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第三脑室底部管状憩室样肿瘤:提示颅咽管瘤的形态学特征。

Duct-like diverticulum at the base of third ventricle tumors: a morphological signature diagnostic of papillary craniopharyngioma.

机构信息

Department of Neurosurgery, La Princesa University Hospital, C/ Diego de León 62, 28006, Madrid, Spain.

Department of Neurosurgery, Puerta de Hierro University Hospital, Madrid, Spain.

出版信息

Neurosurg Rev. 2022 Oct;45(5):3361-3379. doi: 10.1007/s10143-022-01848-7. Epub 2022 Aug 19.

Abstract

This study describes and characterizes a narrow, hollow tubular structure, termed as duct-like diverticulum (DV), found specifically at the basal midline of papillary craniopharyngiomas (PCPs) located within the third ventricle (3V). The presence of this structure was systematically investigated on autopsy studies and magnetic resonance imaging (MRI) scans of 3536 craniopharyngioma (CP) cases published in the medical literature from 1911 to 2021, as well as in other twelve 3V tumor categories (n = 1470 cases). A basal DV was observed in a total of 50 PCPs, including two of our own cases. This DV corresponds to a tubular-shaped recess invaginated at the midline bottom of the tumor, following the same angled trajectory as the pituitary stalk. It can be easily seen as a hypointense linear structure on T1- and T2-weighted MRI scans, with two main length types: long DVs (74%), which reach the tumor center, and short DVs (26%), which penetrate the tumor only a few millimeters. The DV sign identifies the papillary CP type with a specificity of 100% and a sensitivity of 33% in the overall CP population. This finding also serves to establish the strictly intra-3V location of the lesion with a 95% specificity and 42% sensitivity among papillary CPs. No similar basal DV was found in adamantinomatous CPs nor among other categories of strictly 3V tumors. Consequently, the presence of a diverticulum in a 3V tumor represents a morphological signature pathognomonic of the papillary type and a valuable sign to reliably define the strictly 3V topography.

摘要

本研究描述并分析了一种狭窄的中空管状结构,称为管状憩室(DV),它专门存在于位于第三脑室(3V)内的颅咽管瘤(PCP)的基底中线处。在 1911 年至 2021 年发表在医学文献中的 3536 例颅咽管瘤(CP)病例的尸检研究和磁共振成像(MRI)扫描以及其他 12 种 3V 肿瘤类别(n=1470 例)中,系统地研究了这种结构的存在。在总共 50 例 PCP 中观察到基底 DV,其中包括我们自己的两例病例。该 DV 对应于肿瘤中线底部的管状凹陷,与垂体柄的相同角度轨迹一致。它可以很容易地在 T1 和 T2 加权 MRI 扫描上被视为低信号线性结构,有两种主要的长度类型:长 DV(74%),可以到达肿瘤中心,以及短 DV(26%),仅穿透肿瘤几毫米。DV 征在总体 CP 人群中对乳头状 CP 型具有 100%的特异性和 33%的敏感性。这一发现还用于确定病变的严格 3V 位置,在乳头状 CP 中具有 95%的特异性和 42%的敏感性。在造釉细胞瘤性 CP 中或其他严格的 3V 肿瘤类别中均未发现类似的基底 DV。因此,3V 肿瘤中憩室的存在代表了乳头状型的形态学特征,是可靠定义严格 3V topography 的有价值的标志。

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