sich auf den 2. und 3. Ast des Nervs beschränkt, von denen der Patienten ohne ein faziales PNF statistisch nicht zu unterscheiden. Die Messwerte dieser beiden Gruppen unterscheiden sich ebenfalls nicht von denen der Kontrollgruppe. Schlussfolgerung: Diese Untersuchung beweist die Assoziation eines bestimmten Ausbreitungstyps des fazialen PNF bei NF1-Patienten mit merklichen skelettalen Veränderungen der Schädelbasis, hier dargestellt anhand der Form der Sella turcica im seitlichen Röntgenbild. Diese Befunde sind auch bedeutsam für die Diskussion der NF1 als Erkrankung des Skelettsystems und für die Einschätzung der Gehirnentwicklung bei NF1, beide in Zusammenhang mit dem Nachweis eines plexiformen Neurofibroms. Weiterhin liefern die Kenntnisse über die hier vorgestellten Befunde dem Kliniker relevante Informationen für die Planung von Schädelbasiseingriffen an Patienten mit dieser Diagnose., GMS Interdisciplinary Plastic and Reconstructive Surgery DGPW; 6:Doc05

Purpose Neurofibromatosis type 1 (NF1) is an autosomal dominant transmitted tumour suppressor syndrome and also a bone disease. Osseous dysplasia affecting the craniofacial region is characteristic of NF1. The aim of this study was to analyse the lateral cephalograms of NF1 patients in comparison to individuals who were not affected by this condition in order to describe the skeletal phenotype of NF1 in more detail. Materials and methods The study comprises the lateral cephalograms of 172 patients with established NF1 diagnoses (female = 85, male = 87). NF1 patients were distinguished by radiological and/or histological findings of the facial region suggestive of plexiform neurofibroma (PNF) or disseminated cutaneous NF (DNF). The analysed radiographs of a collection of 29 healthy volunteers with ideal occlusion served as controls. The focus of this analysis was cephalometrically defined angles. Results Cephalometric analyses of patients with DNF did not differ from those of controls for the vast majority of parameters. However, the measurement results of patients with PNF differed significantly from those of healthy volunteers and patients with DNF. The number of trigeminal nerve branches affected in PNF patients had an effect on the measurement results. Conclusion Lateral cephalograms revealed no significant alteration of the facial skeleton in NF1 patients as compared to controls. Indeed, the stigma of a so-called ‘NF1 facies' cannot be derived from the cephalometric findings presented. Notably, a wide range of deviating readings were recorded for individuals with facial PNF. Clinicians who treat patients with NF1 should be aware of deviations from cephalometric standards on lateral cephalograms in NF1 patients, especially when craniofacial surgical procedures are planned. Some of these findings, particularly asymmetries of the facial skeleton, could be indicators of an associated PNF.

Odontogenic myxoma (OM) is a rare tumour arising in the jaws. The tumour is purported to be odontogenic in origin due to the frequent localisation of the tumour inside the jaws in close relation to teeth. The aim of this report was to detail the course of a patient who developed OM of the maxilla, underwent adequate ablative surgery and reconstruction, including tooth transplantation to the original tumour site, and subsequently developed a local recurrence in close proximity to the teeth transplanted to the reconstructed maxilla 6 years after the first diagnosis. Once again, a partial maxillary resection was performed, with no reconstruction. The patient has been free from tumour recurrence for over 20 years. We discuss the current hypothesis on OM pathogenesis and the possible impact of actively dividing cells on tumour re-growth.

J Oral Pathol Med (2010) 39: 115–120 Background: Podoplanin expression was described in mouse tooth germ and apical bud cells. The aim of this study was to analyse the podoplanin expression of human tooth germ tissues, adult teeth and odontogenic lesions immunohistochemically. Study Design: Nine human tooth germ biopsies and seven healthy permanent teeth extracted for orthodontic reasons were examined. Anti-podoplanin (D2-40) reactivity was investigated immunohistochemically. Five well-defined cystic odontogenic lesions (10 radicular cysts, 10 follicular cysts, three keratocystic odontogenic tumours, five ameloblastomas, and two adenomatoid odontogenic tumours) were analysed simultaneously. Results: Podoplanin expression was detected in the majority of epithelial and ecto-mesenchymal cells of human tooth germ tissues, odontoblasts and superficial dental pulp fibroblasts of permanent teeth. Cystic odontogenic lesions revealed positive reactions predominantly at the invasion front edge within basal epithelial layers. Conclusion: Podoplanin appears to be involved in the orthologic and pathologic processes of the formation of elongated cell extensions and odontoblastic fibers, in the epithelial–mesenchymal transition and local invasion during tooth germ development as well as in both reactive and neoplastic odontogenic cystic lesions.

Aim: The aim of this study was to analyse jaw malformations and tooth displacement in patients with neurofibromatosis type 1 (NF1). Material and Methods: Forty-eight patients were included in the study (male or female 24 each). All fulfilled the current NIH diagnostic criteria for NF1. The age range was 2.5–66 years. The type of neurofibroma was histologically proven in surgically treated patients. Patients with disseminated cutaneous neurofibromata and those with the plexiform type were distinguished. The analysis was based on physical investigation, photographs, panoramic radiographs and dental casts. Results: With the emphasis on alterations of tooth position, deformities of the adjacent bones and malocclusion, the majority of these patients (26) were affected by plexiform neurofibromata. In the other 22 patients with disseminated neurofibromata, malformations of the alveolar ridge were absent and individual oral symptoms were rarely found and were mild, and in all cases were unimpaired. Numerical aberrations and retention of molars was exclusively associated with a trigeminal nerve affected by plexiform neurofibroma. Aplasia of a second lower molar was recognized in four of these plexiform-neurofibroma patients. Conclusion: It is widely accepted that malformations of the facial skeleton are often of genetic origin. However, in this study these malformations were strongly associated with plexiform neurofibromata originating from the trigeminal nerve. Thus, in addition to presently unknown genetic factors, the pattern of skeletal malformation can be caused by tumour invasion and local destruction, e.g. the neuromuscular unit or prenatal development of the plexiform neurofibroma in the inferior alveolar nerve. It is further concluded that epidemiologic studies on the incidence and severity of NF1 in the oral and maxillofacial region have to distinguish between patients with or without plexiform neurofibroma, especially when analysing alterations and deformities of the jaws, teeth and malocclusion. Aplasia of second inferior molars is an additional (dental) finding associated with plexiform neurofibromata in NF1.

The lateral periodontal cyst (LPC) is a harmless developmental aberration derived from odontogenic epithelia lying between the roots of vital teeth. The exact histogenesis of LPC is a matter of debate but therapy is usually only required to maintain stable periodontal conditions and in case of evidence for growth of the cyst. We report on a case of LPC where successful surgical therapy had initialized osseous restoration. We further discuss the value of histological findings to substantiate a clinical and radiological diagnosis.

The inflammatory paradental cyst (IPC) is a relatively rarely reported lesion arising from the lateral periodontium of vital teeth. However, IPC appear to be under-represented in registers of odontogenic cysts due to the misinterpretation of IPC as infected dentigerous cysts, in particular IPC originating from third molars. IPCs exhibit some temporospatial associations with tooth eruptions and occur almost exclusively in the mandible. The IPC of the first molar is predominantly diagnosed in children younger than 10 years. Bony bulging in the area of cyst formation may alert parents to seek medical advice. This case report details the characteristic clinical and radiological features of a first molar IPC arising in a child. The features of the presented cyst correspond well to the attributes qualifying for a so-called buccal bifurcation cyst. A conservative treatment regimen proved to be successful. Certain immunohistochemical markers are reported to further characterize this entirely benign lesion.

Podoplanin expression has been reported in oral squamous epithelium, myoepithelia of the salivary glands, and odontogenic lesions, and has been linked with inflammatory and neoplastic conditions. We hypothesized that inflamed respiratory mucosa of the maxillary sinus also express podoplanin, especially in cases with odontogenic sinusitis. We retrospectively investigated podoplanin expression in biopsies from maxillary sinus with inflammatory changes. Cases with chronic rhinosinusitis with polyp formation (n=5), chronic rhinosinusitis without polyps (n=5), chronic rhinosinusitis with eosinophilia (n=5), and odontogenic chronic rhinosinusitis (n=5) were investigated immunohistochemically using an established antibody for podoplanin (D2-40). Respiratory epithelium in chronic maxillary sinusitis with polyp formation did not exhibit enhanced podoplanin expression. However, D2-40 positivity was detected in the basal cells in all cases with chronic sinusitis associated with inflammatory infiltrations as well as in the parabasal epithelial layer in chronic sinusitis without polyp formation. We observed podoplanin expression in non-neoplastic maxillary sinus epithelium exhibiting inflammatory changes. We suggest that podoplanin is involved in the pathogenesis of chronic rhinosinusitis, particularly in the intraepithelial migration of inflammatory infiltrates.

Neurofibromatosis type 1 (NF1) is an autosomal dominant inherited disease. Some stigmata of NF1 occur in the orbital region. The aim of this study was to reveal whether alterations of the orbital rim visible on plain radiographs may indicate the presence of a plexiform neurofibroma (PNF), a tumour almost exclusively diagnosed in NF1.The plain orbital radiographs of 73 patients with NF1 (female: N=37, male: N=36) were investigated for alterations of the orbit. The group was further distinguished according to the presence of orbital PNF (N=53) and/or sphenoid wing dysplasia (N=30). Radiographs from patients with NF1 and with exclusion of PNF in the orbitofacial region were used for comparison (N=20). A special cephalometric analysis (Dental Vision™) was adapted to the demands of this study.Patients with NF1 not affected by an orbitofacial PNF exhibited symmetrical orbits. Unilateral increase in orbital height was associated with ipsilateral PNF. The width of orbits affected by a PNF was often slightly increased compared to the non-affected side. The determination of cephalometrically-defined angles disclosed an erection of the PNF-affected orbit compared to the medio-sagittal plane.Plain radiographs are often the first diagnostic measure used to determine skeletal alterations. This study shows that certain parameters of the orbital rim are useful indicators of a PNF in patients who are unilaterally affected by this lesion in the orbital or orbitotemporal region.

The differential diagnosis of osseous jaw lesions includes numerous entities. A specific mandibular bone defect known as Stafne's cavity or cyst presents wirh a characteristic cyst-like lesion on radiographs of the jaw. Although the differential diagnosis from other jaw lesions is mandatory, this lesion does not usually require surgical therapy. Current theories concerning the pathogenesis of Stafne's cavity prefer bone depression as a consequence of constant pressure arising from an adjacent salivary gland. We present a case of a large Stafne's cavity eroding the basal mandibular cortex that was found accidentally on an orthopantogram taken for diagnosis of dental diseases. The patient noted a slight pain on pressure during physical investigation. The lesion was completely occupied by fatty tissue, as shown during surgical exploration and as revealed by histological investigation of the specimen. The theory of parenchymal pressure as the cause of mandibular bone depression appears to be inapplicable in the present case. The pathogenesis of Stafne's cavity is still obscure. Differential diagnosis of mandibular lesions is essential in adequate treatment planning.

This report describes physical and imaging findings in a case of anterior lingual mandibular bone depression in a child. This entity is very rarely diagnosed and even more extremely rarely seen in children. We present some characteristic findings depicted on images provided by different sources and briefly address current hypotheses on its pathogenesis.

In the current case, a 31-year-old patient who presented with a painful unilateral malocclusion and an unclear mass in the region of the right temporo-mandibular joint (TMJ) is reported. The malocclusion had been noticed by the patient a few months earlier. Chewing on the right side had become severely impaired and painful. The patient had no history of trauma to the TMJ. Plain radiographs and computed-tomographic scans depicted an enlarged and deformed mandibular condyle. A condylectomy was performed. The histological investigation of the specimen revealed an osteochondroma. The tumour did not express insulin-like growth factor-1 receptor. Five years following the surgical intervention, there has been no local recurrence and dental occlusion was re-established, without further treatment.

Neurofibromatosis type 1 (NF1) is an autosomal dominant inherited tumour syndrome. NF1 is also a disorder of bone in terms of altered bone metabolism and bone dysplasia. Facial plexiform neurofibroma (PNF) is frequently associated with osseous alterations and may cause severe disfigurement. These PNFs regularly affect the oral cavity and teeth. PNF pose many problems in reconstructive and oral surgery. This study was undertaken to describe oral findings related to PNFs and to investigate the size and position of teeth in these tumours.Forty-eight patients with NF1 were investigated. Tumour type was histologically proven in all patients with PNF following aesthetic/functional orofacial surgery. Twenty-four patients were affected with a PNF and 24 had disseminated cutaneous neurofibroma (DCNF). In the PNF group, the side of the PNF was registered; PNF was unilateral in all cases. DCNF patients were 15 females and 9 males (mean age: 23.92±10.48 years, minimum: 6, maximum 45 years). Patients with PNF were 17 males and 7 females (mean age: 23.92±13.76 years, minimum 6, maximum 61 years). The right side was affected in 16 and the left in 8 PNF patients. All patients were orally investigated and dental casts were obtained. Distances of the alveolar processes and of the teeth were measured in transverse and sagittal planes. In order to evaluate the possible impact of the tumour localisation inside the trigeminal nerve, the topography of the affected facial areas was assigned to the trigeminal nerve branches. Radiographs were taken to asses the formation of dental roots, to reveal retained teeth and to estimate dysplastic areas of the jaws.The size of tooth crowns did not differ from published standards on tooth parameters of Caucasian individuals. The dental arches were symmetrically arranged in all patients of the DCNF group. However, the position of teeth showed some relevant differences in the PNF group. We observed spacing between teeth, probably due to interdental invasion of plexiform neurofibroma or interference of tumour tissue with the mesial drift of teeth. Maxilla and mandible showed characteristic alterations in the PNF group that were confined to the side and extension of a PNF.Oral inspection should be carried out in all patients with NF1. Unilateral alterations of the position of teeth and asymmetries of the jaws should alert the investigator to search for an ipsilateral PNF of the second or third trigeminal branch. The size and dimensions of tooth crowns appear not to be affected by the disease.

Keratocystic odontogenic tumour (KCOT) is a benign oral neoplasm of odontogenic origin. The majority of KCOT develop sporadically. The main differential diagnosis of KCOT is from other odontogenic cysts and ameloblastoma. In the rare nevoid basal cell carcinoma syndrome (NBCCS, synonym: Gorlin(-Goltz)-Syndrome), however, KCOTs are frequently detected and may be the initial sign of this clinical syndrome. Podoplanin is a mucin-type transmembrane protein found in podocytes of human kidneys homologous to T1α-2. The expression of podoplanin in some other non-endothelial tissues raised our interest in studying this antibody in tissues of odontogenic origin. Recently, we reported podoplanin expression in sporadic cases of KCOT. We intended to investigate the podoplanin expression in KCOTs associated with NBCCS.Archival paraffin embedded tissues from six KCOTs from patients with known NBCCCS were analyzed immunohistochemically with antibodies to podoplanin (D2-40) and p63.We observed a continuous linear immunoreactivity of basal epithelial cells for podoplanin in all cases. The staining intensity was strong and did not differ from that for KCOT in previously reported sporadic cases. Strong nuclear P63 expression was detected in basal cell layers and diminished in suprabasal layers.KCOTs exhibited enhanced podoplanin expression in a clinical setting of NBCCS. Although the biological functions of podoplanin have not yet been fully recognized, the overexpression of this protein is capable of promoting the formation of elongated cell extensions, and increasing adhesion and migration of inflammatory cells. Podoplanin expression in KCOT is possibly associated with slow invasion of the adjacent structures and the well-known frequent local tumour recurrences of this odontogenic tumour.

Odontogenic myxoma (OM) is a rare tumour arising in the jaws. The tumour is believed to be of odontogenic origin due to the close relation to teeth. The radiographic appearance of OM is not specific and the diagnosis is frequently unexpected following surgical removal of the lesion. The aim of this study was to analyse the radiographic appearance of OM on conventional radiographs. This type of radiographic diagnosis is typically used by dental practitioners.We studied panoramic radiographs and plain skull radiographs of 14 patients investigated over a period of 30 years (male: 3, female: 11; age at time of diagnosis: 8 to 45 years, ≤16 years: 3). The maxilla was affected in five and the mandible in nine patients. In each case, all tumour findings were restricted to one jaw.Tumour size varied considerably. The largest tumours were seen in the distal parts of the mandible and ramus. Displacement of teeth was a frequent finding (8 cases), but root resorption was rare (2 cases). Honeycomb appearance on plain radiographs was associated with the size of the lesion and restricted to mandibular involvement.The radiographic appearance of OM of the jaws varies considerably. Large lesions may exhibit characteristic radiological signs of a slowly growing lesion. However, discrete displacement of teeth associated with a small osteolytic zone of the alveolar process between two teeth can be an OM. Careful interpretation of conventional radiographs is a must in identifying early lesions.

The nasolabial cyst is a rare, usually unilateral lesion arising in the soft tissues adjacent to the alveolar process of the anterior maxilla, above the apices of frontal teeth and below the alar base. The typical clinical features of nasolabial cysts are: swelling between the upper lip and nasal aperture caused by a smooth and fluctuant, well defined space-occupying lesion, elevation of the nasal ala and obliteration of the nasolabial fold. This report describes some clinical, radiological and morphological findings in a nasolabial cyst. The cyst was lined up with bilayered epithelium showing scattered goblet cells. The immunohistochemical analysis revealed that the basaloid epithelial cells exhibited nuclear positive reactions for p63. The proliferative activity of the epithelial cells was low (5%). Reaction for podoplanin was only discretely positive in basal cells within the non-inflamed portions but was enhanced in areas with inflammatory changes of the cyst wall. Cytokeratin subtyping showed a distinct expression of intermediate filaments in the nasolabial cyst. Nasolabial cysts are developmental cysts that can be cured by adequate surgical techniques. The expression pattern of podoplanin in this entity points to an association of this protein expression with inflammatory reactions to the cyst.

A 29-year-old healthy patient was submitted for exploration of intraosseous, unicystic lesions of the mandibular angle distal to the wisdom tooth of both sides. Histological investigation of the enucleated cystic lesions revealed bilaterally developed lymphoepithelial cysts (LEC). Healing was uneventful and re-ossification of the bone complete. LEC are rarely diagnosed in oral tissues and usually located in the soft tissues. This report details the clinical, radiological and morphological findings of intraosseous LEC of the mandible.

A 6-year-old girl was referred to Eppendorf University Hospital because of a suspected malignant mandibular tumour. An osteolytic lesion was depicted on panoramic radiograph in the premolar region. The tumour was resected with covering skin and the defect was closed by primary intention. Histological investigation revealed fibromatosis. No local recurrence of infantile fibromatosis occurred during seven years of follow-up. The differential diagnosis of fibro-osseous lesion of the facial region is challenging. Especially in children, caution should be exercised in the treatment of benign lesions that resemble malignant conditions derived from connective tissue, with special reference to long-term follow-up.

A 3-year-old male patient was referred to the Maxillofacial Surgery Clinic due to a painless swelling of the right palatal region. Conventional radiographs revealed no alteration of the dentition and did not delineate a lesion in the region of interest. Cone-beam tomography depicted small radiopaque, extraosseous deposits inside the palatal space. Histological examination revealed a minute mixed epithelial-mesenchymal lesion of odontogenic origin. We made the diagnosis of a peripheral developing odontoma, taking into consideration the components and arrangements of structures of the lesion. Early intervention is advisable to prevent these odontogenic lesions from eventually deforming the jaw and displacing adjacent teeth. Cone-beam tomography was a valuable pre-operative diagnostic tool to assess the lesion as being composed in part of hard tissue.

Peripheral giant cell granuloma (GC) of the jaw is a tumour-like lesion, situated on the gingiva. The aim of this study was to: (a) better define the cellular compartments of the lesion and (b) compare the protease expression-profile in GC lesions of the jaws to GC lesions of other sites.This study comprised 54 GC lesions (jaws: 30, tendon sheaths: 22, salivary glands: 2). A microarray technique was applied to the study of osteoclast-specific or osteoclast-like features of different sites (CD68, CD51, RANK, M-CSF). Proteases were immunohistochemically identified [cathepsin K, L, S and matrix metalloproteinase 9 (MMP9)].The GC of all lesions were immunoreactive for CD68 and CD51. Factors indicating the differentiation and activation of osteoclasts were detected in all lesions (RANK, M-CSF, cathepsin K, MMP9). The expression profile of M-CSF in GC and stroma cells was of a medium grade in cases with no apparent destruction of bone, whereas RANK was expressed only weakly in mono- or multinuclear CD68-positive cells.The results of this study reveal an identical cellular composition for all lesions irrespective of site. GC of lesions at all sites contain the same osteolytic proteases and express cytokines that are effective in bone metabolism. The reason for the absence of osteolysis in some 'epulis' cases may be due to the topography of the lesion. Furthermore, the reduced number of binding sites, revealed by the low expression profile of RANK, may possibly be responsible for an absence of or only superficial osteolysis in these cases, despite evidence of M-CSF.

Adenomatoid odontogenic tumour (AOT) is a benign tumour of odontogenic origin. The differential diagnosis of AOT is crucial in terms of surgical management. The 23-year old male patient presented in this case study was referred to the maxillofacial surgery clinic due to the incidental radiological finding of a large osteolytic lesion of the anterior mandible with a retained permanent canine at the base of the bone. Ultrasound imaging revealed the anterior surface of the tooth inside the lesion, indicating extreme thinning of the maintained cortical bone and liquid filling of the cystic lesion. Surgery revealed a bone defect with a deformed, incomplete tooth inside a granulation-like soft tissue. The tooth was extracted and the soft tissues were excavated. Healing was uneventful. Radiographic and physical investigations during long-term follow-up demonstrated complete bone remodeling. Some areas of the tumour were alpha-smooth-muscle-actin positive, indicating a myoepithelial differentiation. Differential diagnosis of AOT to other odontogenic tumours, such as ameloblastoma, is crucial for therapy. Exact morphological diagnosis avoids extensive ablative surgery.

Neurofibromas are the hallmark of neurofibromatosis type 1 (NF1). Interestingly, generalised and localised interference or dysfunction of bone is also a key element of the NF1 phenotype. In the skull, NF1-associated orbital dysplasia often results in a severe disfigurement of affected individuals. However, the underlying pathology of orbital dysplasia is a complex phenomenon and up to now poorly understood. This study was performed to describe the orbit in 42 NF1 patients with large, disfiguring soft-tissue tumour of the orbital/eyelid region (plexiform neurofibroma (PNF)). A dysplastic orbit on the affected side was diagnosed in 80.9%. Orbital PNF extension to adjacent regions revealed a significant correlation of orbit and temporal region (0.33, p0.034), cheek and oral cavity (0.4, p0.011), oral cavity and nose (0.35, p0.026), and temporal region and cheek (0.46, p0.003). Alterations of the optic nerve and adjacent structures were identified on MRI or CT in 14 patients. On plain skull radiographs, only sphenoid wing dysplasia and ipsilateral orbital enlargement were significantly correlated (0.528, p0.01). This study reveals PNF as the main component of soft tissue affecting eyelids and orbit in those cases, which show a soft tissue mass in the affected orbital region. The oval-shaped orbital rim, typically seen on plain skull radiographs in sagittal projections, seems to be strongly associated with the (lateral and caudal) extension of a PNF and independent from sphenoid wing dysplasia. Several factors constitute the individual orbital dysplasia, including the growth of the invasive PNF.

The eruption times of permanent teeth and the sequence of tooth eruption were investigated in 1000 individuals aged 5 to 13 years (female: 469, male: 531). Wisdom teeth were excluded from the evaluation. All individuals were inhabitants of Latakia (Syria). The results were compared to dentition studies originating from neighbour countries and Germany, published in recent years. The teeth emerged symmetrically in each jaw. However, teeth erupted earlier in the mandible than in the maxilla, excluding the slightly earlier eruption of maxillary premolars and first molars. The sequence of emerging teeth in the maxilla of females is: first molar, central incisor, lateral incisor, first premolar, second premolar, canine, and second molar. The sequence of tooth eruption of the mandible of females differs from the maxillary sequence: first molar, central incisor, lateral incisor, first premolar, canine, second premolar, second molar. The sequence of emerging teeth in the maxilla of males is: first molar, central incisor, lateral incisor, first premolar, second premolar, canine, and second molar. The sequence of tooth eruption of the mandible of males differs from the maxillary sequence: first molar, central incisor, lateral incisor, first premolar, canine, second premolar, second molar. The results of this study show some differences compared to the dentition of children and adolescents of other countries, emphasizing the need for further dentition studies to be performed in defined regions and obtained by the same method. Concerning the demands of forensic odontology the age of Syrian children might be underestimated if German standards for age estimation are used.

This report describes the surgical therapy, clinical course and morphological characteristics of an adenomatoid odontogenic tumor that developed in the maxilla of a 16-year-old patient. The cystic tumor filled the maxillary sinus and was removed with the retained tooth. Healing was uneventful and no local recurrence was observed during a two-year period of follow-up control. The tumor showed immunoreactivity for certain types of cytokeratins, vimentin and p63, and was focally immunoreactive for alpha smooth muscle actin and epithelial membrane antigen.

The eruption times of permanent teeth and the sequence of tooth eruption were investigated in 1,856 individuals aged 4 to 24 years (female: 912, male: 944). Wisdom teeth were excluded from the evaluation. All individuals were inhabitants of the German state of Saarland. The results were compared to results of national and international studies on tooth eruption of the 20th century and studies published in recent years. The teeth emerged symmetrically in each jaw. However, teeth erupted earlier in the mandible than in the maxilla, excluding the slightly earlier eruption of maxillary premolars and first molars in males. The sequence of emerging teeth in the maxilla of females is: first molar, central incisor, lateral incisor, first premolar, canine, second premolar, and second molar. The sequence of tooth eruption of the mandible of females differs from the maxillary sequence: first molar, central incisor, lateral incisor, canine, first premolar, second premolar, second molar. The sequence of emerging teeth in the maxilla of males is: first molar, central incisor, lateral incisor, first premolar, second premolar, canine, and second molar. The sequence of tooth eruption of the mandible of males differs from the maxillary sequence: first molar, central incisor, lateral incisor, first premolar, canine, second premolar, second molar. The results of this study are in accordance with those from other current studies of German federal states, obtained by the same method.

Neurofibromatosis type 1 (NF1) is an autosomal dominant inherited disease affecting about 1:3000 humans. Neurofibromas are benign soft tissue tumors. Giant cell granuloma (GCG) is a benign tumor-like lesion that is preferentially located in the jaws. GCG can develop in NF1 patients. A 7-year-old female NF1 patient was successfully treated for a recurrent GCG of the right mandibular premolar region. The serum levels of calcium and phosphate, alkaline phosphatase and parathormone were within the normal range. Genetic analysis of the tumor sample (GCG) and blood using 7 microsatellite markers revealed LOH of the NF1 gene in both sources. Inactivation of the NF1 gene may thus contribute to the development of GCG.

The eruption times of permanent teeth and the sequence of tooth eruption were investigated in 952 individuals aged 4 to 24 years. Wisdom teeth were excluded from the evaluation. All individuals were inhabitants of Tehran, Iran. The results were compared to results of international studies on tooth eruption of the 20th century and studies published in recent years. The teeth emerged symmetrically in each jaw. However, teeth erupted earlier in the mandible than in the maxilla, excluding the earlier eruption of maxillary premolars. The sequence of emerging teeth in the maxilla is: first molar, central incisor, lateral incisor, first premolar, second premolar, canine, and second molar. The sequence of tooth eruption of the mandible differs from the maxillary sequence: first molar, central incisor, lateral incisor, canine, first premolar, second premolar, second molar. The sequence of all teeth is: lower first molar, lower central incisor, upper first molar, upper central incisor, lower lateral incisor, upper lateral incisor, upper first premolar, lower canine, lower first premolar, upper second premolar, upper canine, lower second premolar, lower second molar, and upper second molar. The results support current theories that the differences in tooth eruption times between humans of different ethnic origin are small.

The eruption times of permanent teeth and the sequence of tooth eruption were investigated in 1535 individuals aged 3 to 24 years. Wisdom teeth were excluded from the evaluation. All individuals were inhabitants of Paderborn, Westphalia, Germany. The results were compared to results of other German studies on tooth eruption of the 20th century. The teeth emerged symmetrically in each jaw. However, teeth erupted earlier in the mandible than in the maxilla. Teeth erupted earlier in females than in males. The sequence of emerging teeth in the maxilla is identical in both sexes: first molar, central incisor, lateral incisor, first premolar, and canine, second premolar, second molar. The sequence of tooth eruption of the mandible is identical in both sexes but differs from the maxillary sequence: first molar, central incisor, lateral incisor, canine, first premolar, second premolar, second molar. Since the fifties of the last century the acceleration of teeth emergence has not increased in German studies on this subject.

The human teeth are a valuable source of information concerning identification and age calculation in forensic sciences. Dental treatment often causes permanent alteration of the teeth, which is visible on radiographs. The correlation of prosthetic restorations, root canal fillings and periodontal bone loss with chronological age has not yet been used for determining the completed 18th year of life in teenagers and young adults, e. g. for legal and anthropological purposes. The aim of this study was to determine whether the evaluation of such findings on radiographs correlates with the chronological age in teenagers and young adults.The dental X-rays (orthopantomogram, OPG) of 1053 outpatients (age: 14 to 24 years) were avaluated. The values "prosthetically restored", "filled dental root", or "periodontal bone loss" were recorded for each tooth present. The data were then evaluated using statistical tools.The correlation between the number of prosthetically restored teeth and the chronological age is high. The positive predictive value of determining an age of at least 18 years proved to be very high. However, these findings are rarely present in this age group. The number of endodontically treated teeth correlated less exactly with age. On the other hand, the determination of periodontal bone loss gave reasonable positive predictive values for the threshold value "chronological age of 18 years or more".This study provides for the first time predictive values for the estimation whether a person is 18 years of age, based on the evaluation of teeth with prosthetic restorations, root canal fillings or periodontal bone loss on OPGs only. Whereas the number of patients with such findings is generally low in this age group, some of the positive findings can be used for age estimation as an adjunct to other sources of age calculation. The sole application of these criteria is not recommended, as sanitary conditions of the teeth depend on both social and individual circumstances. Therefore, the calculated values also reflect the culture of the sample studied. Finally, they are time-dependent and have to be compared to data from other populations. These results have some bearing on the field of forensic odontology.

The human teeth are a valuable source of information concerning identification and age calculation in forensic sciences. Dental treatment often causes permanent alteration of the teeth, which is visible on radiographs. The correlation of decayed, missing or filled teeth and chronological age has not yet been used for determining the accomplished 18th year of life in teenagers and young adults, e. g. for legal and anthropological purposes. The aim of this study was to determine whether the evaluation of these findings on radiographs correlates with the chronological age in teenagers and young adults.The dental X-rays (orthopantomograms, OPG) of 1053 outpatients (age: 14 to 24 years) were evaluated. The values "decayed", "missing", or "filled" were recorded for each tooth present. The data were then calculated using statistical tools.The correlation between the number of missing teeth and the chronological age is low. However, the positive predictive value of determining the age of 18 years for certain types of teeth proved to be very high, even up to 100 %. Further, high numbers of filled teeth gave reasonable positive predictive values for an age of 18 years. The number of decayed teeth did not correlate with age.This study provides for the first time predictive values for the estimation whether a person is 18 years of age, based on the evaluation of decayed, missing or filled teeth on OPGs only. Whereas the number of patients with such findings is generally low in this age group, some of the positive findings can be used for age estimation as an adjunct to other sources of age calculation. The sole application of these items is not recommended, as sanitary conditions of the teeth depend on both social and individual circumstances. Therefore, the calculated values also reflect the culture of the sample studied. Finally, they are time-dependent and have to be compared to data from other populations. These results have some bearing on the field of forensic odontology.

Dental findings are an important source of information regarding forensic-odontologic determination of identity and age. A main part of dental age assessment is the determination of the wisdom teeth' development. The aim of this study was to answer the question whether the development of wisdom teeth can give information for the task of individual age estimation. Thereby, the investigation could contribute to age estimation efforts that are carried out on persons with an unknown age and where it is of high importance to know as exactly as possible the age of a person, e.g. the age of 18 years or older, for determining the punishment for a criminal offence.The orthopantomograms of 1053 outpatients were evaluated. The patients were aged 14 to 24 years. The developmental stages of the wisdom teeth were determined according to Demirjian. The data were calculated using statistical tools, like descriptive and inferential statistics in order to provide predictive values for the age to be above or below 18 years.The correlation between the developmental stages of wisdom teeth and the chronological age was high in general. Some values allowed the prediction of an age above 18 years with a probability of more than 70% to 80%, in some instances even more than 90%. The most appropriate developmental stage to determine the age of 18 years was A 1/2 (completed root development, apex open). The positive predictive value of this finding was 94%.This study provides for the first time predictive values for the estimation whether or not a person is 18 years of age, based on the evaluation of developmental stages of wisdom teeth on orthopantomograms only. These results should be compared to data from other populations gained by the same method.

Dental findings are an important source of information regarding forensic-odontologic determination of identity and age. A main part of forensic-odontologic findings is the determination of the wisdom teeth' development. The aim of this study was to answer the question whether the topography of the wisdom teeth influences the development of the teeth. Thereby, the investigation could contribute to age estimation efforts that are carried out on persons with an unknown age and where it is of great importance to precisely determine a person's age, e. g. the age of 18 years or older.The orthopantomograms of 1053 outpatients were evaluated. The patients were aged 14 to 24 years. The developmental stages of the wisdom teeth were determined according to Demirjian. The retention types were classified according to Archer (maxilla) and Wolf and Haunfelder (mandible). The data were calculated using statistical tools, like descriptive and inferential statistics in order to provide predictive values for the age to be above or below 18 years.The correlation between the developmental stages of wisdom teeth and the chronological age was high in general. Some values allowed the prediction of an age above 18 years with a probability of more than 70 % to 80 %, in some instances even more than 90 %. The topography of the wisdom teeth did not influence the timing of the root development. The sexual dimorphism of tooth development was not present in the development of wisdom teeth.This study provides for the first time evidence for the assumption that radiographs of the teeth in adolescents and young adults can be used for the age estimation, irrespective of the topography of the wisdom tooth. These results should be compared to data from other populations gained by the same method. The conclusions of this study might be useful in the field of forensic odontology.

The analysis of panoramic radiographs of the jaws for forensic purposes is well established. The dental findings on the radiographs give valuable information concerning the identity and possible age. Panoramic radiographs also depict the zygomatic arches. Pneumatized spaces of the temporal bone's zygomatic arch process are asymptomatic variations, entitled "zygomatic air cell defect" (ZACD). Data on ZACD prevalence might support the current forensic-odontologic practice in the fields of identification and age assessment. The authors analyzed 6 studies subjected to evaluate ZACD on panoramic radiographs. The out-patients were subjected to systematic radiography prior to treatment planning in specialized dental or maxillofacial surgery clinics. The age of the 7870 patients varied between 6 and 97 years. ZACD was found in 169 patients (prevalence: 2.32%; female: 56.12%, male: 43.9%). Most ZACD were unilateral (70%). In these studies no ZACD occurred in children younger than 7 years of age. There was no statistically significant difference concerning ZACD prevalence in the sub-groups of children aged 9 to 13 vs. 14 to 17 years. This review demonstrates the prevalence of ZACD on orthopantomograms. The prevalence of this finding in the investigated children does not differ from the prevalences reported for adults, and is low in general. The presence of ZACD in persons might be valuable for the identification of humans or human remains and for age estimation in addition to other physical and dental findings.

This report is about a bite mark on the breast of a female corpse. The woman had been killed by ligature strangulation at the age of 76 years. The investigation of the dental casts of a suspected man showed some anomalies in the dentition and orthodontic variations. A comparison of the bite mark with these anomalies demonstrated the correspondence of several details. Consequently, the probability that the suspect was responsible for the bite mark was regarded as being very high. During the further investigations the suspect admitted the offence including the bite injury to the breast. The authors emphasize the importance of an exact photographic documentation as an indispensable prerequisite for an odontological comparison.

Aim: Unfavourable facial growth in patients with cleft lip, alveolus, and palate may occur during puberty. Usually this development is not predictable in a young patient. The aim of the present study was to find an individual growth prediction at an early age that would allow us to decide whether later orthognathic surgery should be included in the treatment plan. Material and Methods: Lateral cephalograms of 41 patients with unilateral clefts of lip, alveolus and palate (uCLAP) with observation intervals of 4 years were computerized, correlations between the variables of the first and second radiograph were calculated and regression equations were established. Results: The skeletal change of the intermaxillary relationship can be explained purely by the lack of midfacial growth. An individual prediction of the angle SNA over a period of 4 years is possible with a correlation coefficient of 0.95. Additional predictions for SNB, Holdaway angle and the index of anterior facial height proportions are demonstrated. Conclusion: The prediction procedure elaborated in this paper facilitates the decision at the age of 12 years already, whether or not orthodontic occlusal treatment has been successfully completed at an early age. The alternative is to limit orthodontic treatment to the simple alignment of the two dental arches independently of their intermaxillary relation, and then to correct the facial skeleton and the dental occlusion simultaneously by combining surgical and orthodontic treatment after the completion of growth.