Synchronous Tumors of the Cerebellopontine Angle

Christopher S. Graffeo, Avital Perry, William R. Copeland, Caterina Giannini, Brian A. Neff, Colin L.W. Driscoll, Michael J. Link

Research output: Contribution to journalArticlepeer-review

5 Scopus citations

Abstract

Background Synchronous tumors of the cerebellopontine angle (CPA) are very rare and inconsistently described. We present 2 cases of contiguous vestibular schwannoma (VS) and meningioma and a systematic literature review of all multiple CPA tumors. Methods Retrospective chart review and systematic literature review were performed. Results A 64-year-old woman and a 42-year-old man presented with symptoms referable to the CPA. Magnetic resonance imaging in both patients revealed 2 separate contiguous tumors. Retrosigmoid craniotomy and tumor removal in each case confirmed VS and meningioma. Systematic literature review identified 42 previous English-language publications describing 46 patients with multiple CPA tumors. Based on Frassanito criteria, there were 4 concomitant tumors (8%), 16 contiguous tumors (33%), 3 collision tumors (6%), 13 mixed tumors (27%), and 11 tumor-to-tumor metastases (23%). Extent of resection was gross total in 16 cases and subtotal in 16 cases (50% each). Unfavorable House-Brackmann grade III–VI function was documented in 27% overall and in 33% of patients with VS and meningioma, a marked increase from the observed range in isolated VS. Conclusions Multiple CPA tumors are rare, heterogeneous lesions with a marked predisposition toward poor facial nerve outcomes, potentially attributable to a paracrine mechanism that simultaneously drives multiple tumor growth and increases invasiveness or adhesiveness at the facial nerve–tumor interface. Preceding nomenclature has been confounding and inconsistent; we recommend classifying all multiple CPA tumors as “synchronous tumors,” with “schwannoma with meningothelial hyperplasia” or “tumor-to-tumor metastases” reserved for rare, specific circumstances.

Original languageEnglish (US)
Pages (from-to)632-643
Number of pages12
JournalWorld neurosurgery
Volume98
DOIs
StatePublished - Feb 1 2017

Keywords

  • Cerebellopontine angle
  • Collision tumor
  • Contiguous tumor
  • Meningioma
  • Mixed tumor
  • Tumor-to-tumor metastasis
  • Vestibular schwannoma

ASJC Scopus subject areas

  • Surgery
  • Clinical Neurology

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