Sandsmark M, Eskeland G, Skullerud K, Abyholm F
Department of Plastic Surgery, Rikshospitalet University Hospital, Oslo, Norway.
Scand J Plast Reconstr Surg Hand Surg. 1994 Jun;28(2):151-4. doi: 10.3109/02844319409071194.
Neurocutaneous melanosis is a rare congenital syndrome characterised by large or numerous congenital pigmented naevi and excessive proliferation of melanin-containing cells in the leptomeninges. The process is diffuse or multifocal, and has a tendency to infiltrate the neural tissue and the cerebrospinal cord; remote metastases may occur. There is usually histological evidence of malignancy (cellular pleomorphism and mitotic activity). Involvement of the basal cisterns is apt to cause internal hydrocephalus, and the prognosis is grave even when there is no histological evidence of malignancy. We present the case history and necropsy findings of a baby boy with neurocutaneous melanosis, followed by a brief review.
神经皮肤黑素沉着症是一种罕见的先天性综合征，其特征为存在巨大或众多先天性色素痣，以及软脑膜中含黑色素细胞的过度增殖。该过程呈弥漫性或多灶性，并有浸润神经组织和脊髓的倾向；可能会发生远处转移。通常有恶性肿瘤的组织学证据（细胞多形性和有丝分裂活性）。基底池受累易导致梗阻性脑积水，即使没有恶性肿瘤的组织学证据，预后也很差。我们报告一名患有神经皮肤黑素沉着症男婴的病例史和尸检结果，随后进行简要综述。
