Neven Johannus, Hulsbergen-van der Kaa Christine, Groot-Loonen Jacqueline, de Wilde Peter C M, Merkx Matthais A W
Oral and Maxillofacial Surgeon, Department of Oral and Maxillofacial Surgery, Radboud University Nijmegen Medical Centre, Nijmegen, The Netherlands.
Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2008 Oct;106(4):493-6. doi: 10.1016/j.tripleo.2008.02.001. Epub 2008 Jul 7.
The case of a 4-month-old male infant treated with combined surgery and chemotherapy for an aggressive recurrent melanotic neuroectodermal tumor of infancy (MNTI) on the top of the alveolar process of the mandible with a long-term follow-up is presented. Initial treatment comprised conservative local excision and curettage of the mandible. After several local recurrences and because radical surgical excision would give gross functional and aesthetic mutilation, finally complete, long-lasting remission was achieved with adjuvant chemotherapy, according to a neuroblastoma protocol (10-year follow-up). The reason for this protocol was because molecular genetic studies of this tumor showed loss of heterozygosity of chromosome 1p and gain of chromosome 7q analogue to neuroblastomas. A combination of surgery and chemotherapy should be the preferred treatment in case of a recurrence MNTI because optimal functional and aesthetic outcome.
本文介绍了一名4个月大的男婴，其下颌牙槽突顶部患有侵袭性复发性婴儿黑素性神经外胚层肿瘤（MNTI），接受了手术和化疗联合治疗，并进行了长期随访。初始治疗包括下颌骨的保守局部切除和刮除术。经过几次局部复发，由于根治性手术切除会导致严重的功能和美观损害，最终根据神经母细胞瘤方案进行辅助化疗，实现了完全、持久的缓解（10年随访）。采用该方案的原因是，对该肿瘤的分子遗传学研究显示，1号染色体杂合性缺失和7号染色体获得，类似于神经母细胞瘤。对于复发性MNTI，手术和化疗联合应是首选治疗方法，因为能获得最佳的功能和美观效果。
