Graffeo Christopher S, Perry Avital, Copeland William R, Giannini Caterina, Neff Brian A, Driscoll Colin L W, Link Michael J
Department of Neurologic Surgery, Mayo Clinic, Rochester, Minnesota, USA.
Department of Pathology and Laboratory Science, Mayo Clinic, Rochester, Minnesota, USA.
World Neurosurg. 2017 Feb;98:632-643. doi: 10.1016/j.wneu.2016.11.002. Epub 2016 Nov 12.
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.
Retrospective chart review and systematic literature review were performed.
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.
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.
小脑脑桥角（CPA）的同步肿瘤非常罕见，且描述不一致。我们报告2例相邻的前庭神经鞘瘤（VS）和脑膜瘤病例，并对所有多发CPA肿瘤进行系统的文献综述。
进行回顾性病历审查和系统的文献综述。
一名64岁女性和一名42岁男性因CPA相关症状就诊。两名患者的磁共振成像均显示2个独立的相邻肿瘤。每例患者均行乙状窦后开颅肿瘤切除术，病理证实为VS和脑膜瘤。系统的文献综述确定了42篇先前的英文出版物，描述了46例多发CPA肿瘤患者。根据弗拉萨尼托标准，有4例伴发肿瘤（8%），16例相邻肿瘤（33%），3例碰撞肿瘤（6%），13例混合肿瘤（27%），以及11例肿瘤转移至肿瘤（23%）。16例患者肿瘤全切，16例次全切除（各占50%）。总体上，27%的患者House-Brackmann分级为III-VI级不良功能，VS和脑膜瘤患者中这一比例为33%，明显高于孤立VS的观察范围。
多发CPA肿瘤罕见，是异质性病变，明显易导致面神经预后不良，可能归因于一种旁分泌机制，该机制同时驱动多个肿瘤生长，并增加面神经-肿瘤界面的侵袭性或粘连性。先前的命名一直混淆且不一致；我们建议将所有多发CPA肿瘤归类为“同步肿瘤”，“伴脑膜上皮增生的神经鞘瘤”或“肿瘤转移至肿瘤”仅用于罕见的特定情况。
