Occurence of Peripheral Primitive Neuroectodermal Tumor within Spinal Nerve Root – a Case Report

Czech version

Authors: P. Buchvald ¹; P. Barsa ¹; A. Blüml ²; R. Lukáš ³
Authors‘ workplace: Neurocentrum, Neurochirurgické oddělení, Krajská nemocnice Liberec, a. s. ¹; Oddělení patologie, Krajská nemocnice Liberec, a. s. ²; Traumatologicko-ortopedické centrum, Krajská nemocnice Liberec, a. s. ³
Published in: Cesk Slov Neurol N 2015; 78/111(3): 344-347
Category: Case Report

Overview

Peripheral primitive neuroectodermal tumor (pPNET) is a highly malignant soft tissue sarcoma probably originating from embryonic neural crest cells. For some common cytological, immunohistochemical and cytogenetic features with bone Ewing’s sarcoma, pPNET is also sometimes inaccurately referred to as Ewing’s sarcoma of soft tissues. pPNET probably is a different morphological expression of the same tumor type. pPNET mainly occurs in younger patients in various locations, always in close proximity to a peripheral nerve. However, its occurrence within a spinal nerve root is very rare and, so far, only a few cases have been described in the literature. In our report, we present a case of 35-year-old woman with pPNET in extraforaminal projection of L5 root. As a result of early diagnosis and treatment, including radical surgery combined with aggressive chemotherapy and irradiation of the tumor bed, no recurrence has been reported so far. The follow-up period is more than five years.

Key words:
peripheral primitive neuroectodermal tumor – spinal nerve root – neurosurgery – chemotherapy – radiotherapy

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE “uniform requirements” for biomedical papers.

Sources

1. Hart MN, Earle KM. Primitive neuroectodermal tumours of the brain in children. Cancer 1973; 32(4): 890– 897.

2. Kielhuer P, Bueger PC, Scheithauer BW. The new WHO classification of brain tumours. Brain Pathol 1993; 3(3): 255– 268.

3. Barbara J. Primitive neuroectodermal tumours. In: Wilkins RH, Rengachary SS (eds). Neurosurgery. New York: McGraw Hill 1996: 1707– 1713.

4. Angervall L, Enzinger FM. Extraskeletal neoplasm resembling Ewing’s sarcoma. Cancer 1975; 36(1): 240– 251.

5. Horowitz ME, Malawer MM, DeLaney TF, Tsokos MG. Ewing’s sarcoma family of tumors: Ewing’s sarcoma of bone and soft tissue and the peripheral primitive neuroectodermal tumors. Principles and practice of pediatric oncology. 2nd ed. Philadelphia: Lippincott Williams & Wilkins 1993: 795– 821.

6. Fink LH, Meriwether MW. Primary epidural Ewing’s sarcoma presenting as a lumbar disc protrusion. J Neurosurg 1979; 51(1): 120– 123.

7. Hadfield MG, Quezado MM, Williams RL, Luo VY. Ewing’s family of tumors involving structures related to the central nervous system: a review. Pediatr Dev Pathol 2000; 3(3): 203– 210.

8. Benesch M, Urban C, Lackner H, Kerbl R, Schwinger W, Beham‑ Schmid C et al. Atypical extraosseous Ewing’s sarcoma of the spinal canal with bone marrow involvement in a two‑month‑ old boy. Med Pediatr Oncol 1999; 32(6): 471– 473.

9. Spaziante R, de Divitiis E, Giamundo A, Gambardella A, Di Prisco B. Ewing’s sarcoma arising primarily in the spinal eoidural space: fifth case report. Neurosurgery 1983; 12(3): 337– 341.

10. Hisaoka M, Hashimoto H, Murao T. Peripheral primitive neuroectodermal tumor with ganglioneuroma‑like areas arising in the cauda equina. Virchows Arch 1997; 431(5): 365– 369.

11. McDermott VG, el‑ Jabbour JN, Sellar RJ, Bell J. Primitive neuroectodermal tumor of the cauda equina. Neuroradiology 1994; 36(3): 228– 230.

12. Isotalo PA, Agbi C, Davidson B. Primary primitive neuroectodermal tumor of the cauda equina. Human Pathol 2000; 31(8): 999– 1001.

13. Weil RJ, Zhuang Z, Pack S, Kumar S, Helman L, Fuller BG et al. Intramedullary Ewing sarcoma of the spinal cord: consequences of molecular diagnosis. J Neurosurg (Spine) 2001; 95 (Suppl 2): 270– 275.

14. Mawrin C, Synowitz HJ, Kirches E, Kutz E, Dietzmann K, Weis S. Primary primitive neuroectodermal tumor of the spinal cord: case report and review of the literature. Clin Neurol Neurosurg 2002; 104(1): 36– 40.

15. Ishikawa S, Ohshima Y, Suzuki T, Oboshi S. Primitive neuroectodermal tumor of spinal nerve root. Acta Pathol Jpn 1979; 29(2): 289– 301.

16. Liu H, Yang WC, Garcia RL, Noh JM, Malhotra V, Leeds NE. Intraspinal primitive neuroectodermal tumor arising from the sacral spinal nerve root. J Comp Tomography 1987; 11(4): 350– 354.

17. Schmidt D, Herrmann C, Jürgens H, Harms D. Malignant peripheral neuroectodermal tumor and its necessary distinction from Ewing’s sarcoma. Cancer 1991; 68(10): 2251– 2259.

18. Lewis I, Jürgens H, Oberlin O, Gadner H, Judson I, Paulussen M. EuroEWING 99. Amended protocol version 2006: 1– 122.