成人肾母细胞瘤的临床特点

目的 探讨成人肾母细胞瘤的临床特点.方法肾母细胞瘤患者6例.男4例,女2例.年龄21～58岁,平均31岁.临床表现为腰腹痛4例,其中伴肉眼血尿1例,伴发热1例;腰腹部肿块2例.查体腹部能触及肿块者4例,有肾区叩击痛5例,下腹部压痛1例.6例尿常规检查2例红细胞(++).6例B超检查发现5.0 cm×5.0 cm～22.0 cm×25.0 cm的低回声团块,边界尚清.4例CT检查均发现密度不均、边界不清的实质性肿块,其中1例增强后肿物不均匀强化,中心有出血坏死;CT值11～40 HU,平均22.5 HU,增强后CT值35～78 HU,平均63.5 HU.2例MRI检查,提示实性占位性病变.3例IVU检查提示肾影增大,肾盏受压变细,肾盂积水1例.术前诊断肾肿瘤5例、盆腔包块1例.6例均手术治疗.根治性肾切除术5例,卵巢切除术1例,均同时行腹膜后淋巴结清扫术.术中见肿瘤侵犯胰尾1例,侵犯侧腹壁1例,侵犯下腔静脉1例;2例肾门淋巴结＞1.5 cm×1.5 cm.剖开肿物见切面灰黄或灰白色,4例包膜完整,3例肿瘤中心出血坏死.术后接受化疗加放疗4例,单纯化疗2例.结果 按美国国家肾母细胞瘤研究组(NWTS)分期:Ⅰ期1例、Ⅱ期2例、Ⅲ期2例、Ⅳ期1例.6例均顺利手术切除肿瘤.肿瘤直径4.5 cm～25.0 cm,平均11.8 cm.术后病理确诊,其中组织学分型显示分化良好型5例,分化不良型1例.有淋巴结转移者2例,局部浸润者3例,肺转移者1例(同时存在局部浸润).术后随访1～7年,平均3.5年.5例无瘤生存,1例术后2年死于肺转移. 结论 成人肾母细胞瘤患者就诊时大多已有肿瘤转移或浸润,发生血行转移者也明显多于其他肾肿瘤,早期诊断、根治性手术及术后放化疗可提高生存率.

Clinical characteristics of adult Wilms' tumor

Objective To investigate the clinical characteristics of adult Wilms'tumor in Chinese adults and to improve the recognition of adult Wilms'tumor.Methods The clinical data of 6 Wilms'tumor patients treated in our hospital were reviewed. Of them, there were 4 male patients and 2 female patients with mean age of 31 years old. Four patients with abdominal and flank pain and 2 patients with abdominal masses were the main clinical manifestations. One case accompanied with hematuria and another one had fever.Tumor masses were palpable by physical examination in 4 patients. Five patients had percussion tenderness over kidney region and 1 case had abdominal tenderness. Six cases underwent urinalysis and red blood cell was positive in 2 cases. There were no abnormal results in other laboratory tests. Chest X-ray was routinely performed before operation. Six patients underwent ultrasonography and all showed hypoechoic masses (5.0cm×5.0 cm-22.0 cm×25.0 cm) with clear margin. Four cases examined by CT and all had solid masses with inhomogeneous density and clear margin. On enhanced CT scan, 1 of 4 cases demonstrated irregular enhancement of the mass with local hemorrhagic necrosis area. The CT value ranged from 11-40 HU, with an average value of 22.5 HU, and increased to 35-78 HU, with an average value of 63.5 HU after administration of contrast. Two cases of MRI revealed solid mass defects.Three cases underwent IVU examination and showed the enlargement of renal umbra as well as thinning of renal calices caused by the compression of tumor and 1 case showed hydronephrosis.Thus, 5 cases were diagnosed with renal tumor and 1 case was diagnosed with cavitas pelvis tumor before operation. Five patients successfully underwent nephrectomies and 1 patient accepted oophorotomy with no subsequent complication.Retroperitoneal lymph node dissection(RPLND) was performed at the time of radical nephrectomy or oophorotomy in all patients. The tumor was found to be about 5-25 cm in size during operation. One of the tumors had invaded pancreas tail, one had invaded to the lateral abdominal wall and one had invaded into the inferior caval vein. Renal hilar lymph node was found to be exceeded 1.5 cm×1.5 cm in 2 patients. The post-operative incisal surface of the tumor presented with gray-yellow color. Capsule was intact in 4 patients. Three cases had hemorrhagic necrosis in tumor center. All the specimens sent for the pathological examination. None received adjuvant therapy before operation. Four patients received chemotherapy plus radiotherapy after surgery and other 2 patients received chemotherapy only. All patients were followed up with routine lab tests, ultrasonography and chest X-ray.Results Six patients were staged according to the National Wilms'Tumor Study staging system as follows: 1 case in stage Ⅰ, 2 cases in stageⅡ, 2 cases in stage Ⅲ and 1 case in stage Ⅳ. Nephrectomies or oophorotomy were performed in all patients successfully. Tumor diameters were from 4.5-25.0 cm, in average 11.8 cm.All cases had pathological diagnosis of Wilm's tumor. Five cases were classified into favorable histologic type and 1 case was in unfavorable histologic type. Two patients with lymph node metastasis and 3 patients with local invasion were observed. One patient with pulmonary metastasis was recorded. Six patients were followed up for 1-7 years. Five patients survived with no evidence of cancer recurrence. One patient died of pulmonary metastasis 2 years after surgery.Conclusions The majority of adult Wilms'tumors are found with local invasion and metastasis. The incidence of hematogenous metastasis in adult Wilms'tumor is higher than other types of renal tumor as well. The early diagnosis and radical surgical treatment and adjuvant chemoradiotherapy could improve the survival rate.