Melanotic Neuroectodermal Tumour of Infancy: Clinical Courses and Therapeutic Options-A Review of Three Cases.

BACKGROUND: Melanotic neuroectodermal tumour of infancy (MNTI) is a rare benign neoplasm. MNTI appears most often during the first year of life, arises predominantly in the maxilla and tends to recur. We discuss possible therapeutic options given in the literature and within our experience in three cases. PATIENTS: In our recent case, we used an intraoral approach to perform resection of the right-sided maxilla. Despite tumour-positive margins, there was no recurrence over the course of one year. In a previous case of MNTI, two recurrences occurred and 6 months after last resection patient received a rib graft for maxillary reconstruction. However, at the age of 7 years, the infant displayed severe maxillary hypoplasia. In a third case of MNTI, the patient was followed up after initial therapy for two decades and underwent multiple reconstruction procedures to achieve successful rehabilitation. CONCLUSION: Surgical treatment of MNTI should respect vital anatomic structures to avoid gross mutilation. The need for extended and repetitive tumour resection in early childhood can lead to growth disturbances and to further multiple reconstruction procedures in adulthood. Because of the rarity of MNTI, an international database is warranted to evaluate therapies and clinical courses over decades.

Total mandibular reconstruction following diffuse sclerosing osteomyelitis.

BACKGROUND: Diffuse sclerosing osteomyelitis (DSO) is a non-purulent chronic recurrent inflammation and affects the mandible in many cases. Belonging to the group of autoinflammatory diseases, in children and in cases with various additional symptoms including synovitis, acne, pustulosis, hyerostosis, and osteitis (SAPHO syndrome), therapy usually consists of non-surgical treatment. Against this background, we present an unusual course of DSO in an adult female patient. CASE REPORT: A 50-year-old female suffering from DSO without SAPHO syndrome was pretreated for years with conservative drug regimens and local surgery. Previous therapy was not successful, and subsequently, multiple surgical procedures were carried out focused on recurrent acute exacerbations of DSO. Surgery resulted in a total resection and alloplastic and autoplastic reconstruction of the mandible including both temporomandibular joints. Prosthetic rehabilitation was possible after dental implant loading, and the final outcome was very satisfactory. CONCLUSION: In the event that non-surgical options are not successful in DSO, an extended surgical therapy becomes necessary. Even if surgery results in complete resection of the mandible, a satisfactory rehabilitation can be achieved after complex reconstruction.

Continuity defects of the mandible: Comparison of three techniques for osseous reconstruction and their impact on implant loading.

Computer-aided design/manufacturing (CAD/CAM) is now widely used, but whether it can help to overcome complications in mandibular reconstruction and accelerate dental implantation is still a matter for debate. Therefore, we aimed to evaluate the benefits of this technique using vascularized iliac crest or fibula flaps in mandibular reconstruction, with respect to the time between reconstruction and implantation, and the ratio of planned to inserted implants. We reviewed retrospectively the records of 54 patients who underwent mandibular reconstructions between 2012 and 2016, and included in our study the last 10 cases representing each of the following groups: iliac crest flap with CAD/CAM (Group 1); fibula flap with CAD/CAM (Group 2); and fibula flap without CAD/CAM (Group 3). Groups 1 (p = 0.045) and 2 (p = 0.034) showed significantly shorter delays when compared with Group 3. Significant differences in average counts of implants placed were also found between Group 1 and Groups 2 (p = 0.04) and 3 (p = 0.019). The ratio of planned to placed implants was highest in Group 1. The observed differences between Group 1 and Groups 2 (p = 0.04) and 3 (p = 0.019) were significant. Our results indicate an accelerating effect of CAD/CAM on graft consolidation and dental rehabilitation.

Increased basic fibroblast growth factor release and proliferation in xenotransplanted squamous cell carcinoma after combined irradiation/anti-vascular endothelial growth factor treatment.

Novel strategies of cancer therapy combine irradiation and anti-angiogenic active compounds. However, little is known concerning the undesired cellular and molecular effects caused by this novel treatment concept. We used a mouse squamous cell carcinoma (SCC) xenotransplantation model to evaluate the potential undesired effects which compromise the success of this therapeutic combination. SCCs were subcutanously implanted in nude mice. Animals were treated with a fractionated irradiation scheme (5x4 Gy) alone or in combination with daily injections of anti-vascular endothelial growth factor (VEGF) antibodies. Controls remained untreated. Before and after treatment, resonance imaging (MRI), ultrasound and near-infrared spectrometry were used to evaluate tumor vessel integrity. Finally, tumors were explanted and VEGF, basic fibroblast growth factor (bFGF), vessel density, proliferation and apoptotic activity were analyzed by immunohistochemistry. Irradiation caused VEGF release and we found evidence for VEGF-mediated vessel protection. In the tumors derived from the combined treatment, blood volume was decreased, and apoptotic indices were increased. Remarkably, bFGF levels and proliferative indices were also increased. Combined irradiation/anti-VEGF treatment resulted in the desired VEGF depletion and increased tumor cell apoptosis. Nonetheless, bFGF and proliferation also increased, possibly suggesting a compensatory response. The application of additional targeted drugs may help develop more effective SCC treatments.