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[后颅窝脂肪瘤型室管膜瘤。一种罕见且鲜为人知的亚型。病例报告及文献复习]

[Lipomatous ependymomas of the posterior fossa. An infrequent and little-known subtype. A case report and review of the literature].

作者信息

Rascón-Ramírez Fernando J, Salazar-Asencio Osman A, Trondin Albert, Vargas-Jiménez Andrés C, Subhi-Issa Issa, Brin-Reyes Juan R

机构信息

Servicio de Neurocirugía, Instituto de Neurociencias, Hospital Clínico San Carlos, Madrid, España.

Servicio de Neurocirugía, Hospital Son Espases, Palma de Mallorca, España.

出版信息

Rev Esp Patol. 2022 Jul-Sep;55(3):207-211. doi: 10.1016/j.patol.2019.08.003. Epub 2019 Nov 6.

Abstract

Ependymomas are well defined glial tumours composed of uniform small cells with round nuclei in a fibrillar matrix. They have characteristic perivascular acellular areas (pseudorosettes) and, in some cases, ependymal rosettes. The three most well-known histological phenotypes are papillary, clear-cell and tanycytic. The WHO classification includes rare cases of ependymoma with lipomatous metaplasia. Lipomatous ependymomas of the posterior fossa are extremely rare; we only found 7reports of cases in adults. They usually arise in the fourth ventricle and may extend into the cerebellum, when they often show extensive vacuolization, pushing the nucleus to the periphery and giving rise to a signet-ring cell appearance. Radiologically, there are few findings characteristic of these tumours. Immunohistochemistry is essential to differentiate this subtype from other more common lesions, such as metastatic adenocarcinoma, especially from breast, intestine and kidney.

摘要

室管膜瘤是边界清晰的神经胶质瘤,由均匀的小细胞组成,细胞核呈圆形,位于纤维状基质中。它们具有特征性的血管周围无细胞区(假菊形团),在某些情况下还有室管膜菊形团。三种最著名的组织学表型是乳头状、透明细胞型和伸长细胞型。世界卫生组织(WHO)分类包括罕见的伴有脂肪化生的室管膜瘤病例。后颅窝脂肪性室管膜瘤极为罕见;我们仅发现7例成人病例报告。它们通常起源于第四脑室,可能延伸至小脑,此时常表现为广泛的空泡化,细胞核被推向周边,产生印戒样细胞外观。在放射学上,这些肿瘤几乎没有特征性表现。免疫组织化学对于将该亚型与其他更常见的病变,如转移性腺癌,尤其是来自乳腺、肠道和肾脏的转移性腺癌相鉴别至关重要。

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