Imaging and Clinical Characteristics of Malignant Rhabdoid Tumors of Kidney in Children
Objective To investigate the imaging and clinical features of malignant rhabdoid tumor of the kidney(MRTK)in children.Methods The clinical data of 9 children with MRTK in Children s Hospital Affiliated to Zhengzhou University were collected,all of which were examined by CT,8 by ultrasound and 3 by MRI before operation.The imaging and clinical features were summarized.Results There were 4 cases of male and 5 cases of female with a median age of 6 months and 5 days,all of them had a single tumor,increased lactate dehydrogenase(LDH),stage Ⅱ-Ⅳ,6 cases in the left kidney and 3 cases in the right kidney,and were hospitalized with symptoms such as abdominal distension and hematuria.Preoperative ultrasonography revealed a cystic or solid mass with central invasive medullary growth and unclear boundary with the residual kidney.There were 6 cases of renal hilar invasion and 1 case with venous tumor thrombus.CT plain scan density was uneven,2 cases had cystic solid boundary transition,2 cases with calcification.The degree of enhancement was less than that of the peripheral residual kidney.Six cases involved the renal pelvis and 4 cases were accompanied by intratumoral hemorrhage and subcapsular fluid.MRI signals were mixed and solid partial diffusion was limited.Seven cases were accompanied by metastasis before surgery,and 1 case was accompanied by intracranial tumor.The pathological tissue was composed of large nucleolus,cytoplasmic rich striated muscle cells and eosinophilic cells.Immunohistochemical Vimentin(+)in 9 cases,EMA(+)in 4 cases,CK(+)in 4 cases,and INI-1(-)in 6 cases.Among the 6 children with surgery and chemotherapy,4 patients had recurrence and metastasis,2 patients with chemotherapy had no reduction of the mass and new lymph node metastasis,1 patient was lost to follow-up,and 1 child gave up treatment and died.Conclusion MRTK has high malignant degree,easy metastasis and poor prognosis.Different imaging methods have their advantages and disadvantages.The imaging findings of MRTK are cystic or solid primary mass,invasive growth,unclear boundary with the residual kidney,gradual transition of cystic solid boundary and subcapsular renal effusion with certain characteristics.
万方数据
维普
