Pathology Department, La Paz University Hospital, Paseo de la Castellana, 261, Madrid, 28046, Spain.
Radiology Department, La Paz University Hospital, Madrid, Spain.
Eur Spine J. 2024 Sep;33(9):3315-3323. doi: 10.1007/s00586-024-08419-y. Epub 2024 Jul 24.
Distinct lesions are derived from notochordal cells (NCDL), ranging from benign to malignant ones. This study presents fifty NCDL cases diagnosed in a tertiary hospital of reference from the past 55 years: forty-two conventional chordomas, including one chondroid chordoma subtype, four benign notochordal cell tumors (BNCT), two conventional chordomas with BNCT foci, and two dedifferentiated chordomas. All patients were adults. Three BNCT were incidentally diagnosed, and one case presented local pain. Chordomas began with local pain and/or neurological symptoms. BNCT were well-defined intraosseous lesions, hypointense on T1-weighted images (WI) and hyperintense on T2-WI, without enhancement in the contrast. Conventional chordomas, including its chondroid subtype, were lobulated masses with cortical disruption and soft tissue extension, hypointense on T1-WI and hyperintense on T2-WI, with variable contrast enhancement. BNCT were histologically composed of solid sheets of vacuolated cells with clear cytoplasm and round and central nuclei. No atypia, lobular growth pattern, myxoid matrix, or bone infiltration were seen. Conventional chordomas were histologically composed of physaliphorous cells in a myxoid stroma with lobulated and infiltrating growth patterns. Observational follow-up using radiological controls was decided on for the BNCT cases. None of these cases presented local recurrence or metastasis. En-bloc resection and adjuvant radiotherapy were selected for sacral and vertebral chordoma cases. Sixteen patients died due to tumor-related factors; twenty-eight presented local recurrence, and four developed distant metastases. New therapeutic options are being studied for chordoma cases. Clinical, radiological, and histopathological data are necessary to properly diagnose and follow up of NCDL.
不同的病变源自脊索瘤细胞（NCDL），范围从良性到恶性。本研究介绍了过去 55 年在一家三级参考医院诊断的 50 例 NCDL 病例：42 例常规脊索瘤，包括 1 例软骨脊索瘤亚型，4 例良性脊索瘤细胞肿瘤（BNCT），2 例常规脊索瘤伴 BNCT 灶，2 例去分化脊索瘤。所有患者均为成年人。3 例 BNCT 为偶然诊断，1 例病例出现局部疼痛。脊索瘤开始时出现局部疼痛和/或神经症状。BNCT 为界限清楚的骨内病变，T1 加权图像（WI）上呈低信号，T2-WI 上呈高信号，无对比增强。常规脊索瘤，包括其软骨亚型，是呈分叶状的肿块，伴有皮质破坏和软组织延伸，T1-WI 上呈低信号，T2-WI 上呈高信号，对比增强程度不一。BNCT 的组织学表现为实性片状空泡细胞，细胞质透明，细胞核呈圆形和中央性。未见非典型性、分叶状生长模式、黏液样基质或骨浸润。常规脊索瘤的组织学表现为黏液基质中的 physaliphorous 细胞，具有分叶状和浸润性生长模式。对 BNCT 病例决定进行影像学对照观察随访。这些病例均未出现局部复发或转移。骶骨和脊柱脊索瘤病例选择整块切除和辅助放疗。16 例患者因肿瘤相关因素死亡；28 例出现局部复发，4 例发生远处转移。目前正在研究新的治疗方法来治疗脊索瘤病例。临床、影像学和组织病理学数据对于正确诊断和随访 NCDL 是必要的。
