Spontaneous regression of vestibular schwannomas after resection of contralateral tumor in neurofibromatosis Type 2

Report of 2 cases

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  • 1 Departments of Neurologic Surgery and
  • 2 Otorhinolaryngology, Mayo Clinic, Rochester, Minnesota
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The authors report on 2 patients with bilateral vestibular schwannomas (VSs) who underwent unilateral surgical tumor removal. One patient was followed up for 4 years, the other for 9; in both cases, the contralateral VS regressed markedly without any additional treatment during the follow-up period. Serial MR imaging was performed to monitor the untreated tumor, which in both cases involved the only hearing ear. The tumors were assessed volumetrically. The contralateral tumors appeared to enlarge mildly at initial follow-up and then, with no treatment, regressed (to 23% of the original maximum volume in Case 1 and to 15% of the original maximum in Case 2). The largest posterior fossa diameter decreased from 30.1 mm to 18.6 mm in Case 1 over 4 years and from 27 mm to 16 mm over 8 years in Case 2. Hearing declined only mildly during follow-up in both patients.

These cases demonstrate the first well-documented, long-term, spontaneous VS regressions in patients with neurofibromatosis Type 2. They underline the importance of careful observation of VS involving the only hearing ear in the management of bilateral VS to determine the natural growth pattern of the tumors. The mechanism of the dramatic spontaneous tumor regression is uncertain.

Abbreviations used in this paper: CPA = cerebellopontine angle; NF2 = neurofibromatosis Type 2; VS = vestibular schwannoma.

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Contributor Notes

Address correspondence to: Michael J. Link, M.D., Department of Neurologic Surgery, Mayo Clinic, 200 1st Street SW, Rochester, Minnesota 55905. email: link.michael@mayo.edu.
  • 1

    Abram S, , Rosenblatt P, & Holcomb S: Stereotactic radiation techniques in the treatment of acoustic schwannomas. Otolaryngol Clin North Am 40:571588, 2007

    • Search Google Scholar
    • Export Citation
  • 2

    Al Sanosi A, , Fagan PA, & Biggs NDW: Conservative management of acoustic neuroma. Skull Base 16:95100, 2006

  • 3

    Anand VT, , Kerr AG, , Byrnes DP, & Smyth GD: Non-surgical management of acoustic neuromas. Clin Otolaryngol Allied Sci 17:406410, 1992

  • 4

    Antinheimo J, , Haapasalo H, , Seppala M, , Sainio M, , Carpen O, & Jaaskelainen J: Proliferative potential of sporadic and neurofibromatosis 2-associated schwannomas as studied by MIB-1 (Ki-67) and PCNA labeling. J Neuropathol Exp Neurol 54:776782, 1995

    • Search Google Scholar
    • Export Citation
  • 5

    Bance M, & Ramsden RT: Management of neurofibromatosis type 2. Ear Nose Throat J 78:9196, 1999

  • 6

    Baser ME, , Makariou EV, & Parry DM: Predictors of vestibular schwannoma growth in patients with neurofibromatosis Type 2. J Neurosurg 96:217222, 2002

    • Search Google Scholar
    • Export Citation
  • 7

    Bederson JB, , von Ammon K, , Wichmann WW, & Yasargil MG: Conservative treatment of patients with acoustic tumors. Neurosurgery 28:646650, 1991

    • Search Google Scholar
    • Export Citation
  • 8

    Deen HG, , Ebersold MJ, , Harner SG, , Beatty CW, , Marion MS, & Wharen RE, : Conservative management of acoustic neuroma: an outcome study. Neurosurgery 39:260264, 1996

    • Search Google Scholar
    • Export Citation
  • 9

    Doherty JK, & Friedman RA: Controversies in building a management algorithm for vestibular schwannomas. Curr Opin Otolaryngol Head Neck Surg 14:305313, 2006

    • Search Google Scholar
    • Export Citation
  • 10

    Erickson BJ, , Ryan WJ, & Gehring DG: Functional requirements of a desktop clinical image display application. J Digit Imaging 14:2 Suppl 1 149152, 2001

    • Search Google Scholar
    • Export Citation
  • 11

    Evans DG, , Baser ME, , O'Reilly B, , Rowe J, , Gleeson M, & Saeed S, : Management of the patient and family with neurofibromatosis 2: a consensus conference statement. Br J Neurosurg 19:512, 2005

    • Search Google Scholar
    • Export Citation
  • 12

    Evans DG, , Huson SM, , Donnai D, , Neary W, , Blair V, & Newton V, : A clinical study of type 2 neurofibromatosis. Q J Med 84:603618, 1992

  • 13

    Gardner G, , Moretz WH Jr, , Robertson JH, , Clark C, & Shea JJ Jr: Nonsurgical management of small and intracanalicular acoustic tumors. Otolaryngol Head Neck Surg 94:328333, 1986

    • Search Google Scholar
    • Export Citation
  • 14

    House JW, & Brackmann DE: Facial nerve grading system. Otolaryngol Head Neck Surg 93:146147, 1985

  • 15

    Jorgensen BG, & Pedersen CB: Acoustic neuroma. Follow-up of 78 patients. Clin Otolaryngol Allied Sci 19:478484, 1994

  • 16

    Kawamoto Y, , Uozumi T, , Kiya K, , Fujioka Y, , Kurisu K, & Kawamoto K, : Clinicopathologic growth factors of acoustic neuromas. Surg Neurol 43:546552, 1995

    • Search Google Scholar
    • Export Citation
  • 17

    Kida Y, , Kobayashi T, , Tanaka T, & Mori Y: Radiosurgery for bilateral neurinomas associated with neurofibromatosis Type 2. Surg Neurol 53:383390, 2000

    • Search Google Scholar
    • Export Citation
  • 18

    Laasonen EM, & Troupp H: Volume growth rate of acoustic neurinomas. Neuroradiology 28:203207, 1986

  • 19

    Levo H, , Pyykkö I, & Blomstedt G: Non-surgical treatment of vestibular schwannoma patients. Acta Otolaryngol 529 Suppl 5658, 1997

  • 20

    Luetje CM: Spontaneous involution of acoustic tumors. Am J Otol 21:393398, 2000

  • 21

    Martuza RL, & Eldridge R: Neurofibromatosis 2 (bilateral acoustic neurofibromatosis). N Engl J Med 318:684688, 1988

  • 22

    Massick DD, , Welling DB, , Dodson EE, , Scholfield M, , Nagaraja HN, & Schmalbrock P, : Tumor growth and audiometric change in vestibular schwannomas managed conservatively. Laryngoscope 110:18431849, 2000

    • Search Google Scholar
    • Export Citation
  • 23

    Mathieu D, , Kondziolka D, , Flickinger JC, , Niranjan A, , Williamson R, & Martin JJ, : Stereotactic radiosurgery for vestibular schwannomas in patients with neurofibromatosis Type 2: an analysis of tumor control, complications, and hearing preservation rates. Neurosurgery 60:460470, 2007

    • Search Google Scholar
    • Export Citation
  • 24

    Mautner VF, , Baser ME, , Thakkar SD, , Feigen UM, , Friedman JM, & Kluwe L: Vestibular schwannoma growth in patients with neurofibromatosis Type 2: a longitudinal study. J Neurosurg 96:223228, 2002

    • Search Google Scholar
    • Export Citation
  • 25

    Mirz F, , Jorgensen B, , Fiirgaard B, , Lundorf E, & Pedersen CB: Investigations into the natural history of vestibular schwannomas. Clin Otolaryngol Allied Sci 24:1318, 1999

    • Search Google Scholar
    • Export Citation
  • 26

    National Institutes of Health Consensus Development Conference: Neurofibromatosis 1 (Recklinghausen disease) and neurofibromatosis 2 (bilateral acoustic neurofibromatosis). Ann Intern Med 113:3952, 1990

    • Search Google Scholar
    • Export Citation
  • 27

    Nedzelski JM, , Schessel DA, , Pfeiderer A, , Kassel EE, & Rowed DW: Conservative management of acoustic neuromas. Otolaryngol Clin North Am 25:691705, 1992

    • Search Google Scholar
    • Export Citation
  • 28

    Ogawa K, , Kanzaki J, , Ogawa S, , Yamamoto M, , Ikeda S, & Shiobara R: The growth rate of acoustic neuromas. Acta Otolaryngol 487 Suppl 157163, 1991

    • Search Google Scholar
    • Export Citation
  • 29

    Rosenberg SI: Natural history of acoustic neuromas. Laryngoscope 110:497508, 2000

  • 30

    Rowe JG, , Radatz M, , Walton L, & Kemeny AA: Stereotactic radiosurgery for Type 2 neurofibromatosis acoustic neuromas: patient selection and tumour size. Stereotact Funct Neurosurg 79:107116, 2002

    • Search Google Scholar
    • Export Citation
  • 31

    Selesnick SH, & Johnson G: Radiologic surveillance of acoustic neuromas. Am J Otol 19:846849, 1998

  • 32

    Slattery WH III, , Fisher LM, , Iqbal Z, & Oppenhiemer M: Vestibular schwannoma growth rates in neurofibromatosis type 2 natural history consortium subjects. Otol Neurotol 25:811817, 2004

    • Search Google Scholar
    • Export Citation
  • 33

    Smouha EE, , Yoo M, , Morh K, & Davis RP: Conservative management of acoustic neuroma: a meta-analysis and proposed treatment algorithm. Laryngoscope 115:450454, 2005

    • Search Google Scholar
    • Export Citation
  • 34

    Sobel RA, & Wang Y: Vestibular (acoustic) schwannomas: histologic features in neurofibromatosis 2 and in unilateral cases. J Neuropathol Exp Neurol 52:106113, 1993

    • Search Google Scholar
    • Export Citation
  • 35

    Stangerup S-E, , Caye-Thomasen P, & Thomsen J: Change in hearing during ‘wait and scan’ management of patients with vestibular schwannoma. J Laryngol Otol 122:673681, 2008

    • Search Google Scholar
    • Export Citation
  • 36

    Stangerup S-E, , Caye-Thomasen P, , Tos M, & Thomsen J: The natural history of vestibular schwannoma. Otol Neurotol 27:547552, 2006

  • 37

    Strasnick B, , Glasscock ME III, , Haynes D, , McMenomey SO, & Minor LB: The natural history of untreated acoustic neuromas. Laryngoscope 104:11151119, 1994

    • Search Google Scholar
    • Export Citation
  • 38

    Thomsen J, & Tos M: Acoustic neuroma: clinical aspects, audiovestibular assessment, diagnostic delay, and growth rate. Am J Otol 11:1219, 1990

    • Search Google Scholar
    • Export Citation
  • 39

    Vachhani JA, & Friedman WA: Radiosurgery in patients with bilateral vestibular schwannomas. Stereotact Funct Neurosurg 85:273278, 2007

  • 40

    Valvassori GE, & Shannon M: Natural history of acoustic neuromas. Skull Base Surg 1:165167, 1991

  • 41

    Wiet RJ, , Zappia JJ, , Hecht CS, & O'Connor CA: Conservative management of patients with small acoustic tumors. Laryngoscope 105:795800, 1995

    • Search Google Scholar
    • Export Citation
  • 42

    Yamamoto M, , Hagiwara S, , Ide M, , Jimbo M, , Arai Y, & Ono Y: Conservative management of acoustic neurinomas: prospective study of long-term changes in tumor volume and auditory function. Minim Invasive Neurosurg 41:8692, 1998

    • Search Google Scholar
    • Export Citation
  • 43

    Yoshimoto Y: Systemic review of the natural history of vestibular schwannoma. J Neurosurg 103:5963, 2005

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