关键词: 神经纤维瘤病Ⅰ型, 腹膜后肿物 , 恶性神经鞘瘤

Abstract:

Type I neurofibromatosis（NF-1）with retroperitoneal massive tumor is rare in clinical practice, and there are rare cases of the retroperitoneal massive tumor complicated with a low grade malignant schwannoma. This paper reports a case of retroperitoneal hyperplasia of NF-1, who presented the multiple subcutaneous nodules and was found right posterior inferior vena cava mass by CT imaging and diagnoses as neurocutaneous syndrome（such as neurofibromatosis type I）. The operation proved that retroperitoneal mass was neurofibroma accompanied with local malignant change. The patient was followed up three months and recovered well.

Key words: neurofibromatosis, retroperitoneal mass, malignant schwannoma