Chiaramonte Carmela, Rabaste Sylvain, Jacquesson Timothee, Meyronet David, Cotton François, Jouanneau Emmanuel, Berhouma Moncef
Skull Base Surgery Unit, Department of Neurosurgery B, University Hospital of Lyon, Hospices Civils de Lyon, Lyon, France; Division of Neurosurgery, Department of Neurosciences and Reproductive and Odontostomatological Sciences, School of Medicine and Surgery, University of Naples Federico II, Naples, Italy.
Department of Imaging, Lyon Sud Hospital, University Hospital of Lyon, Hospices Civils de Lyon, Lyon, France.
World Neurosurg. 2018 Apr;112:18-24. doi: 10.1016/j.wneu.2018.01.012. Epub 2018 Jan 8.
Liponeurocytoma is a very rare tumor classified as grade II (neuronal and mixed neuronal-glial tumors) according to 2016 World Health Organization classification of tumors of the central nervous system. The median age at detection is 50 years, and the most frequent location is the posterior cranial fossa, especially within the cerebellar hemispheres; liponeurocytomas arising in the cerebellopontine angle (CPA) are exceptional.
Here we report the clinical, radiological, and pathological characteristics of a CPA liponeurocytoma in a 35-year-old woman, as well as a review of the literature. This unusual cisternal location raises the issue of the differential imaging diagnosis with much more common CPA tumors (e.g., meningiomas, vestibular schwannomas, ependymomas, epidermoid cyst, hemangioblastomas, medulloblastomas).
To the best of our knowledge, 59 cases of cerebellar liponeurocytomas have been reported to date, which include only 6 cases of CPA liponeurocytomas. Treatment relies on total removal whenever possible, with an excellent prognosis, but a high MIB-1 index (>10%) and/or incomplete tumor resection are the main adverse prognostic factors.
脂肪神经细胞瘤是一种非常罕见的肿瘤，根据2016年世界卫生组织中枢神经系统肿瘤分类，它被归类为II级（神经元和混合性神经元-胶质肿瘤）。其检测时的中位年龄为50岁，最常见的部位是后颅窝，尤其是小脑半球内；起源于桥小脑角（CPA）的脂肪神经细胞瘤极为罕见。
在此，我们报告一名35岁女性CPA脂肪神经细胞瘤的临床、影像学和病理特征，并对相关文献进行综述。这种不寻常的脑池部位引发了与更常见的CPA肿瘤（如脑膜瘤、前庭神经鞘瘤、室管膜瘤、表皮样囊肿、血管母细胞瘤、髓母细胞瘤）进行鉴别影像诊断的问题。
据我们所知，迄今为止已报道59例小脑脂肪神经细胞瘤，其中仅有6例CPA脂肪神经细胞瘤。治疗尽可能依靠全切，预后良好，但高MIB-1指数（>10%）和/或肿瘤切除不完全是主要的不良预后因素。
