22 doi 10. 3969 /j. issn. 1672-7770. 2013. 01. 009 6 MPNST 10 6 MPNST 6 + R739. 42 A 1672-7770 2013 01-0022-05 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 100050 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 NF1 1. 1 4 2 29 ~ 62 WHO Ⅱ-Ⅳ 42. 3 1 ~ 2 6. 5 3 3 3 2001 1 2 ~ 2012 6 2 100050 1. 2 6 MR T 1 WI T 2 WI T 1
2013 10 1 23 2 MR CT MR T 1 T 2 1. 3 1. 8 ~ 4. 1 cm 2. 5 cm 3 2 - - 2 4 2 4 2 2 d 5 1 4 2 2 2 4 7 ~ 14 d 11 d 3 2 3 4 ~ 29 1 5 3 1. 4 6 ~ 29 1 1 2 1 4 1 1 1 MR 2 3 MR 5 1 1 a-b 5 MR A T 2 B T 2 C T 1 2-3 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
24 1 * ** *** **** Thomeer 1981 M 1 42 LS 3 Valdueza 1991 Seppala 1993 M F 18-70 M 3 F 13-37 C 3 T NF1 1 1 3 1 C 1 T NF1 1 4 1 LS 1 12 1 1 1 10 1 18 7 8 1 5 2 7 8 18 6 Acharya 2001 M 1 32 LS 18 Amin 1 2004 M 1 38 LS 10 Yone 2004 M 1 4 LS 21 Adamson 3 2004 M 1 F 1 30 37 C 6/16 1 2 1 Albayrak 4 2006 M 1 25 T NF1 7 Chamoun 5 2008 F 1 5 C 4 Sasamori 6 2012 M 1 25 C NF1 46 Khan 1998 F 1 40 C 12 Gnanalingham 7 2004 F 1 59 T 12 Baek 8 2005 F 1 39 TS 2 Miyakoshi 9 2007 F 1 75 T 6 Moon 10 2008 M 1 41 C 30 Lee 11 2010 F 1 34 T 14 12 2011 M 6 F 6 7-71 C 5 T 3 L 2 S * **M F ***C T L S ****NF1 I 3 Seppala 1993 5 30 ~ 60 40 ~ 44 12 2011 12 5 MPNST 1 /2 1 /3 MPNST NF1 4 NF1 MPNST 28 ~ 36 1. 16 1 13 6 MPNST 10% 16 NF1 1 3 3 42. 3 3 3 1 MPNST MPNST 14 MPNST
2013 10 1 25 MPNST 3 /4 6 MPNST MPNST Ki- - 67 /MIB-1 5% ~ 65% - 1% 50% ~ 70% S-100 S-100 MPNST p53 p27 p16 17 NF1 TP53 22 2 14 13 17 18 3 NF1 a 6 S-100 p53 SYN Vimentin GFAP NF EMA MPNST 2 1 MPNST 4 2 4 MPNST 2 T 1 T 2 2 12 2 d MPNST MPNST MR MPNST MPNST MPNST 7-11 14
26 MPNST 60% MPNST 80% MPNST 5 10 34% 23% MIB-1 > 25% MPNST 13 8 7 7 3 1 7 5 3 6 > 12 2 12 14 10 + 1 10 + 5 3 2 18 2 18 46 + + 1 30 + 1 + + -D + + + 1 21 + + 1 4 4 1 2 5 6-11 6 1 2 1 1 1 2 6 29 Neurol Med Chir Tokyo 1 4 1 2 5 MPNST WHO 1 Amin A Saifuddin A Flanagan A et al. Radiotherapy-induced malignant peripheral nerve sheath tumor of the cauda equine J. Spine 2004 29 E506. 2 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 2004 42 199. 3 Adamson DC Cummings TJ Friedman AH. Malignant peripheral nerve sheath tumor of the spine after radiation therapy for Hodgkin s lymphoma J. Clin Neuropathol 2004 23 245. 4 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 2006 78 187. 5 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 2009 45 230. 6 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. 2012 52 670. 7 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 1976 2004 29 E402. 8 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 2005 74 183. 9 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 2008 33 712. 11 Lee CS Huh JS Chang JW MPNST resonance imaging J. J Korean Neurosurg Soc 2010 12 MRI J. 2012 36 209. 2007 55 64. 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 2011 18 844. 14 1 J. 2012 9 220. MR MPNST 47 51.. CT 13 Than KD Ghori AK Wang AC et al. Metastatic malignant peripheral nerve sheath tumor of the cauda equine J. J Clin. 2012-09-30 2012-10-22