Difficulty distinguishing benign notochordal cell tumor from chordoma further suggests a link between them
Difficulty distinguishing benign notochordal cell tumor from chordoma further suggests a link between them
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DOI：
10.1186/1470-7330-14-4
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发表时间：
2014-04-22
期刊：
影响因子：
4.9
通讯作者：
Vanel, Daniel
中科院分区：
文献类型：
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作者：
Kreshak, Jennifer;Larousserie, Frederique;Vanel, Daniel
背景：关于椎骨中良性脊索细胞组织的许多讨论都集中在其与脊索瘤的关系（如果有的话）的性质上。这些病变通常被称为良性脊索细胞肿瘤 (BNCT)，具有独特的形态学特征，然而，区分椎间盘中的脊索细胞、BNCT 和脊索瘤可能很困难。它们在放射学上被描述为与脊索瘤不同，具有溶解、对比度增强和表明脊索瘤的软组织肿块。方法：对 2008 年之前在我们的机构 Istituto Ortopedico Rizzoli（意大利博洛尼亚）诊断的所有脊索瘤进行了回顾，产生了 174 例病例。五个仅限于骨头；一种是复发性脊索瘤，没有可用的原始数据。其余4人重新进行详细评估。结果：3女1男，年龄33～57岁，平均48岁。两个是 BNCT，两个是包含 BNCT 和脊索瘤的混合病变。在计算机断层扫描中，所有区域都是不透射线的，并有溶解区域。 1 个 BNCT 在磁共振成像上呈异质性，对比后增强。显微镜下，一个 BNCT 具有边界清晰的囊性区域，并具有硬化边界。另一个有一个微小的非典型区域；它复发为脊索瘤。混合病变具有明确的 BNCT 区域、明确的脊索瘤区域和不符合任一标准的非典型区域。所有三个病例中的非典型区域都与脊索瘤或 BNCT 区域“混合”。结论：这些病例说明了区分 BNCT 和脊索瘤所面临的持续挑战。所有这些都具有独特的成像特征；其中三个具有与 BNCT 或脊索瘤混合的非典型微观区域，加强了关于这两个实体之间关系的论点，并支持一些 BNCT 可能进展为脊索瘤的观点。我们的研究消除了任何用于区分脊索瘤和 BNCT 的单一放射学标准都是可靠的观念，开启了关于是否或如何监测 BNCT 的讨论。
Background: Much discussion about benign notochordal cell tissue in vertebrae has centered on the nature of its relationship, if any, to chordoma. Often referred to as benign notochordal cell tumors (BNCTs), these lesions have unique morphological features, however, differentiating between notochordal cells in discs, BNCT, and chordoma can be difficult. They are described as radiologically distinct from chordoma, with lysis, contrast enhancement, and a soft tissue mass indicating chordoma.Methods: All chordomas diagnosed at our institution, the Istituto Ortopedico Rizzoli (Bologna, Italy), prior to 2008 were reviewed, yielding 174 cases. Five were limited to bone; one was a recurrent chordoma without original data available. The remaining four were re-evaluated in detail.Results: There were three women and one man, aged 33-57 years (mean, 48 years). Two were BNCTs and two were mixed lesions containing BNCT and chordoma. On computed tomography, all were radiopaque with areas of lysis. One BNCT was heterogeneous on magnetic resonance imaging, enhancing after contrast. Microscopically, one BNCT had a well-defined cystic area with a sclerotic border. The other had a minute atypical area; it recurred as chordoma. The mixed lesions had areas of definitive BNCT, definitive chordoma, and atypical areas that did not meet the criteria for either. The atypical areas in all three cases 'blended' with areas of chordoma or BNCT.Conclusion: These cases illustrate the ongoing challenges in differentiating between BNCT and chordoma. All had unique imaging features; three had atypical microscopic areas blending with BNCT or chordoma, strengthening the argument for a relationship between the two entities and supporting the idea that some BNCTs may progress to chordoma. Our study dispels the notion that any single radiologic criterion used to distinguish between chordoma and BNCT is reliable, opening the discussion as to whether or how to monitor BNCTs.