Department of Neurosurgery, Sri Sathya Sai Institute of Higher Medical Sciences, EPIP Area, Whitefield, Bangalore, Karnataka 560066, India.
J Craniomaxillofac Surg. 2012 Sep;40(6):e170-3. doi: 10.1016/j.jcms.2011.08.010. Epub 2011 Sep 16.
The piamater, branchial arch derivatives and melanocytes, derivatives of the neural crest, are associated with rare, sporadic, non-inherited embryonic neuroectodermal dysplasia. We report a case of a 13 year-old girl with a malignant melanotic neuroectodermal tumour of infancy, an uncommon malignant extra-axial pigmented tumour with an aggressive clinical course. The clinical presentation, radiology, surgical management, adjuvant therapy are discussed along with a brief review of literature. The patient had widespread intracranial metastasis at presentation and rapidly deteriorated while on adjuvant therapy. A hyperdense extra-axial tumour on plain computed tomogram in a child could suggest a melanotic neuroectodermal tumour. Its malignant variant is associated with a poor prognosis when associated with widespread intracranial metastasis.
软脑膜、鳃弓衍生物和神经嵴衍生的黑色素细胞与罕见的、散发性的、非遗传性的胚胎神经外胚层发育不良有关。我们报告了一例 13 岁女孩患有婴儿恶性黑色素性神经外胚层肿瘤，这是一种罕见的恶性轴外色素性肿瘤，具有侵袭性的临床病程。本文讨论了该病例的临床表现、影像学、手术治疗、辅助治疗，并简要回顾了文献。该患者在就诊时已广泛颅内转移，并在辅助治疗期间迅速恶化。儿童头颅 CT 平扫显示高密度轴外肿瘤可能提示黑色素性神经外胚层肿瘤。当伴有广泛颅内转移时，其恶性变异与预后不良相关。
