Terry D G, Sauser D D, Gordon M D
VA Medical Center, Imaging Service, Portland, OR 97201, USA.
Skeletal Radiol. 1998 Jun;27(6):346-9. doi: 10.1007/s002560050395.
Malignant peripheral nerve sheath tumors (MPNSTs) are uncommon sarcomas that almost always arise in soft tissue. They can develop in pre-existing neurofibromas or schwannomas, de novo from peripheral nerves, or following radiation therapy. Primary intraosseous MPNST is rare and has been reported most frequently in the mandible. Of the reported cases involving the long bones, none has been associated with neurofibromatosis type 1 (NF-1). We report a case of MPNST arising in the femur in a patient with NF-1.
恶性外周神经鞘瘤（MPNSTs）是一种罕见的肉瘤，几乎总是发生于软组织。它们可在已存在的神经纤维瘤或施万细胞瘤中发生，也可原发于外周神经，或在放射治疗后出现。原发性骨内MPNST罕见，最常报道于下颌骨。在报道的累及长骨的病例中，无一例与1型神经纤维瘤病（NF-1）相关。我们报告1例发生于1例NF-1患者股骨的MPNST。
