磷酸盐尿性间叶性肿瘤影像学表现及文献回顾  

Imaging manifestations and literature review of phosphateuria mesenchymal tumors

作　　者：岳梦情 张家雄[1] YUE Meng-qing;ZHANG Jia-xiong(Department of Radiology,Foshan Hospital of Traditional Chinese Medicine,Guangdong 528000,China)

机构地区：[1]佛山市中医院放射科,广东528000

出　　处：《放射学实践》2022年第7期874-879,共6页Radiologic Practice

摘　　要：目的:探讨磷酸盐尿性间叶性肿瘤(PMT)的临床和影像学特征,提高对该病的认识。方法:分析本院4例经手术病理证实的PMT的临床表现、实验室检查、影像学表现和病理结果。结果:本文4例PMT中,男3例,女1例;年龄38~56岁。原发瘤部位:臀部软组织1例,肱骨1例,胫骨1例,颅内1例。原发瘤影像除颅内肿瘤征象明确之外,其他部位肿瘤影像征象隐蔽。伴全身骨质疏松软化3例,且均伴多发性陈旧性骨折;另1例颅内肿瘤患者,无明显骨质疏松软化征象。4例中3例血磷降低,1例血磷正常;4例患者血钙均正常;3例碱性磷酸酶升高,1例碱性磷酸酶正常;4例患者均行肿瘤手术切除,术后病理3例为PMT;1例为右臀部肿物为纤维硬化性脂肪瘤(纤维瘤样脂肪瘤)。3例低血磷患者原发肿瘤切术后,血磷逐渐恢复正常。结论:磷酸盐尿性间叶性肿瘤多数有典型的临床和影像学特征,如成年人发生不明原因骨质疏松、骨质软化及低血磷时,应该考虑到该病可能性,需做全身影像学检查以查找原发瘤。Objective:To investigate the clinical and imaging features of phosphateuria mesenchymal tumors in order to improve the understanding of the disease.Methods:The clinical manifestations,laboratory examinations,imaging manifestations and pathological results of 4 cases of phosphateuria mesenchymal tumors(PMT)confirmed by surgery and pathology in our hospital were analyzed.Results:Among the 4 cases in this article,3 were male and 1 was female,aged 38~56 years.Primary tumor location:1 case in the soft tissue of the buttock,1 case in the humerus,1 case in the tibia,1 case in the brain;the imaging of the primary tumor was clear except for the clear signs of intracranial tumors,but the imaging signs of other tumors were hidden.There were 3 cases with systemic osteoporosis and multiple old fractures.Another patient with intracranial tumor had no obvious signs of osteoporosis.Among the 4 cases,blood phosphorus was decreased in 3 cases and normal in 1 case;blood calcuim was normal in 4 all cases;alkaline phosphatase was increased in 3 cases and normal in 1 case;4 cases underwent surgical resection of tumors.Pathological results showed 3 cases were phosphateuria mesenchymal tumors(PMT);1 case was fibrosclerosing lipoma(fibromatous lipoma)on the right hip.After resection of the primary tumor in 3 patients with low blood phosphorus,blood phosphorus gradually returned to normal.Conclusion:Phosphateuriic mesenchymal tumors(PMT)mostly have typical clinical and imaging features.For example,in adults with unexplained osteoporosis,osteomalacia,and hypophosphatemia,phosphateuria mesenchymal tumors should be considered,whole-body imaging examinations are suggested to find the primary tumor.

关 键 词：骨软化 骨质疏松 磷酸盐尿性间叶性肿瘤 肿瘤 低血磷 

分 类 号：R814.42[医药卫生—影像医学与核医学] R589[医药卫生—放射医学]