Simultaneous presentation in the neck and abdomen of malignant peripheral nerve sheath tumors involving two different nerve tracts

Abstract 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.