磷酸盐尿性间叶肿瘤误诊分析(附7例)

Misdiagnosis analysis of phosphaturic mesenchymal tumor(7 cases)

目的:分析磷酸盐尿性间叶肿瘤(PMT)误诊原因,明确影像学检查路径。方法:回顾性分析我院2017年7月至2022年3月经病理证实PMT患者临床、影像、病理资料。结合文献复习,总结误诊原因,提出影像学检查路径。结果:7例患者3例男性,4例女性,平均年龄为(50.29±15.66)岁(范围17~69岁)。患者表现为不同程度乏力、骨痛等,均伴随骨折,病程平均(5.00±3.25)年(范围1~12年)。患者术前血磷均减低,术后血磷均升高,6例患者血磷在术后7~15天恢复正常,1例失访。6例患者术前碱性磷酸酶升高,1例正常。肿瘤最大径(2.01±1.32)cm(范围0.8~2.5 cm),2例位于软组织,5例位于骨组织。6例行X线检查,显示全身或局部骨质不同程度骨软化和伴随骨折,3例病灶无法显示。6例CT检查中5例骨组织病灶显示为高密度,1例为软组织密度结节。6例MRI平扫T1WI为低、等信号为主,偶尔见高信号,T2WI混杂高信号,内部或边缘可见低信号区。4例MRI增强检查显示病灶中度至明显强化,强化不均匀。结论:PMT影像表现缺乏特异性,容易被误诊。骨质软化、骨痛、骨折以及低血磷、高碱性磷酸酶患者,需考虑此病。可先行OCT检查发现可疑病灶,再行解剖学检查(X线、CT,MRI)对可疑致病肿瘤进一步诊断和术前定位、评估切缘和治疗随访。

Objective:To analyze the causes of misdiagnosis of phosphaturic mesenchymal tumor(PMT)and clarify imaging pathway.Methods:The clinical,imaging and pathological data of patients with PMT confirmed by pathology in our hospital from July 2017 to March 2022 were analyzed retrospectively.Combined with literature review,summary the causes of misdiagnosis and suggested imaging pathway.Results:There were 7 patients,3 males and 4 females,with an average age of(50.29±15.66)years(range 17~69).The patients showed different degrees of fatigue and bone pain,accompanied by fractures,with an average course of(5.00±3.25)years(range 1~12 years).The blood phosphorus of all patients decreased before operation and increased after operation.The blood phosphorus in 6 patients returned to normal 7~15 days after operation,and 1 case was lost to follow-up.Alkaline phosphatase increased in 6 patients before operation,and 1 case was normal.The maximum diameter of the tumor was(2.01±1.32)cm(range 0.8~2.5 cm),2 cases were located in soft tissue and 5 cases in bone tissue.X-ray examination of 6 cases showed osteomalacia of different degrees in the whole body or local area and concomitant fracture.The lesion could not be displayed in 3 cases.CT examination was performed in 6 cases,5 cases of bone tissue lesions showed high density,1 case was soft tissue density nodule.On plain MRI scans,6 cases showed low and equal signal on T1WI,and occasionally high signal.6 cases showed low and equal signal mixed high signal on T2WI,and low signal areas were seen in some parts of the interior or edges.MRI enhanced lesions in 4 cases showed moderate to obvious enhancement and uneven enhancement.Conclusion:PMT images lack specificity and are easy to be misdiagnosed.Patients with osteomalacia,bone pain,fracture,low blood phosphorus and high alkaline phosphatase should be considered.Suspicious lesions can be found by OCT first,and then anatomic examination(X-ray,CT,MRI)can be performed to further diagnose and locate the suspected pathogenic tumor before operation,evaluate the surgical margin and follow-up treatment.