泌尿系统肉瘤样癌和癌肉瘤八例报告并文献复习

目的 探讨泌尿系统肉瘤样癌和癌肉瘤的组织学特点、临床表现、治疗和预后.方法 泌尿系统肉瘤样癌和癌肉瘤8例.其中膀胱肉瘤样癌4例,男3例,女1例,年龄58、63、78、79岁,均因无痛性肉眼血尿就诊,肿瘤直径平均4.5(1.5～6.7)cm,为不规则、广基的浸润性肿物.行膀胱部分切除术2例,TURBt 1例,膀胱癌根治术1例.肾肉瘤样癌1例,男,64岁,因肉眼血尿就诊,伴同侧肾上腺和胰腺转移,行肾癌根治术和胰腺体尾切除术.转移性右肾上腺肉瘤样癌1例,男,47岁,原发灶为左肺肉瘤样癌,行左肺下叶和右肾上腺切除术.肾盂癌肉瘤1例,女,64岁,表现为无痛性肉眼血尿,行左肾、输尿管及部分膀胱切除术.膀胱癌肉瘤1例,男,77岁,无痛性肉眼血尿,肿瘤直径2.5～3.0 cm,行TURBt术.8例均经病理检查确诊.8例均获随访,随访时间36～96个月.结果 膀胱肉瘤样癌4例中:1例G_3、T_(2a)者行TURBt,术后行丝裂霉素膀胱灌注化疗,随访96个月无瘤生存;1例63岁女性先行TURBt,术后病理为肉瘤样癌、T_1伴有原位癌(T_m),1个月后行膀胱癌根治切除术.随访36个月无瘤生存;1例T3a者行膀胱部分切除术,术后辅以放疗,随访36个月无瘤生存;1例T4a者行姑息性膀胱部分切除术,术后病理切缘阳性,术后2个月死于肿瘤多脏器转移.肾肉瘤样癌患者术后2个月开始化疗(盐酸吉西他滨加卡铂),又出现肝、肺转移,术后5个月死亡.转移性右肾上腺肉瘤样癌患者术后在外院行3个疗程化疗(顺铂加依托泊苷),5个月后出现左肾上腺转移,术后7个月死亡.肾盂癌肉瘤患者的肿瘤以软骨肉瘤为主,伴少许鳞状细胞癌及未分化癌,随访60个月无瘤生存.膀胱癌肉瘤患者肿瘤病理分期为T2a,主要是平滑肌肉瘤,伴有鳞状细胞痛和腺癌,术后予以全身化疗(盐酸吉西他滨加卡铂),随访12个月死于慢性阻塞性肺气肿、肺部感染.结论 泌尿系统肉瘤样癌和癌肉瘤少见,多见于老年男性,恶性程度高,浸润性强,预后差.肿瘤的病理分期和治疗是影响预后的因素,早期诊断和积极的综合治疗可望获得较好的治疗结果、延长患者生存期.

Urinary system sarcomatoid carcinoma and carcinosarcoma: report of 8 cases

Objective To investigate the histological features.clinical manifestations,treatment options and prognosis of urinary system sarcomatoid carcinoma and carcinosarcoma. Methods Pa- thological and clinical records of 8 patients with urinary systemic sareomatoid carcinoma and carcino- sarcoma admitted from April 200 1 to May 2006 were reviewed.Four patients were with bladder sarco- matoid carcinoma.They were 3 males and 1 female with age ranged 58 to 79 years and mean tumor size of 4.5 cm(range 1.5 to 6.7 cm).The tumor consisted of an interlaced spindle cell malignancy with coexistent of the higher grade urothelial carcinoma.One patient was kidney sarcomatoid carcino- ma with adrenal gland and pancrease metastasis.He was a male,64 years and underwent radical ne- phrectomy and partial panereectomy.One patient was with metastatic adrenal sarcomatoid carcinoma from lung.He was a male,47 years and underwent adrenalectomy and pneumonectomy.One patient was with renal pelvic earcinosarcoma.She was a female,64 years and underwent left nephrectomy, ureterectomy and partial cystectomy.One patient was with bladder carcinosarcoma.He was a male. 77 years and received TURBt and systemic chemotherapy.All the patients underwent operation and were followed up(from 36 to 96 months). Results Follow-up of 4 bladder sarcomatoid carcinoma patients revealed that 1 patient(T2.,TURBt+intravesical chemotherapy)was alive with tumor-free at 96 months;1 patient(T1+T18,radical cystectomy)survived 36 months with tumor-free;1 patient (T3b.partial cystectomy+radiation)survived 36 months with tumor-free;1 patient(T4.,partial cystectomy)died of the tumor at 2 months after surgery.The kidney sarcomatoid carcinoma and me- tastasis of adrenal gland and pancreas patient died of the tumor at 5 months after surgery even he re- ceived one cycle chemotherapy after surgery.The metastatic adrenal sarcomatoid carcinoma from lung patient died of the tumor at 7 months after surgery even he accepted three cycle chemotherapy after- wards.The renal pelvic careinosarcoma patient was followed up for 60 months and showed that this patient was alive with tumor-free.The bladder carcinosarcoma patient received TURBt and systemic chemotherapy.He died of obstructive emphysema and pneumonia at 1 2 months after surgery. Con- clusions The urinary systemic sarcomatoid carcinoma and carcinosarcoma are rare neoplasms,which usually happen on old males.The tumors are highly aggressive malignancies with poor prognosis.The tumor stage and treatment are predictive factors of survival.The early diagnosis and aggressive treat- ment may obtain better effect and prolonged survival.