Journal of Korean Neurosurgical Society

The Korean Neurosurgical Society (대한신경외과학회)
권호 표지
DOI QR코드

DOI QR Code

A Long-Term Survival Case of a Primary Malignant Intracerebral Nerve Sheath Tumor

  • Lee, Byung Sun (Department of Neurosurgery, Chungbuk National University School of Medicine & Medical Research Institute) ;
  • Kim, Young Gyu (Department of Neurosurgery, Chungbuk National University School of Medicine & Medical Research Institute) ;
  • Kim, Dong Ho (Department of Neurosurgery, Chungbuk National University School of Medicine & Medical Research Institute) ;
  • Lee, Mou Seop (Department of Neurosurgery, Chungbuk National University School of Medicine & Medical Research Institute)
  • Received : 2013.05.28
  • Accepted : 2013.09.15
  • Published : 2013.09.28
Download PDF
⟨ Previous Next ⟩

Abstract

We report a long-term survival case of a primary malignant intracerebral nerve sheath tumor (MINST) occurring in the right frontal lobe of a 13-year old boy. After the gross total resection (GTR), we have performed radiation therapy but it recurred 50 months after the surgery, so the second GTR was performed. Later, second tumor recurrence was found 4 months after the second surgery. Subsequently the third GTR, radiotherapy, and chemotherapy were carried out. At present, the patient has been remaining alive for 77 months without evidence of tumor recurrence. According to the previous reports, the primary MINST is very rare : there are only 8 cases reported. It is also a fast-growing, invasive tumor with poor outcome. This is the first case that had no recurrence for 50 months after the surgery among the reported cases that had been followed up for more than 5 years. It is supposed that a period of recurrence free survival after GTR and low mitotic activity are associated with the patient's prognosis. A GTR followed by adjuvant radiation therapy and chemotherapy will be recommended to patients of MINST.

Keywords

References

  1. Barnard ZR, Agarwalla PK, Jeyaretna DS, Farrell CJ, Gerstner ER, Tian D : Sporadic primary malignant intracerebral nerve sheath tumor : case report and literature review. J Neurooncol 104 : 605-610, 2011 https://doi.org/10.1007/s11060-011-0531-7
  2. Beauchesne P, Mosnier JF, Schmitt T, Brunon J : Malignant nerve sheath tumor of the right cerebral peduncle : case report. Neurosurgery 54 : 500-504, 2004 https://doi.org/10.1227/01.NEU.0000103491.18482.E3
  3. Bhattacharyya AK, Perrin R, Guha A : Peripheral nerve tumors : management strategies and molecular insights. J Neurooncol 69 : 335-349, 2004 https://doi.org/10.1023/B:NEON.0000041891.39474.cb
  4. Bruner JM, Humphreys JH, Armstrong DL : Immunocytochemistry of recurring intracerebral nerve sheath tumor. J Neuropathol Exp Neurol 43 : 1, 1984 https://doi.org/10.1097/00005072-198401000-00001
  5. Carli M, Ferrari A, Mattke A, Zanetti I, Casanova M, Bisogno G, et al. : Pediatric Malignant Peripheral Nerve Sheath Tumor : The Italian and German Soft Tissue Sarcoma Cooperative Group. J Clin Oncol 20 : 8422-8430, 2005
  6. Chen L, Mao Y, Chen H, Zhou LF : Diagnosis and management of intracranial malignant peripheral nerve sheath tumors. Neurosurgery 62 : 825-832, 2008 https://doi.org/10.1227/01.neu.0000318167.97966.f3
  7. Ducatman BS, Scheithauer BW, Piepgras DG, Reiman HM, Ilstrup DM : Malignant peripheral nerve sheath tumors. A clinicopatholgic study of 120 cases. Cancer 57 : 2006-2021, 1986 https://doi.org/10.1002/1097-0142(19860515)57:10<2006::AID-CNCR2820571022>3.0.CO;2-6
  8. Enzinger FM, Weiss SW : Malignant tumor of the peripheral nerves, in Enzinger FM, Weiss SW (eds) : Soft Tissue Tumors, ed 3. St Louis, MO, CV Mosby, 1995, pp889-928
  9. Kim ET, Namgung H, Shin HD, Lee SI, Kwon JE, Chang MC, et al. : Oncologic manifestations of neurofibromatosis type 1 in Korea. J Korean Surg Soc 82 : 205-210, 2012 https://doi.org/10.4174/jkss.2012.82.4.205
  10. Kozic D, Nagulic M, Samardzic M, Ostojic J, Rasulic L, Cvetkovic-Dozic D : Intrapontine malignant nerve sheath tumor : MRI and MRS features. Acta Neurol Belg 108 : 67-71, 2008
  11. Rees J : Advances in magnetic resonance imaging of brain tumors. Curr Opin Neurol 16 : 643-650, 2003 https://doi.org/10.1097/00019052-200312000-00001
  12. Sharma S, Abbott RI, Zagzag D : Malignant intracerebral nerve sheath tumor : A case report and review of the literature. Cancer 82 : 545-552, 1998. https://doi.org/10.1002/(SICI)1097-0142(19980201)82:3<545::AID-CNCR17>3.0.CO;2-1
  13. Shin HY : Korean society for pediatric neuro-oncology (KSPNO). Korean J pediatr Hematol Oncol 12 : 188-200, 2005
  14. Singh RVP, Suys S, Campbell DA, Broome JC : Malignant schwannoma of the cerebellum: case report. Surg Neurol 39 : 128-132, 1993 https://doi.org/10.1016/0090-3019(93)90090-N
  15. Stefanko SZ, Vuzevski VD, Maas AI, van Vroonhoven CC : Intracerebral malignant schwannoma. Acta Neuropathol 71 : 321-325, 1986 https://doi.org/10.1007/BF00688056
  16. Tanaka M, Shibui S, Nomura K, Nakanishi Y, Hasegawa T, Hirose T : Malignant intracerebral nerve sheath tumor with intratumoral calcification. Case report. J Neurosurg 92 : 338-341, 2000 https://doi.org/10.3171/jns.2000.92.2.0338

Cited by

  1. Epithelioid Malignant Intracerebral Nerve Sheath Tumor: A Case Report and a Comparison with Conventional Type vol.17, pp.2, 2013, https://doi.org/10.12729/jbtr.2016.17.2.040
  2. Intracranial Malignant Peripheral Nerve Sheath Tumors Not Associated with a Cranial Nerve: Systematic Review and Illustrative Case vol.156, pp.None, 2013, https://doi.org/10.1016/j.wneu.2021.09.072