Résumé
Objective To study the pathologic and clinical features of malignant rhabdoid tumor of the kidney(MRTK),and to improve the diagnosis and treatment of the disease.Methods The clinical and pathologic data of 5 patients(4 men and 1 woman;mean age,50 years;age range,21-67 years)with MRTK(3 tumors on the left and 2 on the right)were retrospectively analyzed in combination with review of the relevant literature.Of the 5 cases,I was incidentally diagnosed with renal tumor during physical examina- tion;and 3 had gross hematuria,low back pain and discomfort,and abdominal masses.Results Radical nephrectomy was performed in all 5 cases.The tumors averaged 6.5cm in diameter.By NWTS staging,4 ca- ses had stageⅡtumors and 1 case had stageⅢtumor.Pathological features were as follows.Rhabdoid cells were characterized by eccentric nuclei,prominent nucleoli,and abundant cytoplasm containing eosinophilic inclusions that were strongly positive for vimentin and epithelial membrane antigen(EMA).Electron micros- copy showed intermediate filaments and round,irregular fibroid or whorl-like corpuscles in the cytoplasm. Follow-up was available in 4 patients(mean,6.8 months;range,3-24 months).Of them,2 died of metasta- sis or complications 12 months after operation;and 2 were alive without recurrence and metastasis for 6 months.Conclusions MRTK is a rare and morphologically distinctive neoplasm with specific findings of pathological features.The tumor has a poorer prognosis,but comparatively it is better in adults than in adoles- cents.