Phosphaturic mesenchymal tumor(mixed connective tissue variant)(PMT-MCT) occurs simultaneously in clivus and temporal bone——A case report

【作者】 王琦； 张黎； 于炎冰；

【Author】 Department of neurosurgery,China-Japan Friendship Hospital;

【机构】 Department of neurosurgery,China-Japan Friendship Hospital；

【摘要】 The phosphaturic mesenchymal tumor(mixed connective tissue variant)(PMT-MCT) is a very rare tumor.It always leads to paraneoplastic syndrome known as tumor-induced osteomalacia(TIO) manifested with hypophosphatemia,muscle pain and multiple fractures.Intracranial occurrence of this tumor is extremely rare.Here we report a case of a large PMT-MCT occurs simultaneously in the clivus and temporal bone for the first time.The patients complained of bone pain throughout the body for more than 10 years and a gradually worsened hearing loss in the left ear for 3 years.Serum test revealed a low phosphate level.MRI scan demonstrated an enhanced lesion in the clivus and left temporal bone.After subtotal resection,the patient was referred to Gamma Knife radiotherapy.Histopathological sections of the tumor confirmed a diagnosis of PMTMCT.3 months following the section of the tumor,the patient’s serum phosphorus level normalized and bone pain was significantly relieved.Previous publications and our case indicate complete resection or subtotal resection followed by radiotherapy could provide good prognosis of the tumor.更多还原

【Abstract】 The phosphaturic mesenchymal tumor(mixed connective tissue variant)(PMT-MCT) is a very rare tumor.It always leads to paraneoplastic syndrome known as tumor-induced osteomalacia(TIO) manifested with hypophosphatemia,muscle pain and multiple fractures.Intracranial occurrence of this tumor is extremely rare.Here we report a case of a large PMT-MCT occurs simultaneously in the clivus and temporal bone for the first time.The patients complained of bone pain throughout the body for more than 10 years and a gradually worsened hearing loss in the left ear for 3 years.Serum test revealed a low phosphate level.MRI scan demonstrated an enhanced lesion in the clivus and left temporal bone.After subtotal resection,the patient was referred to Gamma Knife radiotherapy.Histopathological sections of the tumor confirmed a diagnosis of PMTMCT.3 months following the section of the tumor,the patient’s serum phosphorus level normalized and bone pain was significantly relieved.Previous publications and our case indicate complete resection or subtotal resection followed by radiotherapy could provide good prognosis of the tumor.更多还原