Synchronous Tumors of the Cerebellopontine Angle

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.

Keywords: Cerebellopontine angle; Collision tumor; Contiguous tumor; Meningioma; Mixed tumor; Tumor-to-tumor metastasis; Vestibular schwannoma.