J Clin Neurosurg February 2013 Vol. 10 No. 1. doi /j. issn MPNST MPNST

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1 22 doi /j. issn MPNST 10 6 MPNST 6 + R A Primary spinal malignant peripheral nerve sheath tumor report of six cases and review of literature MA Jun WANG Gui-huai. Department of Neurosurgery Beijing Tiantan Hospital Affiliated to Capital Medical University Beijing China Abstract Objective To investigate the clinical characteristic and treatment of primary spinal malignant peripheral nerve sheath tumor MPNST. Methods The clinical data of 6 patients with primary spinal MPNST of our neurosurgery department in recent 10 years were analyzed with review of literature. Results The total or subtotal tumor resection were performed for each patient. The characteristics of the tumor vary from person to person. The diagnosis of MPNST was confirmed by postoperative pathology examination. Some went on for adjuvant radiotherapy. The symptoms relieved well after long-term of follow-up. But the recurrence rate of the tumor was relatively high. Conclusions Primary spinal MPNST has a low incidence. Preoperative diagnosis proves to be a challenge. Total tumor resection with adjuvant radiotherapy may be recommended. But given the malignant characteristic of the tumor total resection is difficult to achieve and due to the lack of experience of adjuvant therapy the overall prognosis is poor. Key words spinal tumor primary malignant peripheral nerve sheath tumor MPNST 6 5% 1 /2 1 /3 1 I NF ~ 62 WHO Ⅱ-Ⅳ ~ ~ MR T 1 WI T 2 WI T 1

2 MR CT MR T 1 T ~ 4. 1 cm 2. 5 cm d ~ 14 d 11 d ~ ~ MR 2 3 MR a-b 5 MR A T 2 B T 2 C T T 1 T 2 c-d 5 4 MR f T 2 g T 1 e-f 1 MR H T 2 I T 1 i HE 100 h HE 400

3 24 1 * ** *** **** Thomeer 1981 M 1 42 LS 3 Valdueza 1991 Seppala 1993 M F M 3 F C 3 T NF C 1 T NF LS Acharya 2001 M 1 32 LS 18 Amin M 1 38 LS 10 Yone 2004 M 1 4 LS 21 Adamson M 1 F C 6/ Albayrak M 1 25 T NF1 7 Chamoun F 1 5 C 4 Sasamori M 1 25 C NF1 46 Khan 1998 F 1 40 C 12 Gnanalingham F 1 59 T 12 Baek F 1 39 TS 2 Miyakoshi F 1 75 T 6 Moon M 1 41 C 30 Lee F 1 34 T M 6 F C 5 T 3 L 2 S * **M F ***C T L S ****NF1 I 3 Seppala ~ ~ MPNST 1 /2 1 /3 MPNST NF1 4 NF1 MPNST 28 ~ MPNST 10% 16 NF MPNST MPNST 14 MPNST

4 MPNST 3 /4 6 MPNST MPNST Ki /MIB-1 5% ~ 65% - 1% 50% ~ 70% S-100 S-100 MPNST p53 p27 p16 17 NF1 TP NF1 a 6 S-100 p53 SYN Vimentin GFAP NF EMA MPNST 2 1 MPNST MPNST 2 T 1 T d MPNST MPNST MR MPNST MPNST MPNST

5 26 MPNST 60% MPNST 80% MPNST % 23% MIB-1 > 25% MPNST > D Neurol Med Chir Tokyo MPNST WHO 1 Amin A Saifuddin A Flanagan A et al. Radiotherapy-induced malignant peripheral nerve sheath tumor of the cauda equine J. Spine E Yone K Ijiri K Hayashi K et al. Primary malignant peripheral nerve sheath tumor of the cauda equina in a child case report J. Spinal Cord Adamson DC Cummings TJ Friedman AH. Malignant peripheral nerve sheath tumor of the spine after radiation therapy for Hodgkin s lymphoma J. Clin Neuropathol Albayrak BS Gorgulu A Kose T. A case of intra-dural malignant peripheral nerve sheath tumor in thoracic spine associated with neurofibromatosis type 1 J. J Neurooncol Roukoz B. Chamoun William E. Whitehead Robert C. Dauser et al. Primary disseminated intradural malignant peripheral nerve sheath tumor of the spine in a child case report and review of the literature J. Pediatric Neurosurgery Toru Sasamori Kazutoshi Hida Shunsuke Yano et al. Favorable outcome after radical resection and subsequent local irradiation of malignant peripheral nerve sheath tumor in the cervical spine J Gnanalingham K Bhattacharjee S O Neill K. Intraosseous malignant peripheral nerve sheath tumor MPNST of the thoracic spine a rare cause of spinal cord compression J. Spine Phila pa E Baek WS Pytel P Undevia SD et al. Spinal cord metastasis of a non-neurofibromatosis type-1 malignant peripheral nerve sheath tumor an unusual manifestation of a rare tumor J. J Neurooncol Miyakoshi N Nishikawa Y Shimada Y et al. Intraosseous malignant peripheral nerve sheath tumor with focal epithelioid Ⅱ-Ⅳ differentiation of the thoracic spine J. Neurol India 10 Moon SJ Lee JK Seo BR and review of the literature J. Spine Lee CS Huh JS Chang JW MPNST resonance imaging J. J Korean Neurosurg Soc MRI J et al. An intraosseous malignant peripheral nerve sheath tumor of the cervical spine a case report et al. The early detection of recurrence of malignant peripheral nerve sheath tumor by frequent magnetic WHO Ⅲ MPNST Neurosci J MR MPNST CT 13 Than KD Ghori AK Wang AC et al. Metastatic malignant peripheral nerve sheath tumor of the cauda equine J. J Clin