Metastasis of a Solitary Fibrous Tumor in the Mandible: A Case Report.

BACKGROUND/AIM: The solitary fibrous tumor (SFT) is a mesenchymal neoplasm and belongs to the group of soft tissue sarcomas. The SFT is characterized by indolent, slowly progressive growth and manifests itself clinically by compression of neighboring structures. The treatment of choice is surgical removal of the tumor. In advanced stages, there is also the possibility of chemotherapy, systemic therapy, or immunotherapy, as well as radiotherapy. Depending on their location and severity, SFTs show different recurrence rates and survival functions. CASE REPORT: The present case report shows an extremely rare localization of a low-risk SFT in the floor of the mouth. Despite complete surgical removal of the SFT, the patient showed a metastasis of the SFT in the mandible two years postoperatively. Therefore, this case report shows that even a low-risk SFT in the localized stage can metastasize despite of total surgical removal. Consequently, SFTs of the head and neck region require close clinical and imaging follow-up. CONCLUSION: Although the localization of the SFT in the oral cavity is a rarity, this entity should be included in the differential diagnosis in the case of long-term space-occupying processes in the head and neck region. This report is the first regarding metastasis of a SFT to the mandible.

Recurrent Swelling and Microfilaremia Caused by Dirofilaria repens Infection after Travel to India.

Human subcutaneous dirofilariasis is an emerging mosquitoborne zoonosis. A traveler returning to Germany from India experienced Dirofilaria infection with concomitant microfilaremia. Molecular analysis indicated Dirofilaria repens nematodes of an Asian genotype. Microfilaremia showed no clear periodicity. Presence of Wolbachia endosymbionts enabled successful treatment with doxycycline.

Analysis of orbital plain radiographs for orbital deformities in neurofibromatosis type 1 patients, with special reference to alterations of the orbital rim as indicators of adjacent plexiform neurofibroma.

UNLABELLED: 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. MATERIAL AND METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Size of tooth crowns and position of teeth concerning the extension of facial plexiform neurofibroma in patients with neurofibromatosis type 1.

UNLABELLED: 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. MATERIALS AND METHODS: 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. RESULTS: 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. CONCLUSION: 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.

Dysplasia of the orbit and adjacent bone associated with plexiform neurofibroma and ocular disease in 42 NF-1 patients.

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, p<0.034), cheek and oral cavity (0.4, p>0.011), oral cavity and nose (0.35, p<0.026), and temporal region and cheek (0.46, p<0.003). 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, p<0.01). 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.

Pleomorphic adenoma of the salivary glands: analysis of 94 patients.

UNLABELLED: Pleomorphic adenoma is a benign epithelial tumor of adenoid structure preferentially arising from the parotid gland. It was shown that complete tumor excision is a curative measure and recurrence is likely in incompletely excised tumors. The aim of this study was to analyse the outcome of patients with pleomorphic adenoma from salivary glands in order to evaluate the surgical strategy of a single institution. MATERIALS AND METHODS: The files of 94 patients were evaluated. Special attention was given to the development of malignancy in pleomorphic adenoma. RESULTS: The pleomorphic adenoma preferentially originated in the parotid gland (n=73; right 48, left: 25), and rarely in other glands. The tumor occurred more often in females than in males (45:28, parotid). In 73 patients a slowly growing swelling of the parotid was first noted, which was located at different sites within the gland. At least one recurrence was noted in 18 patients, the majority of them aged 50 to 70 years (n=12). Malignant transformation to carcinoma was found in 8.5%. CONCLUSION: Pleomorphic adenoma from salivary glands is a benign tumor. However, in a noteworthy number of patients, inside the benign tumor a phenotype develops with distinct properties of malignancy. Surgery with safety margins is the therapy of choice. Multinodular tumors are prone to recurrent disease.

Jaw malformations plus displacement and numerical aberrations of teeth in neurofibromatosis type 1: a descriptive analysis of 48 patients based on panoramic radiographs and oral findings.

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.