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首页> 外文期刊>Neuropathology: official journal of the Japanese Society of Neuropathology >Multiple neuroenteric cysts at cerebello-pontine angle and foramen magnum: a case report and review of the literature.
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Multiple neuroenteric cysts at cerebello-pontine angle and foramen magnum: a case report and review of the literature.

机译：小脑桥桥角和大孔眼多个神经肠囊肿：1例病例并文献复习。

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摘要

Neuroenteric cysts of the CNS are uncommon benign lesions usually involving the spinal cord or rarely the cerebellopontine angle (CPA). We report a rare example of multiple neuroenteric cysts arising from the CPA and foramen magnum in a 20-year-old Caucasian woman who presented with headaches and dizziness. An MRI showed three separate lesions, not communicating with each other. The first lesion, within the left posterior lateral aspect of the CPA, demonstrated isointensity to gray matter on the fluid-attenuated inversion recovery (FLAIR) sequence. The second lesion, within the left foramen of Luschka at the level of the CPA, demonstrated hyperintensity on the T(2)-weighted sequences, intermediate to slightly hyperintense on T(1)-weighted sequence and hyperintensity on FLAIR. The third lesion, within the anterior/inferior left cerebellum at the level of the foramen magnum, followed CSF signal intensity throughout. None of the lesions demonstrated significant enhancement or bone lesions. Due to compression effect, surgery was performed. Pathologic examination revealed cystic structures lined by a single layer of non-ciliated well-differentiated mucin-producing columnar epithelium with eosinophilic to amphophilic cytoplasm and round to oval nuclei with focal pseudostratification. Immunohistochemical studies showed focal positivity for cytokeratin 7, CK 5/6, synaptophysin, and carcinoembryonic antigen (CEA), diffuse positive staining for epithelial membrane antigen (EMA) and BerEP4; and negative staining for cytokeratin 20, TTF-1, and GFAP. The MIB-1 proliferation index was < 1%. One-year follow-up has shown no recurrence. The differential diagnosis and a brief review of the literature are also presented.
机译：中枢神经系统的神经肠囊肿是罕见的良性病变，通常累及脊髓或很少见小脑桥脑角（CPA）。我们报告了一个罕见的例子，在一名20岁的白人妇女中，由于CPA和大孔气孔而引起的多发性神经肠囊肿，出现头痛和头晕。 MRI显示三个独立的病变，彼此不连通。在CPA的左后外侧区域内，第一个病变在液体减量反转恢复（FLAIR）序列上显示出对灰质的等强度。第二个病变位于Luschka的左眼孔内，处于CPA水平，在T（2）加权序列上表现出高强度，在T（1）加权序列上表现为轻度高强度，而在FLAIR上表现为高强度。在大孔前水平的左小脑前/左小脑内的第三个病变，始终遵循CSF信号强度。没有一个病变显示出明显的增强或骨病变。由于压缩作用，进行了手术。病理检查显示，囊性结构由单层非纤毛的高分化产粘蛋白的柱状上皮衬着，嗜酸性至两亲性细胞质，圆形至椭圆形核具局灶性伪分层。免疫组织化学研究显示，细胞角蛋白7，CK 5/6，突触素和癌胚抗原（CEA）呈局部阳性，上皮膜抗原（EMA）和BerEP4呈弥漫阳性。以及细胞角蛋白20，TTF-1和GFAP的阴性染色。 MIB-1增殖指数＜1％。一年的随访未发现复发。还介绍了鉴别诊断和文献综述。

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