Kokandkar H R, Vyas A S, Kumbhakarna N R, Totala R J
Govt. Medical College, Maharashta State, India.
Indian J Cancer. 1994 Jun;31(2):130-2.
Congenital plexiform neurofibroma is regarded as pathognomonic of neurofibromatosis (NF) especially when it is large and involves a major nerve trunk with changes of elephantiasis neuromatosa. Only very rarely malignant changes have been reported in cases who have NF for less than 5 years. 'Borderline' lesions are seen especially in patients with NF. In such cases criteria of mitotic activity should be utilized in establishing the diagnosis of malignancy. A case of 3 month child with congenital plexiform neurofibroma involving neck with elephantiasis neuromatosa with sarcomatous nodule has been described in the present article. The criteria for malignancy in nerve sheath tumors have also been discussed.
先天性丛状神经纤维瘤被视为神经纤维瘤病（NF）的特征性表现，尤其是当它体积较大且累及主要神经干并伴有神经瘤性象皮肿改变时。在患NF不到5年的病例中，仅有极少恶变的报道。“临界性”病变尤其见于NF患者。在这类病例中，有丝分裂活性标准应被用于确立恶性肿瘤的诊断。本文描述了1例3个月大的儿童，患有累及颈部并伴有神经瘤性象皮肿及肉瘤样结节的先天性丛状神经纤维瘤。文中还讨论了神经鞘瘤恶变的标准。
