da Costa Marcos Devanir Silva, Hoshino Karina, Stavale João Norberto, Lanzoni Oreste Paulo, Cavalheiro Sergio, Paiva Neto Manoel Antonio
Department of Neurosurgery, Federal University of Sao Paulo, Sao Paulo-SP, Brazil.
Department of Neurosurgery, Federal University of Sao Paulo, Sao Paulo-SP, Brazil.
World Neurosurg. 2017 Apr;100:713.e5-713.e8. doi: 10.1016/j.wneu.2017.01.013. Epub 2017 Jan 16.
Neurofibromas are benign nerve sheath tumors that usually affect peripheral nerves and are related to neurofibromatosis type 1; however, they have not been described as a cause of intraparenchymal brain tumor.
We report a case of intracranial myxoid neurofibroma in a 19-year-old female patient manifested as an intense and progressive cephalea, followed by nausea, vomiting, photophobia, and phonophobia. Computed tomography and magnetic resonance imaging showed an extant, expansive left frontoparietal parafalcine/parasagittal tumor. Histopathologic examination determined S-100 protein and CD34 positivity, as well as sparse expression of Ki67 protein, and indicated Schwann cells with characteristic wavy nuclei and intraneural fibroblasts in a myxoid background. Together, these observations characterized the tumor as myxoid neurofibroma. The tumor was excised, and the patient recovered without deficits and with no signs of recurrence after 6 years of follow-up.
This is a novel presentation of a myxoid neurofibroma. The tumorigenesis mechanisms are likely complex and possibly involve the differentiation of Schwann cells present in adrenergic autonomic nerves in the subarachnoid arterial branches or in trigeminal nerves present in the meningeal convexity.
神经纤维瘤是一种良性神经鞘瘤，通常累及周围神经，与1型神经纤维瘤病相关；然而，它们尚未被描述为脑实质内脑肿瘤的病因。
我们报告一例19岁女性患者的颅内黏液样神经纤维瘤，表现为剧烈且进行性的头痛，随后出现恶心、呕吐、畏光和畏声。计算机断层扫描和磁共振成像显示存在一个膨胀性的左额顶叶镰旁/矢状窦旁肿瘤。组织病理学检查确定S-100蛋白和CD34呈阳性，Ki67蛋白表达稀疏，并显示在黏液样背景中有具有特征性波浪状核的施万细胞和神经内成纤维细胞。综合这些观察结果，该肿瘤被诊断为黏液样神经纤维瘤。肿瘤被切除，患者恢复良好，无功能缺损，随访6年后无复发迹象。
这是黏液样神经纤维瘤的一种新表现形式。其肿瘤发生机制可能很复杂，可能涉及蛛网膜下动脉分支中肾上腺素能自主神经或脑膜凸面三叉神经中存在的施万细胞的分化。
