1/8. role of radical surgery for intracranial melanotic neuroectodermal tumor of infancy: case report.OBJECTIVE AND IMPORTANCE: Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, locally aggressive tumor that arises most commonly from the maxilla or mandible. Infrequently, it originates from the cranial vault, and recent reports have described a favorable outcome after radical surgery. Some lesions are particularly problematic, such as those located along the cranial midline or cranial base and those with significant intracranial extension. Currently, there is no effective adjuvant therapy for MNTI; radiation is precluded by the patients' young age, and chemotherapy trials have not demonstrated long-term efficacy. CLINICAL PRESENTATION: A 2-month-old infant boy presented with a firm, immobile subcutaneous mass behind the right ear. The mass had been present at birth and enlarged with time. INTERVENTION: Initial resective surgery down to the dura resulted in massive tumor recurrence within weeks. Successful management required repeat surgery including excision of the dura and dural venous sinuses. CONCLUSION: This patient's large MNTI of the cranial base was successfully managed by radical surgery. Although MNTI is a rapidly growing tumor that is locally highly invasive, radical surgery may be associated with a favorable outcome and offers the potential for long-term cure.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
2/8. Melanotic neuroectodermal tumor of infancy in the mandible: report of a case.A case of melanotic neuroectodermal tumor of infancy occurring in the mandible is described. The patient was a 1-month-old boy with a rapidly growing tumor of the mandible. Computed tomography showed 2 well-defined osteolytic lesions in the right mandible. Histopathologic diagnosis of a biopsy specimen was melanotic neuroectodermal tumor of infancy. The tumor was excised with removal of the surrounding bone, but 1(1/2) months later it recurred, and segmental mandibulectomy and reconstruction of the defect with a titanium miniplate was performed. Retrospectively, evidence of recurrence was noted on computed tomography taken on the tenth postoperative day. The recurrence was caused by incomplete removal of the tumor. Histopathologically, the tumor cells of the recurrent lesion were dispersed extensively in the bone marrow, and bone remodeling was active. The surgical procedure may have stimulated tumor cell proliferation and reactive bone formation. The patient was followed for 2 years with no evidence of recurrence or metastasis.- - - - - - - - - - ranking = 7keywords = mandible (Clic here for more details about this article) |
3/8. Melanotic neuroectodermal tumor of infancy involving the mandible: 7-year follow-up after hemimandibulectomy and costochondral graft reconstruction.Oral and maxillofacial tumors occur rarely in the pediatric population compared with the adult population. We report a case of a 6-months old female infant suffering from a melanotic neuroectodermal tumor of infancy involving the mandible. Tumor resection was performed using a submandibular approach; the mandibular defect was reconstructed primarily with autogenous costochondral grafts. During a 7-year follow-up period, there has been no tumor recurrence. The costochondral graft healed well; tracing of panoramic radiographs at 2, 3, and 6 years documented some vertical overgrowth and growth retardation in the transversal dimension. The authors conclude that the use of costochondral grafts despite its controversial role for mandibular reconstruction can be recommended in particular after continuity resections in newborn infants. However, long-term follow-up is necessary as well as secondary corrective surgery at early skeletal maturity.- - - - - - - - - - ranking = 5keywords = mandible (Clic here for more details about this article) |
4/8. Melanotic neuroectodermal tumor of infancy: case report.INTRODUCTION: Melanotic neuroectodermal tumor of infancy (MNTI) is a rare tumor, locally aggressive, usually originated from maxilla and mandible and rarely from the skull. A case of a 4 month-old child presenting a bulging lesion in the midline of the occipitoparietal region with progressive growth is reported. CASE REPORT: The neurologic examination had normal developmental milestones. Computerized tomography scan and magnetic resonance Image showed a highly enhancing tumor, dislocating anteriorly and inferiorly the superior sinuses. In order to prevent excessive bleeding, surgical resection was performed in three stages, with complete removal. CONCLUSION: Based on the absence of tumor recurrence, we believe in a favorable neurological prognosis and in a possible of cure, although the patient was not submitted to any adjuvant treatment.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
5/8. Long-term results after mandibular continuity resection in infancy: the role of autogenous rib grafts for mandibular restoration.Reconstruction of mandibular defects after tumor resection in infants is a particular challenge. Although autogenous rib grafts have no relevance nowadays for restoration of mandibular bone defects after ablative tumor surgery because of limited bone stock and the availability of other donor areas, they are a useful surgical alternative after tumor surgery in infants.We here report on a 6-month, 5-year, and 10-year follow-up of three children who were diagnosed with benign tumors of the mandible with osseous destruction at the ages of 4 months, 6 months, and 2 years, respectively. Histologic diagnoses were melanotic neuroectodermal tumor, hemangioendothelioma of the mandible, and ameloblastoma. After continuity resection of the mandible, latero-mandibular bone defects were restored using autogenous rib grafts. Both clinical and radiologic follow-up visits in all children were performed to assess growth of the facial skeleton as well as the mandibular growth.Cephalometric measurements on Panorex films and three-dimensional computed tomographic scans revealed slight vertical growth excess and transversal growth inhibition of the reconstructed mandible in comparison with the nonoperated side.Although further growth of rib grafts is difficult to predict and occlusal disharmony may occur because of physiologic maxillary growth and growth of the unaffected mandible, we think that autogenous rib grafts can be ideally used for restoration of mandibular continuity defects in newborns and young children. Clinical follow-up visits on a yearly basis and orthodontic controls are useful for early orthodontic treatment of growth deficits. Further corrective surgery with bone augmentation or osseous distraction is required after completion of growth of the facial skeleton.- - - - - - - - - - ranking = 5keywords = mandible (Clic here for more details about this article) |
6/8. Melanotic neuroectodermal tumor of infancy: systematic review of the literature and presentation of a case.Melanotic neuroectodermal tumor of infancy (MNTI) is a rare, distinctive neoplasm of early infancy with rapid expansile growth and a high rate of recurrences. Most commonly the lesion affects the maxilla of infants during the first year of life, but it may also occur in the mandible, skull, brain, epididymis, and other rare locations. The origin of the tumor is the neural crest. The expansive, destructive, and rapid growth of MNTI and its effects on the surrounding tissues are the most obvious clinical features. Microscopically, large polygonal epithelioid cells resembling melanocytes, with variable deposits of melanin, and smaller neuroblast-like round cells characterize MNTI. Malignant transformation may occur. Since the first description in 1918, only 215 cases were reported up to the last extensive review in 1992. The present review supplements another 140 published cases of MNTI up to 2004, including an original case report. Clinical features, treatment alternatives, and follow-up are discussed.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
7/8. Melanotic neuroectodermal tumour of infancy arising in the maxilla.Melanotic neuroectodermal tumour of infancy is an uncommon neoplasm that usually occurs in children aged one year or less. Difficulty in deciding the cellular origin of this tumour has led to numerous names, including congenital melanocarcinoma, melanotic epithelial odontoma, melanotic ameloblastoma, and retinal anlage tumour. Electron microscopy and histochemical studies, however, have now established the neural crest origin. The most frequent site of occurrence is the maxilla followed by the skull, the brain and the mandible. The genital organs are the most frequent extracranial site. We present two cases of melanotic neuroectodermal tumour of infancy arising in the maxilla.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |
8/8. Melanotic neuroectodermal tumor of infancy.A 6-month-old female presented with a swelling of the left superior maxillary bone. A radiograph revealed a radiolucent lesion in the left anterior maxilla, and CT, showed a solid mass. At surgery, the lesion was a cystic dental tumor of ectopic location which was treated by excision. The pathological diagnosis was melanotic neurorectodermal tumor. This is an uncommon benign tumor of the neural crest origin that occurs mainly in the maxilla (70% of cases) but can occur in other areas such as the skull and the mandible. CT reveals a hyperdense mass and MR shows a hypointense mass on T1-weighted images and an iso-intense mass on T2-weighted images. The evolution is usually benign after surgical removal.- - - - - - - - - - ranking = 1keywords = mandible (Clic here for more details about this article) |