Huang Wenpeng, Qiu Yongkang, Chen Zhao, Yang Qi, Nong Lin, Kang Lei
Department of Nuclear Medicine, Peking University First Hospital Beijing 100034, China.
Department of Pathology, Peking University First Hospital Beijing 100034, China.
Am J Nucl Med Mol Imaging. 2023 Jun 25;13(3):118-125. eCollection 2023.
Erdheim-Chester disease (ECD) is a rare and clinically heterogeneous non-Langerhans cell histiocytosis, and its diagnosis relies on established clinical, radiologic, histopathological criteria. ECD can be evaluated by whole-body preoperative imaging methods. Although F-FDG PET/CT shows negative findings in some splenic benign or borderline lesions, such as splenic inflammatory myofibroblastic tumors and hemangioendotheliomas, it can provide value in differentiating some malignant diseases, such as hemangiosarcoma and metastases. Here, we report the CT, MRI, and F-FDG PET/CT imaging performance of an ECD patient who presented with only spleen involvement. Even though some clinical and radiological descriptions can be found in the literature, ECD reports with only splenic involvement mimicking splenic hemangioma as the first presentation are rare, to the best of our knowledge. Histopathology and molecular analysis of this case confirmed the diagnosis of ECD. Clinicians should pay attention to the possibility of ECD occurrence in the spleen, while negative findings on F-FDG PET/CT of the spleen indicated a low risk for high-grade malignant splenic tumors and metastases.
厄尔德海姆-切斯特病（ECD）是一种罕见的、临床异质性的非朗格汉斯细胞组织细胞增多症，其诊断依赖于既定的临床、放射学和组织病理学标准。ECD可通过全身术前成像方法进行评估。尽管F-FDG PET/CT在一些脾脏良性或交界性病变（如脾脏炎性肌纤维母细胞瘤和血管内皮瘤）中显示阴性结果，但它在鉴别一些恶性疾病（如血管肉瘤和转移瘤）方面具有价值。在此，我们报告一例仅累及脾脏的ECD患者的CT、MRI及F-FDG PET/CT成像表现。据我们所知，尽管文献中可找到一些临床和放射学描述，但以仅累及脾脏且表现类似脾血管瘤为首发症状的ECD报道罕见。该病例的组织病理学和分子分析确诊为ECD。临床医生应注意脾脏发生ECD的可能性，而脾脏F-FDG PET/CT的阴性结果表明高级别恶性脾脏肿瘤和转移瘤的风险较低。
