摘要目的 分析磷酸盐尿性间叶肿瘤(PMT)的临床及影像表现,以提高诊断水平.方法 回顾性分析我院2014年11月至2017年9月经病理证实的22例PMT患者,其中男12例、女10例,年龄30～72(47±11)岁.搜集临床及影像资料,临床资料包括临床表现、实验室检查结果[包括血钙、血磷、碱性磷酸酶、甲状旁腺激素及1,25-(OH)2D],影像资料包括X线、CT、MRI、核素检查结果.16例患者行SPECT检查,7例行PET/CT显像,20例行普通X线检查,18例行CT检查(CT增强5例),12例行MRI增强检查.结果 患者均有不同程度全身多部位疼痛,尤以腰背部及双下肢多见,伴有肌肉无力,病程1～15年.所有患者血磷降低、血钙正常;21例碱性磷酸酶增高;16例甲状旁腺腺激素升高;15例1,25-(OH)2D减低.13例病灶位于骨髓腔内,7例位于软组织内,2例位于鼻窦.19例患者X线平片可见不同程度骨小梁稀疏、骨质疏松、骨软化表现;15例多发假性骨折,其中骨盆合并股骨骨折4例,骨盆、股骨及腓骨单发各6、4、1例;7例多发椎体压缩骨折.13例病灶呈软组织密度,4例位于骨髓腔者呈高密度;12例患者行MRI增强检查,病灶呈T1WI低信号、T2WI压脂高低混杂信号,增强后明显强化.结论 对于血磷减低、碱性磷酸酶升高且伴有骨质软化、骨折而高度怀疑PMT的患者,可行奥曲肽等核素检查明确病灶位置,CT及MRI检查可进一步显示病灶特征,提高诊断准确性.

abstractsObjective To investigate the clinical and imaging characteristics of phosphaturic mesenchymal tumor and improve the clinical diagnosis. Methods From November 2014 to September 2017, 22 patients with pathologically confirmed diagnosis as phosphaturic mesenchymal tumor (PMT) were retrospectively analyzed, including 12 males and 10 females, age ranged from 30-72 years, mean (47 ± 11) years old. The clinical data, laboratory tests [serum calcium, phosphorus, alkaline phosphatase, parathyroid hormone and 1, 25- (OH) 2 D] and imaging examinations (X-ray, CT, MRI, nuclide) were collected and explored. Sixteen patients underwent SPECT scan and seven underwent PET/CT scan. Twenty patients had X-ray, eighteen patients had CT and 12 patients had MRI with enhancement. Results All patients suffered from diffuse pain for one to fifteen years, especially in lower back and lower extremities. All patients were found with low serum phosphorus, normal serum calcium. Twenty-one patients were found with elevated alkaline phosphatase, 16 with increased parathyroid hormone and 15 with decreased 1, 25 - (OH) 2 D. Thirteen lesions were located in the medullary cavity, seven in the soft tissue and two in the sinuses. Nineteen cases showed varying degrees of trabecular bone sparse, osteoporosis and osteomalacia on X-ray;There were 15 cases of multiple pseudo-fractures, including four cases of pelvic fracture complicated with femoral fracture, six cases of single fracture of pelvis, four cases of femur and one case of fibula. And seven cases showed multiple vertebral compression fractures. Thirteen lesions showed soft-tissue density and four in the medullary cavity showed high density on CT scan. The lesions presented low signal intensity on T1WI,high or low signal intensity on T2WI FLAIR and obviously enhanced in 12 patients who underwent MRI enhancement. Conclusion For patients with decreased serum phosphorus, elevated alkaline phosphatase, bone softening and fracture, octreotide or other nuclides should be primary imaging modality for confirming the location of the lesion. CT and MRI can further evaluate the nature of the lesion and improve diagnostic accuracy.