The authors report a rare case of primary disseminated intradural malignant peripheral nerve sheath tumor (MPNST) of the spine in a 5-year-old child without neurofibromatosis type I (NF-I). This child presented with abdominal pain and gait disturbance. MRI revealed a large intradural extramedullary tumor at C4–5 with dissemination to the thoracic spine, cauda equina and leptomeninges. Following a 2-level cervical laminectomy, the tumor was biopsied and debulked. Based on pathological and immunohistological findings, the tumor was diagnosed as an MPNST. Because of the rarity of this pathological entity, a review of the literature was performed. The reported clinical outcomes for adult and pediatric patients with intradural MPNST are very poor. No established standard for the treatment of these tumors exists. We report the first pediatric case – without or with NF-I – of disseminated intradural MPNST primarily localized proximal to the conus medullaris. It must always be considered in the differential diagnosis for intradural extramedullary tumors of the pediatric spine, along with neurofibromas and schwannomas, even in children without NF-I.

Keywords:
Intradural extramedullary spine tumor, Malignant peripheral nerve sheath tumor, Pediatric spine
1.
Baysefer A, Akay K, Izci Y, Kayali H, Timurkaynak E: The clinical and surgical aspects of spinal tumors in children. Pediatr Neurol 2004;31:261–266.
2.
Yamamoto Y, Raffel C: Spinal extradural neoplasms and intradural extramedullary neoplasms; in Albright A, Pollack I, Adelson P (eds): Principles and Practice of Pediatric Neurosurgery. New York, Thieme, 1999.
3.
Kim D, Murovic J, Tiel R, Moes G, Kline D: A series of 397 peripheral neural sheath tumors: 30-year experience at Louisiana State University Health Sciences Center. J Neurosurg 2005;102:246–255.
4.
Stark A, Buhl R, Hiugo H, Mehdorn H: Malignant peripheral nerve sheath tumours – report of 8 cases and review of the literature. Acta Neurochir (Wien) 2001;143:357–364.
5.
Gupta G, Maniker A: Malignant peripheral nerve sheath tumors. Neurosurg Focus 2007;22:E12.
6.
West D, Parra R, Manepalli A, Bernardi R, Cummings J: Development of a malignant peripheral nerve sheath tumor following treatment for testicular seminoma. Urology 1997;50:292–294.
7.
Amin A, Saifuddin A, Flanagan A, Patterson D, Lehovsky J: Radiotherapy-induced malignant peripheral nerve sheath tumor of the cauda equina. Spine 2004;29:E506–E509.
8.
Thomeer RT, Bots GT, van Dulken H, Luyendijk W, Helle P: Neurofibrosarcoma of the cauda equina. Case Report, J Neurosurg 1981;54:409–411.
9.
Valdueza JM, Hagel C, Westphal M, Hänsel M, Herrmann HD: Primary spinal malignant schwannoma: clinical, histological, and cytogenetic findings. Neurosurg Rev 1991;14:283–291.
10.
Seppala MT, Haltia MJ: Spinal malignant nerve-sheath tumor or cellular schwannoma? A striking difference in prognosis. J Neurosurg 1993;79:528–532.
11.
Acharya R, Bhalla S, Sehgal AD: Malignant peripheral nerve sheath tumor of the cauda equina. Neurol Sci 2001;22:267–270.
12.
Amin A, Saifuddin A, Flanagan A, Patterson D, Lehovsky J: Radiotherapy-induced malignant peripheral nerve sheath tumor of the cauda equina. Spine 2004;29:E506–E509.
13.
Yone K, Ijiri K, Hayashi K, Yokouchi M, Takenouchi T, Manago K, Nerome Y, Ijichi O, Ikarimoto N, Komiya S: Primary malignant peripheral nerve sheath tumor of the cauda equina in a child: case report. Spinal Cord 2004;42:199–203.
14.
Adamson DC, Cummings TJ, Friedman AH: Malignant peripheral nerve sheath tumor of the spine after radiation therapy for Hodgkin’s lymphoma. Clin Neuropathol 2004;23:245–255.
15.
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 Neurooncol 2006;78:187–190.
16.
Baehring J, Betensky R, Batchelor T: Malignant peripheral nerve sheath tumor: the clinical spectrum and outcome of treatment. Neurology 2003;61:696–698.
17.
Hajdu SI: Peripheral nerve sheath tumors: histogenesis, classification, and prognosis. Cancer 1993;72:3549–3552.
18.
Ducatman B, Scheithauer B, Piepgras D, Reiman H, Ilstrup D: Malignant peripheral nerve sheath tumors: a clinicopathologic study of 120 cases. Cancer 1986;57:2006–2021.
19.
Glover T, Stein C, Lequius E, Andersen L, Brereton A, Johnson S: Molecular and cytogenetic analysis of tumors in von Recklinghausen neurofibromatosis. Genes Chromosomes Cancer 1991;3:62–70.
20.
Angelov L, Davis A, O’Sullivan B, Bell R, Guha A: Neurogenic sarcomas: experience at the University of Toronto. Neurosurgery 1998;43:56–64.
21.
Evans D, Baser M, McGaughran J, Sharif S, Howard E, Moran A: Malignant peripheral nerve sheath tumors in neurofibromatosis 1. J Med Genet 2002;39:311–314.
22.
Grnja V, Allen W, Osborn D, Kier E: Sacral neurofibrosarcoma: an angiographic evaluation. J Neurosurg 1974;40:767–771.
23.
Kloos R, Rufini V, Gross M, Shapiro B: Bone scans in neurofibromatosis: neurofibroma, plexiform neuroma and neurofibrosarcoma. J Nucl Med 1996;37:1778–1783.
24.
Riccardi V, Powell P: Neurofibrosarcomas as a complication of von Recklinghausen neurofibromatosis. Neurofibromatosis 1989;2:152–165.
25.
Kett-White R, Martin J, Jones E, O’Brien C: Malignant spinal neurofibrosarcoma. Spine 2000;25:752–755.
26.
Cybulski G, Greager J, Gleason T, Reyes M, Fayez Homsi M: Malignant schwannoma (neurofibrosarcoma) metastatic to spine. Spine 1989;14:549–551.
27.
D’Agostino A, Soule E, Miller R: Sarcomas of the peripheral nerves and somatic soft tissues associated with multiple neurofibromatosis. Cancer 1963;16:1015–1027.
28.
Preston F, Walsh W, Clarke T: Cutaneous neurofibromatosis (von Recklinghausen’s disease). Arch Surg 1962;64:813–827.
29.
Baek W, Pytel P, Undevia S, Rubeiz H: Spinal cord metastasis of a non-neurofibromatosis type-1 malignant peripheral nerve sheath tumor: an unusual manifestation of a rare tumor. J Neurooncol 2005;74:183–185.
30.
Mazel C, Topouchian V, Grunenwald D: Effectiveness of radical resections in malignant dumbbell tumors of the thoracic spine: review of three cases. J Spinal Disord Tech 2002;15:507–512.
31.
Ferner R, Gutmann D: International consensus statement on malignant peripheral nerve sheath tumors in neurofibromatosis 1. Cancer Res 2002;62:1573–1577.
32.
Carli M, Ferrari A, Mattke A, Zanetti I, Casanova M, Bisogno G, Cecchetto G, Alaggio R, De Sio L, Koscielniak E, et al: Pediatric malignant peripheral nerve sheath tumor: the Italian and German Soft Tissue Sarcoma Cooperative Group. J Clin Oncol 2005;23:8422–8530.
33.
Doorn P, Molenaar W, Buter J, Hoekstra H: Malignant peripheral nerve sheath tumors in patients with and without neurofibromatosis. Eur J Surg Oncol 1995;21:78–82.
34.
Wanebo J, Malik J, VandenBerg S, Wanebo H, Driesen N, Persing J: Malignant peripheral nerve sheath tumors: a clinicopathologic study of 28 cases. Cancer 1993;71:1247–1253.
35.
Vege D, Chinoy R, Ganesh B, Parikh D: Malignant peripheral nerve sheath tumors of the head and neck: a clinicopathological study. J Surg Oncol 1994;55:100–103.
36.
Wong W, Hirose T, Scheithauer B, Schild S, Gunderson L: Malignant peripheral nerve sheath tumor: analysis of treatment outcome. Int J Radiat Oncol Biol Phys 1998;42:351–360.
37.
Kourea H, Bilsky M, Leung D, Lewis J, Woodruff J: Subdiaphragmatic and intrathoracic paraspinal malignant peripheral nerve sheath tumors: a clinicopathologic study of 25 patients and 26 tumors. Cancer 1998;82:2191–2203.
38.
Meis J, Enzinger F, Martz K, Neal J: Malignant peripheral nerve sheath tumors (malignant schwannoma) in children. Am J Surg Pathol 1992;16:694–707.
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2009
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