Skull Base 2010; 20(5): 381-387
DOI: 10.1055/s-0030-1253576
CASE REPORT

© Thieme Medical Publishers

Malignant Transformation of Acoustic Neuroma/Vestibular Schwannoma 10 Years after Gamma Knife Stereotactic Radiosurgery

Andreas K. Demetriades1 , Nicholas Saunders2 , Peter Rose3 , Cyril Fisher4 , Jeremy Rowe5 , Robert Tranter2 , Carl Hardwidge1
  • 1Department of Neurosurgery, Hurstwood Park Neurological Centre, Brighton and Sussex University Hospitals, London, United Kingdom
  • 2Neuro-Otology, Hurstwood Park Neurological Centre, Brighton and Sussex University Hospitals, London, United Kingdom
  • 3Neuropathology, Hurstwood Park Neurological Centre, Brighton and Sussex University Hospitals, London, United Kingdom, London, United Kingdom
  • 4Department of Pathology, Royal Marsden Hospital, London, United Kingdom
  • 5Department of Neurosurgery, National Centre for Stereotactic Radiosurgery, Royal Hallamshire Hospital, London, United Kingdom
Further Information

Publication History

Publication Date:
28 April 2010 (online)

ABSTRACT

Only a handful of cases of de-novo malignancies of the vestibulocochlear nerve have been reported. Even rarer is the malignant transformation of a previously histologically diagnosed benign vestibular schwannoma. We present the case of a young adult who had combined operative/Gamma knife treatment for a benign vestibular schwannoma, followed by further surgery 2 years later. He represented 10 years after original diagnosis with facial numbness and ataxia, MRI showing gross tumor recurrence. After radical resection, histology showed malignant transformation to a malignant peripheral nerve sheath tumor. Within 3 months there was rapid, aggressive recurrence with brainstem compression, requiring further surgery for brainstem decompression. Histology confirmed further de-differentiation to an anaplastic sarcoma. While awaiting radiotherapy the tumor recurred again, the patient succumbing. The patient had no features of neurofibromatosis type 2. In the literature there are 13 other cases of malignant vestibular schwannomata. Only six had radiotherapy and of these only two had histological confirmation of a benign lesion preradiotherapy. Neither of these had neurofibromatosis. Three other cases had histological proof of malignancy postradiosurgery, but with no preradiotherapy histology; of these, two were positive for neurofibromatosis. The tumor biology of vestibular schwannomata as well as the radiobiology in the context of malignant transformation is discussed.

REFERENCES

  • 1 Mathieu D, Kondziolka D, Flickinger J C et al.. Stereotactic radiosurgery for vestibular schwannomas in patients with neurofibromatosis type 2: an analysis of tumor control, complications, and hearing preservation rates.  Neurosurgery. 2007;  60(3) 460-468 discussion 468-470
  • 2 Rowe J, Grainger A, Walton L, Silcocks P, Radatz M, Kemeny A. Risk of malignancy after gamma knife stereotactic radiosurgery.  Neurosurgery. 2007;  60(1) 60-65 discussion 65-66
  • 3 Shin M, Ueki K, Kurita H, Kirino T. Malignant transformation of a vestibular schwannoma after gamma knife radiosurgery.  Lancet. 2002;  360(9329) 309-310
  • 4 Thomsen J, Mirz F, Wetke R, Astrup J, Bojsen-Møller M, Nielsen E. Intracranial sarcoma in a patient with neurofibromatosis type 2 treated with gamma knife radiosurgery for vestibular schwannoma.  Am J Otol. 2000;  21(3) 364-370
  • 5 Chang S M, Barker II F G, Larson D A, Bollen A W, Prados M D. Sarcomas subsequent to cranial irradiation.  Neurosurgery. 1995;  36(4) 685-690
  • 6 Gupta G, Maniker A. Malignant peripheral nerve sheath tumors.  Neurosurg Focus. 2007;  22(6) E12
  • 7 Malkin D, Li F P, Strong L C et al.. Germ line p53 mutations in a familial syndrome of breast cancer, sarcomas, and other neoplasms.  Science. 1990;  250(4985) 1233-1238
  • 8 Brachman D G, Hallahan D E, Beckett M A, Yandell D W, Weichselbaum R R. p53 gene mutations and abnormal retinoblastoma protein in radiation-induced human sarcomas.  Cancer Res. 1991;  51(23 Pt 1) 6393-6396
  • 9 Cordon-Cardo C, Latres E, Drobnjak M et al.. Molecular abnormalities of mdm2 and p53 genes in adult soft tissue sarcomas.  Cancer Res. 1994;  54(3) 794-799
  • 10 Jacoby L B, MacCollin M, Barone R, Ramesh V, Gusella J F. Frequency and distribution of NF2 mutations in schwannomas.  Genes Chromosomes Cancer. 1996;  17(1) 45-55
  • 11 Lee D J, Maseyesva B, Westra W, Long D, Niparko J K, Califano J. Microsatellite analysis of recurrent vestibular schwannoma (acoustic neuroma) following stereotactic radiosurgery.  Otol Neurotol. 2006;  27(2) 213-219
  • 12 Light J P, Roland Jr J T, Fishman A, Miller D C, Cohen N L. Atypical and low-grade malignant vestibular schwannomas: clinical implications of proliferative activity.  Otol Neurotol. 2001;  22(6) 922-927
  • 13 Bari M E, Forster D M, Kemeny A A, Walton L, Hardy D, Anderson J R. Malignancy in a vestibular schwannoma. Report of a case with central neurofibromatosis, treated by both stereotactic radiosurgery and surgical excision, with a review of the literature.  Br J Neurosurg. 2002;  16(3) 284-289
  • 14 McLean C A, Laidlaw J D, Brownbill D S, Gonzales M F. Recurrence of acoustic neurilemoma as a malignant spindle-cell neoplasm. Case report.  J Neurosurg. 1990;  73(6) 946-950
  • 15 Comey C H, McLaughlin M R, Jho H D, Martinez A J, Lunsford L D. Death from a malignant cerebellopontine angle triton tumor despite stereotactic radiosurgery. Case report.  J Neurosurg. 1998;  89(4) 653-658
  • 16 Hanabusa K, Morikawa A, Murata T, Taki W. Acoustic neuroma with malignant transformation. Case report.  J Neurosurg. 2001;  95(3) 518-521
  • 17 Kudo M, Matsumoto M, Terao H. Malignant nerve sheath tumor of acoustic nerve.  Arch Pathol Lab Med. 1983;  107(6) 293-297
  • 18 Shin M, Ueki K, Kurita H, Kirino T. Malignant transformation of a vestibular schwannoma after gamma knife radiosurgery.  Lancet. 2002;  360(9329) 309-310
  • 19 Gruber B, Petchenik L, Williams M, Thomas C, Luken M G. Malignant vestibular schwannoma.  Skull Base Surg. 1994;  4(4) 227-231
  • 20 Thomsen J, Mirz F, Wetke R, Astrup J, Bojsen-Møller M, Nielsen E. Intracranial sarcoma in a patient with neurofibromatosis type 2 treated with gamma knife radiosurgery for vestibular schwannoma.  Am J Otol. 2000;  21(3) 364-370
  • 21 Mrak R E, Flanigan S, Collins C L. Malignant acoustic schwannoma.  Arch Pathol Lab Med. 1994;  118(5) 557-561
  • 22 Matsumoto M, Sakata Y, Sanpei K, Onagi A, Terao H, Kudo M. [Malignant schwannoma of acoustic nerve: a case report].  No Shinkei Geka. 1990;  18(1) 59-62
  • 23 Gonzalez L F, Lekovic G P, Eschbacher J, Coons S, Spetzler R F. A true malignant schwannoma of the eighth cranial nerve: case report.  Neurosurgery. 2007;  61(2) E421-E422, discussion E422
  • 24 Wilkinson J S, Reid H, Armstrong G R. Malignant transformation of a recurrent vestibular schwannoma.  J Clin Pathol. 2004;  57(1) 109-110
  • 25 Son E I, Kim I M, Kim S P. Vestibular schwannoma with malignant transformation: a case report.  J Korean Med Sci. 2001;  16(6) 817-821

Andreas K Demetriades

Department of Neurosurgery, King’s College Hospital

Denmark Hill, London SE5 9RS, UK

Email: andreas.demetriades@gmail.com