國防醫學院 三軍總醫院 外科部 泌尿外科1
桃園國軍總醫院 外科部 泌尿外科
Adult Wilms’ tumor: Single institution experience and review of literatures
Yu-Hua Shau1,2, Sheng-Tang Wu1, Shou-Hung Tang1, Tai-Lung Cha1 , Chih-Wei Tsao1, En Meng1, Dah-Shyong Yu1, Guang-Huan Sun1 and Chien-Chang Kao1
1.Division of Urology, Department of Surgery, Tri-Service General Hospital, National Defense Medical Center, Taipei, Taiwan, R.O.C.
2. Division of Urology, Department of surgery, Taoyuan armed forces general hospital , Taiwan
Wilms’ tumor (nephroblastoma) is the most common solid primary renal malignancy in children but extremely rare in adult population. We present single institution experience of adult Wilms’ tumor in order to assess the clinical characteristics, radiological features, and surgical prognosis.
A retrospective review was carried out to investigate the patients with adult Wilms’ tumor at the Tri-Service General Hospital during a 45-year period (1970 - 2014). Seven patients ≧ 16 years old (one female and six male; median age at the time of diagnosis of 24 years; range 24 - 48 years) were staged according to the National Wilms' Tumor Study (NWTS) staging system as follows: 3 patients had stage II disease, 2 patients had stage III disease and 2 patients had stage IV disease
The most common presentation is flank pain (57%) followed by palpable mass ( 43 %). Only one patient presented with gross hematuria. Four patients underwent renal arteriography which all disclosed irregular, tortuous vessel pattern with faint tumor stain. All patients underwent radical nephrectomy (one hand-assisted laparoscopic nephrectomy and 6 open transperitoneal nephrectomy). Two patients developed lung metastases after radical surgery. All but one patient received multimodal therapy, including surgery, chemotherapy and radiation therapy. The mean disease-free survival is 81 months. Three-year and five-year survival rate is 60 % and 50 % separately.
Selective renal arteriography provides valuable diagnostic hint in adult Wilms’ tumor. Using multimodality therapy, improved prognosis could be achieved.