Mandibular Melanotic Neuroectodermal Tumor of Infancy: Interdisciplinary Treatment from 2 Months to 19 Years of Age

婴儿下颌黑色素性神经外胚层肿瘤:2个月至19岁患者的多学科治疗

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Abstract

Melanotic neuroectodermal tumor of infancy (MNTI) is a clinically distinctive, benign neoplasm of neural crest origin. The tumor develops usually in the anterior maxilla and rarely in the skull and mandible. This is a report of the interdisciplinary treatment of a rare case of MNTI occurring in the mandible. The patient was initially addressed for examination at the age of 2 months with a rapidly growing tumor of the mandible that had increased double in size in a week. A well-defined lesion in the left mandible shown on MRI and high urine vanillylmandelic acid (VMA) level determined the diagnosis. The complete interdisciplinary treatment included four interventions. Surgery with enucleation and curettage, performed as first intervention, at the age of 2 months. The second intervention lasted from age 7 to age 15 and included a first phase of Orthodontic treatment to monitor normal growth, followed by interceptive Orthodontic treatment and Prosthodontic intervention with interim dentures. The third intervention accomplished after competition of growth and included the pre-prosthetic surgery with an augmentation of the height of the edentulous atrophic mandible and placement of 4 implants. In the fourth intervention the permanent prosthodontic restoration supported by implants was applied.

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