Department of Internal Medicine, Mount Sinai School of Medicine/Queens Hospital Center, 62-86 164th Street, Jamaica, NY 11432, USA.
J Clin Neurosci. 2013 Apr;20(4):602-4. doi: 10.1016/j.jocn.2012.02.040. Epub 2012 Dec 28.
Neurofibromatosis-1 (NF-1) is a relatively common autosomal dominant disease and it is caused by mutation of the NF-1 tumor suppressor gene; hence, the individuals affected have increased risk for developing familial cancer. Abdominal cavity neurofibromas are common but the simultaneous occurrence of a malignant peripheral nerve sheath tumor (MPNST) in the chest and in the abdomen is rare. Here, we present a patient with MPNST (spindle cell sarcoma) affecting simultaneously the vagus nerve in the head and neck region, mediastinum, pleura as well as celiac plexus branches near the stomach.
神经纤维瘤病 1 型(NF-1)是一种相对常见的常染色体显性疾病,由 NF-1 肿瘤抑制基因的突变引起;因此,受影响的个体发生家族性癌症的风险增加。腹腔神经纤维瘤很常见,但同时发生胸部和腹部的恶性外周神经鞘瘤(MPNST)则很少见。在这里,我们介绍了一例 MPNST(梭形细胞肉瘤)患者,该患者同时累及头颈部、纵隔、胸膜的迷走神经以及胃附近的腹腔神经丛分支。