Neurofibromatosis type1 （NF1）, also known as Von Recklinghausen＇s disease with approximate occurrence of 1/3000 births, can be considered as a common autosomal dominant condition. Abdominal organs can be involved a...Neurofibromatosis type1 （NF1）, also known as Von Recklinghausen＇s disease with approximate occurrence of 1/3000 births, can be considered as a common autosomal dominant condition. Abdominal organs can be involved as neuro-fibroma and tumor growth in the liver, mesentery, retropedtoneum, stomach and bowel. Association of NF and gastrointes-tinal neoplasms occur in 2%-25% of patients. There is a 5%-10% lifetime risk of developing an NFl-associated malignant peripheral nerve sheath tumor （MPNST）, usually adsing within a pre-existing plexiform neurofibroma which metastasizes widely heraldinga poor outcome. We report a 55 year old man with gastrointestinal NF which ultimately developed MPNST and deceased.展开更多
文摘Neurofibromatosis type1 （NF1）, also known as Von Recklinghausen＇s disease with approximate occurrence of 1/3000 births, can be considered as a common autosomal dominant condition. Abdominal organs can be involved as neuro-fibroma and tumor growth in the liver, mesentery, retropedtoneum, stomach and bowel. Association of NF and gastrointes-tinal neoplasms occur in 2%-25% of patients. There is a 5%-10% lifetime risk of developing an NFl-associated malignant peripheral nerve sheath tumor （MPNST）, usually adsing within a pre-existing plexiform neurofibroma which metastasizes widely heraldinga poor outcome. We report a 55 year old man with gastrointestinal NF which ultimately developed MPNST and deceased.
