磷酸盐尿性间叶性肿瘤影像学表现

Imaging Findings of Phosphaturic Mesenchymal Tumor

目的分析磷酸盐尿性间叶性肿瘤的多模态影像学表现。资料与方法回顾性收集2012年11月—2022年6月华中科技大学同济医学院附属同济医院经病理及分子影像证实的磷酸盐尿性间叶性肿瘤10例,分析4例CT、7例MRI及10例核医学影像特征和临床特点。结果10例患者主要临床症状:疼痛8例,无力4例,伴多发骨折9例。实验室检查:10例血磷降低,6例血25-羟维生素D降低,10例血碱性磷酸酶升高。CT平扫混杂密度4例,MRI平扫混杂信号6例,钙化多见,部分伴囊变及脂肪成分;增强后7例呈中度到明显不均匀强化。8例99Tcm-MDP SPECT全身骨显像示多发骨及关节摄取增高,8例18FFDG PET/CT示病灶代谢无增高或轻度增高,10例68Ga-DOTATATE PET/CT均表现为病灶部位明显高摄取。结论磷酸盐尿性间叶性肿瘤患者以疼痛、骨折及低磷血症为典型临床表现,影像学特征为病灶小且隐匿、成分复杂,68Ga-DOTATATE PET/CT显像对检出肿瘤高度敏感。

Purpose To analyze the multimodal imaging manifestations of phosphaturic mesenchymal tumor.Materials and Methods A retrospective analysis was performed on ten patients with phosphaturic mesenchymal tumor confirmed by pathology and molecular imaging from November 2012 to June 2022 in Tongji Hospital,Tongji Medical College,Huazhong University of Science and Technology.The imaging features of CT in four cases,of MRI in seven cases,and of nuclear medicine in ten cases were reviewed,along with the clinical characteristics of all patients.Results The main clinical symptoms of ten patients,eight cases had pain,four cases had weakness,and nine cases had multiple fractures.Laboratory tests results:ten cases showed decreased blood phosphorus,six cases showed decreased blood 25-hydroxyvitamin D and ten cases showed elevated blood alkaline phosphatase.The lesions were heterogeneous in four cases on CT scans and in six cases on MRI scans.Calcification was common,and some had cysts and fatty components.After enhancement,seven cases showed moderate to obvious uneven enhancement.99Tcm-MDP SPECT showed increased uptake of multiple bones and joints in eight cases,18F-FDG PET/CT showed no or slight increase in lesion metabolism in eight cases,68Ga-DOTATATE PET/CT showed significantly high uptake at the lesion site in ten cases.Conclusion Phosphaturic mesenchymal tumor patients typically present with pain,fractures and hypophosphatemia.The conventional imaging features are characterized by small and concealed lesions and complex components.Particularly,68Ga-DOTATATE PET/CT imaging exhibits high sensitivity for the tumor detection.