Multiple hepatic neurofibromatosis 1 case of malignant peripheral nerve sheath tumor.doc

 PAGE \* MERGEFORMAT 9 Multiple hepatic neurofibromatosis 1 case of malignant peripheral nerve sheath tumor Of: Li Zhen Wu Gang Han Xinwei Li Tengfei [Keywords:] pancreatic neoplasms; solid pseudopapillary tumor; X-ray computed Case Information Female patients, aged 40, for “right upper quadrant abdominal discomfort with pain in March,” admitted. Examination: multiple coffee pigmented body can be seen, the lower abdomen and right hip palpable mass in several sizes, soft, no tenderness, higher than the skin about 2cm, the size was 20cm × 15cm, 15cm × 10cm. laboratory AFP negative, CT showed a huge space-occupying liver (Figure 1-2), showed slightly abnormal liver function, no other special. hepatic needle biopsy, diagnosed as low grade fibrosarcoma (Figure 3), immunohistochemistry confirmed as malignant peripheral nerve sheath tumor. Because the patient refused surgical treatment, carry out by hepatic artery infusion chemotherapy (TACE), surgery, the tumor blood vessels increased, tortuous disorders (Figure 4), then indwelling catheter tip to the tumor feeding artery, the catheter micro-pump for 48 hours continuous infusion of cisplatin 70mg, THP40mg, THP and 48% after lipiodol emulsion embolization the tumor bed, gelatin sponge feeding artery embolization. significantly reduced postoperative symptoms of .1 month later abdominal CT showed liver lesions better lipiodol deposition (Figure 5), also found that 2,3-right side of the lumbar nerve root and soft tissue mass, given nerve fiber Tumor; l colon tumors (Figure 6 arrow), colon colonoscopy showed an irregular liver see uplift, occupy the lumen of about 3 / 4, about 6cm, surface erosion, ulcers, hard, pathological consider malignancy proposed immunohistochemistry. but the patient abandoned further treatment. Discussion Multiple neurofibromatosis is derived from neural crest cells in autosomal dominant genetic disease that can lead to multi-system damage, the clinical features of skin milk coff