Download PDF
Skull Base Rep 2011; 1(2): 089-094
DOI: 10.1055/s-0031-1276722
© Thieme Medical Publishers

Ganglioneuroma of the Internal Auditory Canal Presenting as a Vestibular Schwannoma

Kimon Bekelis1 , Duncan A. Meiklejohn2 , Symeon Missios1 , Brent Harris3 , James E. Saunders2 , Kadir Erkmen1
  • 1Section of Neurosurgery, Dartmouth–Hitchcock Medical Center, Lebanon, New Hampshire
  • 2Section of Ear Nose and Throat Surgery, Dartmouth–Hitchcock Medical Center, Lebanon, New Hampshire
  • 3Section of Pathology, Dartmouth–Hitchcock Medical Center, Lebanon, New Hampshire
Further Information

Publication History

Publication Date:
11 April 2011 (online)

   Permissions and Reprints

ABSTRACT

In most series, 90% of cerebellopontine angle tumors are vestibular schwannomas. Meningiomas and epidermoid tumors follow with decreased frequency. Ganglioneuroma is a benign tumor usually found in the retroperitoneum and posterior mediastinum. We report a case of a 21-year-old man with gradual sensorineural hearing loss and a minimally enhancing lesion of the internal auditory canal, which was excised through a middle fossa approach and found histologically to be a ganglioneuroma. Like vestibular schwannomas, these lesions are benign in nature and may be managed in a similar fashion, although the possibility of malignant transformation may support surgical resection over conservative management or radiosurgery. Ganglioneuromas should be considered in patients with atypical radiographic findings for vestibular schwannomas.

KEYWORDS

Ganglioneuroma - vestibular schwannoma - internal auditory canal - cerebellopontine angle

REFERENCES

  • 1 Geoerger B, Hero B, Harms D, Grebe J, Scheidhauer K, Berthold F. Metabolic activity and clinical features of primary ganglioneuromas.  Cancer. 2001;  91 1905-1913
    CrossrefSearch in Google Scholar
  • 2 Scheithauer B W, Woodruff J M, Erlandson R A. Ganglioneuroma. In: Tumors of the Peripheral Nervous System. Bethesda, MD: Armed Forces Institute of Pathology; 1999: 259
    Search in Google Scholar
  • 3 Slattery III W H, Brackmann D E, Hitselberger W. Middle fossa approach for hearing preservation with acoustic neuromas.  Am J Otol. 1997;  18 596-601
    Search in Google Scholar
  • 4 Abe T, Asano T, Manabe T, Matsuura H, Furuta T, Taguchi K. Trigeminal ganglioneuroma.  Brain Tumor Pathol. 1999;  16 49-53
    CrossrefSearch in Google Scholar
  • 5 Brouwers F M, Eisenhofer G, Lenders J W, Pacak K. Emergencies caused by pheochromocytoma, neuroblastoma, or ganglioneuroma.  Endocrinol Metab Clin North Am. 2006;  35 699-724 viii
    CrossrefSearch in Google Scholar
  • 6 Estefano J, Algaba J, Gorostiaga F, Urruticoechea A, Hernández J M. Ganglioneuroma of the middle ear. Apropos of a case.  An Otorrinolaringol Ibero Am. 1992;  19 5-12
    Search in Google Scholar
  • 7 Kaufman M R, Rhee J S, Fliegelman L J, Costantino P D. Ganglioneuroma of the parapharyngeal space in a pediatric patient.  Otolaryngol Head Neck Surg. 2001;  124 702-704
    CrossrefSearch in Google Scholar
  • 8 Lonergan G J, Schwab C M, Suarez E S, Carlson C L. Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma: radiologic-pathologic correlation.  Radiographics. 2002;  22 911-934
    CrossrefSearch in Google Scholar
  • 9 Ozluoglu L N, Yilmaz I, Cagici C A, Bal N, Erdogan B. Ganglioneuroma of the internal auditory canal: a case report.  Audiol Neurootol. 2007;  12 160-164
    Search in Google Scholar
  • 10 Radin R, David C L, Goldfarb H, Francis I R. Adrenal and extra-adrenal retroperitoneal ganglioneuroma: imaging findings in 13 adults.  Radiology. 1997;  202 703-707
    Search in Google Scholar
  • 11 Palmer C A, Townsend J J, Harnsberger H R, Parkin J L, Apfelbaum R I. Cerebellopontine angle glioneuronal hamartoma.  Surg Neurol. 1996;  45 467-469
    CrossrefSearch in Google Scholar
  • 12 Ambros I M, Zellner A, Roald B et al.. Role of ploidy, chromosome 1p, and Schwann cells in the maturation of neuroblastoma.  N Engl J Med. 1996;  334 1505-1511
    CrossrefSearch in Google Scholar
  • 13 Miura K, Mineta H, Yokota N, Tsutsui Y. Olfactory neuroblastoma with epithelial and endocrine differentiation transformed into ganglioneuroma after chemoradiotherapy.  Pathol Int. 2001;  51 942-947
    CrossrefSearch in Google Scholar
  • 14 Ichikawa T, Ohtomo K, Araki T et al.. Ganglioneuroma: computed tomography and magnetic resonance features.  Br J Radiol. 1996;  69 114-121
    CrossrefSearch in Google Scholar
  • 15 Rha S E, Byun J Y, Jung S E, Chun H J, Lee H G, Lee J M. Neurogenic tumors in the abdomen: tumor types and imaging characteristics.  Radiographics. 2003;  23 29-43
    CrossrefSearch in Google Scholar
  • 16 Califano L, Zupi A, Mangone G M, Long F. Cervical ganglioneuroma: report of a case.  Otolaryngol Head Neck Surg. 2001;  124 115-116
    CrossrefSearch in Google Scholar
  • 17 Leonardis M, Sperb D, Alster C, Campisi C, Herter N T. Ganglioneuroma of the neck, masquerading as a goiter.  Eur J Surg Oncol. 2003;  29 929-930
    CrossrefSearch in Google Scholar
  • 18 Hillman T, Chen D A, Arriaga M A, Quigley M. Facial nerve function and hearing preservation acoustic tumor surgery: does the approach matter?.  Otolaryngol Head Neck Surg. 2010;  142 115-119
    CrossrefSearch in Google Scholar
  • 19 Irving R M, Jackler R K, Pitts L H. Hearing preservation in patients undergoing vestibular schwannoma surgery: comparison of middle fossa and retrosigmoid approaches.  J Neurosurg. 1998;  88 840-845
    CrossrefPubMedSearch in Google Scholar
  • 20 Stárek I, Mihál V, Novák Z, Pospísilová D, Vomácka J, Vokurka J. Pediatric tumors of the parapharyngeal space. Three case reports and a literature review.  Int J Pediatr Otorhinolaryngol. 2004;  68 601-606
    CrossrefSearch in Google Scholar
  • 21 Carvalho G A, Matthies C, Osorio E, Samii M. Hamartomas of the internal auditory canal: report of two cases.  Neurosurgery. 2003;  52 944-948 discussion 948-949
    CrossrefSearch in Google Scholar
  • 22 Gonul E, Yetiser S, Tasar M, Ongoru O. Glioneural hamartoma of the VIIIth nerve.  J Laryngol Otol. 2006;  120 70-73
    Search in Google Scholar
  • 23 Pucci A, Pucci E, Santini F et al.. A ganglioneuroma with features of a thyroid nodule: intense pain on fine needle biopsy as a diagnostic clue.  Thyroid. 2009;  19 201-204
    CrossrefSearch in Google Scholar

Kadir ErkmenM.D. 

Section of Neurosurgery, Dartmouth-Hitchcock Medical Center

One Medical Center Drive, Lebanon, NH 03756

Email: Kadir.Erkmen@hitchcock.org