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Imaging of spinal chordoma and benign notochordal cell tumor (BNCT) with radiologic pathologic correlation
Skeletal Radiology ( IF 2.1 ) Pub Date : 2022-09-05 , DOI: 10.1007/s00256-022-04158-7
Mark D Murphey 1, 2, 3 , Matthew J Minn 1, 3, 4 , Alejandro Luiña Contreras 5 , Kelly K Koeller 1, 6 , Robert Y Shih 1, 2, 3 , Carrie Y Inwards 7 , Takehiko Yamaguchi 8
Affiliation  

Benign notochordal cell tumor (BNCT) and chordoma are neoplasms of notochordal differentiation. BNCT represents notochordal rests, commonly an incidental lesion present in the spine in 19% of cadaveric specimens. BNCTs are often radiographically occult. CT of BNCT frequently reveals patchy sclerosis between areas of maintained underlying trabeculae. BNCT demonstrates marrow replacement on T1-weighted MR images with high signal intensity on T2-weighting. BNCTs are frequently smaller than 35 mm and lack significant enhancement, bone destruction, cortical permeation, or soft tissue components. Biopsy or surgical resection of BNCT is usually not warranted, although imaging surveillance may be indicated. Chordoma is a rare low-grade locally aggressive malignancy representing 1–4% of primary malignant bone tumors. Chordoma is most frequent between the ages of 50–60 years with a male predilection. Clinical symptoms, while nonspecific and location dependent, include back pain, numbness, myelopathy, and bowel/bladder incontinence. Unfortunately, lesions are often large at presentation owing to diagnosis delay. Imaging of chordoma shows variable mixtures of bone destruction and sclerosis, calcification (50–70% at CT) and large soft tissue components. MR imaging of chordoma reveals multilobulated areas of marrow replacement on T1-weighting and high signal intensity on T2-weighting reflecting the myxoid component within the lesion and areas of hemorrhage seen histologically. Treatment of chordoma is primarily surgical with prognosis related to resection extent. Unfortunately, complete resection is often not possible (21–75%) resulting in high local recurrence incidence (19–75%) and a 5-year survival rate of 45–86%. This article reviews and illustrates the clinical characteristics, pathologic features, imaging appearance spectrum, treatment, and prognosis of BNCT and spinal chordoma.



中文翻译:

脊柱脊索瘤和良性脊索细胞瘤 (BNCT) 的影像学与放射病理相关性

良性脊索细胞瘤(BNCT)和脊索瘤是脊索分化的肿瘤。BNCT 代表脊索休息,通常是 19% 的尸体标本中脊柱中存在的偶然损伤。BNCT 通常在放射学上是隐匿性的。BNCT 的 CT 经常显示维持的下方小梁区域之间存在斑片状硬化。BNCT 在 T1 加权 MR 图像上展示了骨髓替代,在 T2 加权上具有高信号强度。BNCT 通常小于 35 毫米,并且缺乏明显的增强、骨质破坏、皮质渗透或软组织成分。通常不需要 BNCT 的活检或手术切除,尽管可能需要进行影像学监测。脊索瘤是一种罕见的低度局部侵袭性恶性肿瘤,占原发性恶性骨肿瘤的 1-4%。脊索瘤最常见于 50-60 岁之间,男性好发。临床症状虽然非特异性且取决于位置,但包括背痛、麻木、脊髓病和肠/膀胱失禁。不幸的是,由于诊断延误,病变在就诊时往往很大。脊索瘤的成像显示骨质破坏和硬化、钙化(CT 上 50-70%)和大的软组织成分的可变混合物。脊索瘤的 MR 成像显示 T1 加权上的骨髓替代多分叶区域和 T2 加权上的高信号强度反映了组织学上观察到的病变和出血区域中的粘液样成分。脊索瘤的治疗主要是手术,预后与切除范围有关。很遗憾,完全切除通常是不可能的 (21–75%),导致局部复发率高 (19–75%),5 年生存率为 45–86%。本文就 BNCT 和脊柱脊索瘤的临床特点、病理特征、影像学表现、治疗和预后进行综述和说明。

更新日期:2022-09-05
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