Malignant rhabdoid tumor of the kidney in adults: a clinical and pathologic study
- VernacularTitle:成人肾恶性横纹肌样瘤临床与病理学特点分析
- Author:
Yi WANG
;
Guang SUN
;
Tao LI
;
Jian-Gang PAN
;
Shu-Min ZHANG
;
Zhan-Jun GUO
;
Hai-Tao NIU
;
Yi-bing ZHANG
- Publication Type:Journal Article
- Keywords:
Kidney neoplasms;
Carcinoma;
Malignant rhabdoid tumor;
Pathology;
Prognosis
- From:
Chinese Journal of Urology
2000;0(12):-
- CountryChina
- Language:Chinese
-
Abstract:
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.
