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成磷酸盐性间叶性肿瘤的影像学特征。

Imaging characteristics of phosphaturic mesenchymal tumors.

机构信息

Department of Radiodiagnosis and Interventional Radiology, All India Institute of Medical Sciences, New Delhi, India.

Department of Nuclear Medicine, All India Institute of Medical Sciences, New Delhi, India.

出版信息

Acta Radiol. 2023 Jun;64(6):2061-2073. doi: 10.1177/02841851231160086. Epub 2023 Mar 13.

DOI:10.1177/02841851231160086
PMID:36915965
Abstract

BACKGROUND

Tumor-induced osteomalacia (TIO) is a rare paraneoplastic syndrome associated with phosphaturic mesenchymal tumors (PMTs). Localization of the causative tumor in these cases is an arduous task since the culprit lesions are usually small, slow-growing, and can be located almost anywhere from head to toe.

PURPOSE

To describe the morphological characteristics of histologically proven PMTs on various radiological modalities.

MATERIAL AND METHODS

After institutional ethical approval, this retrospective study analyzed 20 cases with a histopathological evidence of PMT. Various imaging characteristics of the tumors on available computed tomography (CT) and magnetic resonance imaging (MRI) scans were evaluated. Descriptive statistical analyses were conducted.

RESULTS

The tumors were located in diverse locations: lower extremities (n = 10); head and neck (n = 5); vertebral column (n = 3); pelvis (n = 1); and upper extremities (n = 1). Bone lesions seen on CT had variable morphology: sclerotic (n = 3/8, 37.5%); lytic (n = 3/8, 37.5%), and both lytic and sclerotic (n = 2/8, 25%) with presence of narrow zone of transition in all cases (n = 8/8) and amorphous internal matrix calcifications in 25% of cases (n = 2/8). Of the tumors, 68.4% (n = 13/19) were hypointense on T1 and all of them showed hyperintense signal on T2-weighted and STIR images (n = 19/19) and contrast enhancement (n = 16/16). Of the tumors, 66.7% (n = 6/9) showed restricted diffusion. DOTANOC PET/CT showed tumor uptake in all cases (n = 8/8).

CONCLUSION

PMTs may have variable and non-specific tumor appearances on various imaging modalities. However, in an appropriate clinical scenario and a background of suggestive biochemical work-up, the radiologist should keep a high index of suspicion.

摘要

背景

肿瘤相关性骨软化症(TIO)是一种罕见的副瘤综合征,与磷酸尿嘧啶基质肿瘤(PMTs)有关。在这些情况下,定位致病肿瘤是一项艰巨的任务,因为罪魁祸首病变通常很小、生长缓慢,并且可以位于从头到脚的任何地方。

目的

描述组织学证实的 PMTs 在各种放射学方式下的形态学特征。

材料与方法

在获得机构伦理批准后,本回顾性研究分析了 20 例具有 PMT 组织病理学证据的病例。评估了肿瘤在现有计算机断层扫描(CT)和磁共振成像(MRI)扫描上的各种影像学特征。进行了描述性统计分析。

结果

肿瘤位于不同部位:下肢(n=10);头颈部(n=5);脊柱(n=3);骨盆(n=1);上肢(n=1)。CT 上可见的骨病变具有不同的形态:硬化性(n=3/8,37.5%);溶骨性(n=3/8,37.5%),以及溶骨性和硬化性(n=2/8,25%),所有病例均存在狭窄的过渡带(n=8/8)和 25%的病例存在无定形内部基质钙化(n=2/8)。肿瘤中,68.4%(n=13/19)在 T1 上呈低信号,所有肿瘤在 T2 加权和 STIR 图像上均显示高信号(n=19/19)和对比增强(n=16/16)。肿瘤中,66.7%(n=6/9)显示弥散受限。DOTANOC PET/CT 在所有病例中均显示肿瘤摄取(n=8/8)。

结论

PMTs 在各种影像学方式上可能具有不同的、非特异性的肿瘤表现。然而,在适当的临床情况下和提示性生化检查背景下,放射科医生应保持高度怀疑。

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Osteomalacia in Adults: A Practical Insight for Clinicians.
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