原发性膀胱小细胞癌诊治体会

目的 探讨膀胱小细胞癌的临床及病理特点.方法 回顾性分析9例膀胱小细胞癌的基本资料.男6例,女3例.年龄45～79岁,平均62岁.临床表现为肉眼血尿7例,排尿困难及下腹部疼痛2例.肿瘤直径0.5～7.0 cm,平均2.0 cm;多发2例,单发5例,全膀胱弥漫性生长2例.7例行尿脱落细胞学检查,阳性4例.9例均行手术治疗,其中4例术前诊断为浅表性者行TURBt,术后均定期行吡柔比星膀胱灌注,1例化疗3个周期;膀胱部分切除2例,均定期行吡柔比星膀胱灌注,1例化疗2个周期;根治性膀胱全切3例,静脉化疗2例.结果 术后病理检查:肿瘤细胞体积小,呈圆形,胞质稀少,核浓染,缺乏巢状结构.免疫组化染色:嗜铬素A(+)、神经元特异性烯醇化酶(+).诊断为小细胞癌,其中1例含移行细胞癌成分,1例合并前列腺癌.1例术前检查显示高血钙(3.15 mmol/L)和低血磷(0.61 mmol/L),术后1个月血钙及血磷恢复正常.保留膀胱者随访4例,3例分别于术后4、9、25个月死于转移,1例术后化疗者随访24个月未见复发及转移.膀胱全切3例中2例分别于术后2、28个月死亡,1例术后随访32个月未见肿瘤复发及转移.结论膀胱小细胞癌恶性程度高,预后差,根治性膀胱全切加全身化疗是主要的治疗方法,保留膀胱的手术应配合全身化疗.决定预后的是肿瘤的临床分期及治疗方法.

Abstract：

Objective To investigate the clinical and pathological features of small cell carcinoma of the urinary bladder. Methods The pathological and clinical data of 9 cases of small cell carcinoma were analyzed retrospectively. There were 6 males and 3 females, ages 45 to 79 years (mean age, 62 years). Clinical manifestations of 7 cases included gross hematuria and dysuria, the other 2 cases experienced lower abdominal pain. The mean tumor size was 2.0 cm (ranged, 0.5 to 7.0 cm). Two cases had multiple tumors and 5 cases had single tumors. The growth pattern in 2 cases was diffuse growth in the whole bladder. In 4 cases tumor cells were found in urine cytology. All 9 patients underwent surgical treatment, including TURBt. Four patients were diagnosed as superficial tumors before operation. All the patients underwent regular theprubicine irrigation in the bladder. One case underwent additional intravenous chemotherapy for 3 cycles. Partial cystectomy was performed in 2 cases, with regular theprubicine irrigation in bladder and 1 case underwent intravenous chemotherapy for 2 cycles. Radical cystectomy was performed in 3 cases, with 2 cases undergoing intravenous chemotherapy after operation. Results Pathological findings showed that tumor cells were small and round in shape. These hyperchromatic nuclei showed limited cytoplasm with lack of nesting characters. CgA and NSE were positive in immunohistochemistry. The final diagnosis was small cell carcinoma, with 1 case accompanied with transitional cell carcinoma and 1 case accompanied with prostate cancer. One case showed high preoperative serum calcium (3.15 mmol/L) and low serum phosphate (0.61 mmol/L), which returned to normal 1 month after operation. Four cases who′s bladder was preserved were followed up, 3 cases were alive for 4, 9 and 25 months after operation. The 1 case who underwent intravenous chemotherapy was followed up for 24 months and there was no sign of relapse or metastasis. In all the 3 cases with radical cystectomy, 2 cases died 2 and 28 months postoperativly. Another case with adjuvant chemotherapy was followed up for 24 months without recurrence or metastasis. Conclusions Small cell carcinoma of the urinary bladder is highly malignant with poor prognosis. Radical cystectomy in combination with systemic chemotherapy has better efficacy. Retained bladder surgery with systemic chemotherapy is an alternative choice. The most important factors which influence the prognosis of the tumor are clinical stage and therapeutic methods.     

膀胱副神经节瘤二例报告并文献复习

目的 总结膀胱副神经节瘤的临床病理学特征及诊疗方法.方法膀胱副神经节瘤2例.例1,女,35岁.临床表现排尿后心慌、头痛,B超及CT检查示膀胱左侧壁肿块2.6 cm×1.5cm,尿3-甲氧-4-羟苦杏仁酸(VMA)35.3～43.3 μmol/24 h(正常值10～35 μmol/24 h).例2,男,22岁.临床表现无痛性全程血尿,B超及CT检查示膀胱右前壁肿块2.5 cm× 2.0 cm,尿VMA 17.9～31.3 μmol/24 h.结果行经尿道膀胱肿瘤电切术1例,行膀胱部分切除术1例.病理诊断为膀胱副神经节瘤.免疫组化CgA、Syn、S-100蛋白和NSE(+).患者术后症状消失,行膀胱镜及B超分别随访3个月、3年肿瘤无复发.结论膀胱副神经节瘤为潜在恶性肿瘤,诊断需结合临床、病理及免疫组化结果判断,膀胱部分切除为主要治疗手段.

Abstract：

Objective To study the clinical,histopathological,immunohistochemical features and the diagnosis and treatment of paraganglioma of urinary bladder. Methods Two cases of paraganglioma of urinary bladder were treated. The first case was a male with painless haematuria. The abdominal ultrasonography and CT scan showed a 2.5 cm× 2.0 cm mass in the right anterior wall of the bladder, and urine vanillylmandelic acid elevated to 17. 9- 31. 3 μmol/24 h (normal range 10- 35 μmol/24 h). The second case was a female who presented with 8 years history of headache and palpitation after voiding. Abdominal ultrasonography and CT scan showed a 2. 6 cm× 1.5 cm mass in the left wall of the bladder, and her urine vanillylmandelic acid was 35.3-43.3 μmol/24 h. Results One patient underwent transurethral resection (TURBT) and the other underwent partial cystectomy.The two cases were diagnosed as bladder paraganglioma by pathological examination. Immunohistochemically, the tumor cells were positive for CgA, Syn, NSE and S-100. No evidence of recurrence was detected during follow-up at 3 months and 3 years. Conclusions Paraganglioma of urinary bladder should be considered as a low grade malignancy. Partial cystectomy should be recommended. The diagnosis depends on clinical symptoms, pathological and immunohistochemical results.     

肾母细胞瘤合并肾母细胞瘤病6例临床分析

目的 探讨肾母细胞瘤(WT)合并肾母细胞瘤病(Nbm)的诊治方法及预后.方法 回顾分析2006年4月至2010年7月收治的6例WT合并Nbm患儿的临床资料.男4例,女2例;发病年龄5～14个月,平均9个月.WT位于左侧4例,右侧2例; Nbm位于WT同侧3例,对侧2例,双侧1例.WT均为单发,最大径分别为3、4、8、10、11、12 cm,Nbm单发3例,多发3例.行瘤肾切除术2例,保留肾脏的肿瘤切除+对侧肾上下极结节活检术1例,瘤肾切除+对侧保留肾脏的肿瘤切除术2例,保留肾脏的肿瘤及同侧结节切除术1例.术后以长春新碱、更生霉素或阿霉素化疗.结果 1例术后化疗15个月,33个月后肿瘤复发,手术切除后5个月再次复发,继续化疗11个月后死于肝脏、肺部转移.4例术后化疗6个月,其中3例已停化疗分别9、12、21个月;1例拟化疗15个月,目前已化疗8个月.此5例目前未见肿瘤复发,仍在密切随访中.结论 Nbm恶变率高,合并Nbm的WT复发率增高,化疗可降低Nbm恶变率,恶变或化疗无效的Nbm应行保留肾脏的肿瘤切除术.

Abstract：

Objective To investigate the diagnosis,treatment and prognosis of nephroblastomatosis (Nbm) combined with Wilms'tumor (WT). Methods Clinical data of six patients treated for WT combined with Nbm in Beijing Children's Hospital from 2006 to 2010 were reviewed retrospectively.The patients'ages ranged from five to 14 months.Two of the patients were female and four were male.The WTs were left-sided in four cases and right-sided in two cases.The Nbms were ipsilateral with WT in three cases,contralateral in two cases and bilateral in one case. The Nbms were single In three cases and multiple in three cases.WTs were all single and the maximum diameter was 3,4,8,10,11,and 12 cm respectively.Two paitents underwent nephrectomy.Nephron sparing surgery and upper and lower pole nodule biopsy was conducted in two cases,Nephrectomy and contralateral nephron sparing surgery was conducted in an additional two cases.Adjuvant chemotherapy included vincristine,actinomycin and doxorubicin. Results One patient had tumor recurrence 33 months after a 15 month regimen of postoperative chemotherapy. One patient had tumor recurrence and died after nephron sparing surgery 5 months after a 11 month regimen of chemotherapy.Four patients underwent 6 months of chemotherapy,and it was 9,12,and 21 months respectively after stop of chemotherapy.Another patient was still in chemotherapy. Conclusions Nbm is a pre-neoplastic proliferative process with high risk of developing WT.Chemotherapy may reduce the rate of Nbm malignancy.If Nbm is malignant or chemotherapy is invalid,nephron sparing surgery is recommended.     

肾癌多中心病灶和假包膜外肿瘤浸润的病理研究

目的 探讨保留肾单位手术(NSS)治疗肾癌时肿瘤周围正常肾组织安全有效的切除范围.方法2005年10月至2008年10月肾癌标本131例,其中行肾癌根治术103例,行NSS 28例.先行大体病理检查,然后分别在肿瘤假包膜外侧和距离肿瘤边缘3、5、10、15 mm各层面取材,每个层面取4块组织,HE组织染色.观察有无肾癌多中心病灶和假包膜外肿瘤的浸润范围等病理指标,分别测量病变到肿瘤边缘的距离,并统计肿瘤大小与以上指标的相关性.结果 131例肾癌标本中,肿瘤直径＜4.0 cm者61例,均未发现肿瘤周围浸润和卫星灶.肿瘤直径4～7 cm者46例,发现肿瘤周围浸润或卫星灶3例(6.5%),其中G3透明细胞癌1例,分别在距离肿瘤10、15 mm处发现卫星灶;集合管癌1例和G3透明细胞癌伴肉瘤样癌1例距离肿瘤周围15 mm均可见肿瘤浸润生长,集合管癌患者同时伴有远处转移和肾静脉瘤栓.肿瘤直径＞7 cm者24例,有肿瘤周围浸润或卫星灶4例(16.7%),其中1例G3透明细胞癌在肿瘤周围3 mm处发现卫星灶,另3例G2、G3透明细胞癌在肿瘤周围15 mm范围发现肿瘤浸润生长;有肾静脉瘤栓4例(16.7%);远处转移2例(8.3%).肿瘤直径与肿瘤周围浸润生长和卫星灶之间呈显著相关性(P＜0.05).结论直径＜4 cm的肾癌,行距离肿瘤周围正常肾组织切除宽度小的NSS,甚至简单的肿瘤剜除术安全有效;对部分仔细选择的4～7 cm肾癌,只要技术可行能够完整切除肿瘤,采用NSS治疗合理可行;而对于＞7 cm的肾癌,不建议行NSS.

Abstract：

Objective To explore the safe and effective width of a healthy parenchymal surgical margin in nephron-sparing surgery (NSS) for renal cell carcinoma. Methods From October, 2005to October, 2008, 131 renal carcinoma specimens (103 cases performed by radical nephrectomy and 28 cases by NSS) were studied. The tissue materials were taken at the site of pseudo-capsule, 3, 5, 10,15 mm laterally from the tumor edge respectively and HE staining. Specimens were examined grossly and microscopically for multifocal tumors, infiltration of tumor pseudo-capsule and other pathological features. The correlation between the renal tumor size and the pathological features were analyzed statistically. Results There were 131 specimens of renal carcinoma. In 61 cases with tumor diameter ＜4 cm, no case (0.0%) had multifocal tumors and infiltration of tumor pseudo-capsule. In 46 cases with tumor diameter 4-7 cm, multifocal tumors were found in 3 cases (6.5%), and infiltration of tumor pseudo-capsule was found in 2 cases. Among the 46 cases there was 1 collecting duct cancer accompanied with distant metastasis and renal vein tumor embolus. In 24 cases with tumor diameter ＞7cm, multifocal tumors were found in 4 cases (16. 7%) and infiltration of tumor pseudo-capsule was found in 3 cases. Four cases (16.7%) had renal vein tumor embolus. Two cases (8.3%) had distant metastasis. The renal tumor size was apparently associated with multifocal tumors and infiltration of tumor pseudo-capsule (P＜0. 05). Conclusions Mini-margin NSS, even simple enucleoresection, is a safe and effective approach for treating localized renal tumor of ＜4 cm. For carefully selected patients with tumor 4-7 cm, NSS is reasonable and feasible. But for the patient with tumor ＞7 cm,NSS is not recommended.     

胰腺实性假乳头状瘤的诊治(附4例)

Objective To discuss the management of solid-pseudopapillary tumor of the pancreas. Methods Four patients of solid-pseudopapillary tumor of the pancreas (SPT) were diagnosed by pathology. One patient was underwent magnetic resonance and two enhanced computer tomography scan. The mass was located in tuberculum (1 case), neck (1 case), and body-tail (2 cases) of pancreases respectively. Four cases were underwent duodenopancreatectomy, local resection of mass, resection of body-tail of pancreases respectively. The maximal diameter of tumor ranged from 5 cm to 16cm, averaged 10.3cm. The blood regular test and biochemistry were normal, and the tumor markers including AFP,CA19-9,CA125,CEA also were normal. The specimens were examined by pathology and immunohistochemistry. Results Four cases all were diagnosed solid-pseudopapillary tumor of the pancreas by pathology. At histologic analysis, the tumor was composed of uniform polygonal cells with moderate to abundant amphophilic cytoplasm and arranged in solid nests with areas of degeneration characterized by separation of the cells into pseudopapillary aggregates with intervening accumulation of mucopolysaccharide rich ground substance. The expressions of Vimentin, α-ACT, α-AAT were positive, and CgA negative. Four cases were followed up for six months and norecurred. Conclusions SPT of pancreases was a kind of tumor of low potential malignancy, and the magnetic resonance and computer tomography were often to be used. The effective therapy was thoroughly resection, and the prognosis in most patients is excellent.     

肾移植术后并发泌尿系统恶性肿瘤22例

目的 分析肾移植受者泌尿系统恶性肿瘤的发病情况,并探讨其发病机理及治疗方法.方法 回顾性分析1978年至2010年12月间肾移植受者发生泌尿系统恶性肿瘤22例的资料.结果 22例的病理检查结果分别为膀胱移行上皮细胞癌9例(其中1例第3次手术后发现转化为腺癌),膀胱鳞状细胞癌1例,膀胱腺癌1例,肾透明细胞癌3例(其中2例为双侧肾癌),肾低分化癌1例,肾盂移行细胞癌1例,肾盂+膀胱移行细胞癌1例,输尿管移行细胞癌2例,输尿管+膀胱移行细胞癌2例,输尿管移行细胞癌+膀胱腺癌1例.肾癌及输尿管癌均发生在患者原肾及输尿管.11例膀胱癌患者中9例存活,均保有全部或部分肾功能;4例肾癌患者均在发病后半年内死亡;肾盂癌、输尿管癌除2例术后早期死亡外,其余5例存活.22例发现肿瘤后1年存活率为73.7%.结论 肾移植后泌尿系统恶性肿瘤可见少见的病理类型.治疗中应注意免疫抑制剂的使用和移植肾功能保护的问题.肾实质性恶性肿瘤预后很差.

Abstract：

Objective To investigate the incidence of urological malignancy in renal allograft recipients and explore the mechanism of increased incidence in China and the management. Methods A retrospective study was performed on 22 patients with urological malignancy in renal allograft recipients between 1978 and 2010. Results Twenty-two cases of urological malignancy were diagnosed by pathologic evidence, including 9 cases of transitional cell carcinoma (TCC) of bladder, 1 case of squamous cell carcinoma of bladder, 1 case of adenocarcinoma of bladder, 1 case of TCC of pelvis, 1 case of TCC of bladder and pelvis, 1 case of TCC of ureter complicated with adenocarcinoma of bladder, 2 cases of TCC of ureter, 2 cases of TCC of ureter and bladder, 3 cases of clear cell carcinoma of kidney, and 1 case of undifferentiated carcinoma of kidney. All the malignancies belonged to native organs. All the patients suffering bladder cancer had normal function of allograft. Five patients with TCC of pelvis or ureter survived and 2 cases died early after operation. All the patients suffering renal carcinoma deceased within 6 months after diagnosis. One-year survival rate was 73. 7 % after the diagnosis of urological malignancy. Conclusion Urological malignancy ranked highest in malignancy in renal allograft recipients, and rare pathological types of urological malignancy in non-renal allograft recipients are often demonstrated. The strategy of treatment should take consideration of the relationship between the usage of immunosupressive agents and the preservation of allograft function. It is critical for the therapy of malignancies to possess satisfactory allograft function. The prognosis of renal cell carcinoma is poor.     

胰腺实性假乳头状瘤的诊治(附4例)

Objective To discuss the management of solid-pseudopapillary tumor of the pancreas. Methods Four patients of solid-pseudopapillary tumor of the pancreas (SPT) were diagnosed by pathology. One patient was underwent magnetic resonance and two enhanced computer tomography scan. The mass was located in tuberculum (1 case), neck (1 case), and body-tail (2 cases) of pancreases respectively. Four cases were underwent duodenopancreatectomy, local resection of mass, resection of body-tail of pancreases respectively. The maximal diameter of tumor ranged from 5 cm to 16cm, averaged 10.3cm. The blood regular test and biochemistry were normal, and the tumor markers including AFP,CA19-9,CA125,CEA also were normal. The specimens were examined by pathology and immunohistochemistry. Results Four cases all were diagnosed solid-pseudopapillary tumor of the pancreas by pathology. At histologic analysis, the tumor was composed of uniform polygonal cells with moderate to abundant amphophilic cytoplasm and arranged in solid nests with areas of degeneration characterized by separation of the cells into pseudopapillary aggregates with intervening accumulation of mucopolysaccharide rich ground substance. The expressions of Vimentin, α-ACT, α-AAT were positive, and CgA negative. Four cases were followed up for six months and norecurred. Conclusions SPT of pancreases was a kind of tumor of low potential malignancy, and the magnetic resonance and computer tomography were often to be used. The effective therapy was thoroughly resection, and the prognosis in most patients is excellent.     

肾盂鳞状细胞癌临床特点分析

目的 探讨肾盂鳞状细胞癌的诊治特点.方法 回顾性分析1991年10月至2009年5月收治8例肾盂鳞状细胞癌患者资料.临床表现血尿8例,腰痛7例,腹部包块1例.B超检查8例,IVU检查8例,CT检查4例.术前诊断为肿瘤3例,诊断为肾结石5例,结石术中发现肿瘤并经冰冻病理确诊2例.8例患者均经手术治疗,行根治性肾输尿管切除4例、单纯性肾切除3例、姑息性切除术1例.结果 8例病理诊断均为鳞状细胞癌.中分化6例,高分化和低分化各1例;pT1 1例,pT2 1例,pT3 3例,pT4 3例;淋巴结转移2例.获随访7例,失访1例.术后生存时间2～42个月,中位时间6个月,患者均死于肿瘤复发及转移.结论 肾盂鳞状细胞癌恶性程度高,常合并结石,术前诊断困难,确诊时多为中晚期,术后短期内易复发转移,预后极差.

Abstract：

Objective To review the diagnosis and treatment of squamous cell carcinoma of renal pelvis. Methods The clinical data from October 1991 to May 2009 of eight cases of squamous cell carcinoma of renal pelvis were reviewed and analyzed retrospectively. The symptoms of the patients were hematuria (eight cases), pain (seven cases) and abdominal mass (one case). All patients underwent B-ultrasound and IVU examination and four cases underwent CT scan. Three cases were diagnosed as having a tumor before surgery. Five cases were diagnosed as renal calculus, two of the five cases were diagnosed by intraoperative frozen section. Radical nephroureterectomy were performed in four cases, nephrectomy in three cases and palliative resection in one case. Results Histological classification revealed that six cases were moderately differentiated, one case was well differentiated and one case was poorly differentiated. Two cases had stage pT1/pT2 and six cases had stage pT3/pT4. 2 cases had regional lymph nodes metastasis. Seven cases were followed-up. All patients died of tumor recurrence or metastasis. The median tumor specific survive time was six months (range from two months to 42 months). Conclusions Squamous cell carcinoma of renal pelvis is often occurs concurrently with urolithiasis which could lead to difficulty in diagnose before operation. As the most of the patients were diagnosed with advanced stage disease, squamous cell carcinoma of renal pelvis tended to early recurrence and metastasis and the prognosis was very poor.     

肾移植术后并发自体尿路上皮多器官癌六例的临床分析

Objective To analyze the etiology, clinical diagnosis and outcomes of 6 cases of multifocal urothelial carcinomas following transplantation. Methods A retrospective analysis was performed on 6 cases of multifocal urothelial carcinomas following renal transplantation in our center. Results Six patients were diagnosed as having multifocal urothelial carcinomas, including 1 case of related renal transplantation. Five cases were diagnosed by painless gross hematuria 2～48 months after renal transplantation, and I patient was diagnosed as having bladder tumors by B-uhrasound. All lesions happened un-simultaneously, from 1.5～16 months, and each case accepted operation 2～5 times. One case accepted radical cystectomy and cutaneous ureterostomy of the graft, and one accepted radical cystectomy, cutaneous ureterostomy of the graft and urethrectomy. All of 6 cases received OKT3 or Daclizumab as the induced-immunosuppressive therapy and tacrolimus or cyclosporine A + mycofenolate mofetil + steroid as the maintenance therapy. Intravesical chemotherapy started in all patients immediately after the surgery. All operations were successful and most patients got satisfactory results. Conclusions Kidney recipients have a higher rate of transitional cell carcinomas which are characterized by easy metastasis, and mostly have unsatisfactory prognosis. Kidney recipients with hematuria should be examined carefully with cystoscopy, retrograde pyelography and cystourethroscopy. A routine examination at regular intervals after transplantation is very important.     

肾移植术后并发自体尿路上皮多器官癌六例的临床分析

Objective To analyze the etiology, clinical diagnosis and outcomes of 6 cases of multifocal urothelial carcinomas following transplantation. Methods A retrospective analysis was performed on 6 cases of multifocal urothelial carcinomas following renal transplantation in our center. Results Six patients were diagnosed as having multifocal urothelial carcinomas, including 1 case of related renal transplantation. Five cases were diagnosed by painless gross hematuria 2～48 months after renal transplantation, and I patient was diagnosed as having bladder tumors by B-uhrasound. All lesions happened un-simultaneously, from 1.5～16 months, and each case accepted operation 2～5 times. One case accepted radical cystectomy and cutaneous ureterostomy of the graft, and one accepted radical cystectomy, cutaneous ureterostomy of the graft and urethrectomy. All of 6 cases received OKT3 or Daclizumab as the induced-immunosuppressive therapy and tacrolimus or cyclosporine A + mycofenolate mofetil + steroid as the maintenance therapy. Intravesical chemotherapy started in all patients immediately after the surgery. All operations were successful and most patients got satisfactory results. Conclusions Kidney recipients have a higher rate of transitional cell carcinomas which are characterized by easy metastasis, and mostly have unsatisfactory prognosis. Kidney recipients with hematuria should be examined carefully with cystoscopy, retrograde pyelography and cystourethroscopy. A routine examination at regular intervals after transplantation is very important.     

A case of primitive neuroectodermal tumor of the kidney]

A primitive neuroectodermal tumor (PNET) is a solid tumor originating from the neural crest. This tumor is known to occur in the central nervous system and soft tissue, but recently determined to also invade the kidney. Although primary renal PNET is very rare, we encountered a case of primary renal PNET with multiple lung metastasis. This case was a 35-year-old man with a chief complaint of macroscopic hematuria, which he noticed in January 1996. He was hospitalized because of right renal tumor detected with ultrasonography. On CT scan, a low-density solid tumor with a maximum diameter of 8 cm was visualized in the right kidney. Low-signal and high-signal tumorous lesions were demonstrated in the same region on T 1-weighted and T 2-weighted MR images, respectively. Then right radical nephrectomy was performed on Feb. 5, 1996. On histopathological observation, the tumor was composed of small tumor cells with solid growth. Immunohistochemical staining revealed that the tumor was positive for CD 99. Thus our final diagnosis was primary right renal PNET. Although 2 metastatic lesions with a diameter of about 1 cm were observed in the right lower lung before the time of surgery, pulmonary metastatic lesions markedly increased in number 3 months after surgery. Thus we initiated CAP chemotherapy with cyclophosphamide, doxorubicin, and CDDP. After 3 cycles, pulmonary metastatic lesions disappeared at CT scan, indicating complete remission. However, a tumor with a maximum diameter of about 10 cm recurred in the retroperitoneum 7 months after complete remission. Although 2 cycles of CAV/PE therapy with cyclophosphamide, doxorubicin, vincristine, CDDP, and VP-16 were performed following resection of the retroperitoneal tumor, he showed no response and decreased 24 months after surgery.     

脊柱原始神经外胚叶肿瘤的诊断与治疗

目的 总结脊柱原始神经外胚叶肿瘤(primitive neuroectodermal tumor,PNET)的诊断和治疗经验.方法 回顾性分析1999年至2009年收治的PNET患者共13例,男8例,女5例.年龄4～43岁,平均(26.9±11.1)岁.肿瘤累及颈椎6例,腰椎5例,胸椎1例,骶椎1例.9例为手术后依据病...     

肾脏原发性恶性淋巴瘤(附三例报告)

目的 探讨肾脏原发性恶性淋巴瘤的临床特点。方法 总结3例肾脏原发性恶性淋巴瘤患者临床诊治资料，结合文献讨论其发病特点，影像学特征，治疗和预后。结果 3例患者中手术治疗2例，术后行化疗和放疗，l例病理确诊后单纯行化疗，3例病理均诊断为非霍奇金淋巴瘤(弥漫型)。l例术后1年3个月死于肾衰；另2例分别存活1年和2年1个月，仍在随访中。结论 肾脏原发性恶性淋巴瘤影像学征象与肾细胞癌相似，治疗应根据组织学分型、分期及肿瘤大小，采取手术联合化疗及放疗。     

胸部原始神经外胚层肿瘤(附10例报告)

目的 提高对胸部原始神经外胚层肿瘤（PNET）的认识，改进治疗效果。方法 回顾性分析1999年至2004年手术切除并经病理证实的10例胸部PNET治疗结果。结果 肿瘤位于胸腔内6例、胸壁2例，心包内和后纵隔各1例。胸内肿瘤完全摘除2例，大部分切除2例，肿瘤并肺叶切除2例。2例胸壁肿瘤合并受累肋骨切除胸壁重建，1例行肿瘤及心包部分切除，1例后纵隔哑铃形肿瘤由胸外科和神经外科同期切除。全组无手术死亡或住院死亡。病理标本免疫组化检查，10例CD99均为阳性，LEA均为阴性。术后4例接受化疗，4例放化疗，2例未行任何辅助治疗。术后随访6年，死亡7例，生存最长27个月，最短9个月，平均17个月。3例生存已超过12个月者仍在随访中。结论 胸部PNET恶性程度高，进展快，术前诊断率低。诊断与鉴别诊断需要病理组织学和免疫组化确定。彻底摘除肿瘤及受累组织是治疗的重要环节，术后放化疗有助延长生存期。提高生存率还需要寻找更有效的方法。     

内镜下钬激光治疗早期上尿路上皮肿瘤特殊病例1O例报告

目的探讨内镜下钬激光治疗特殊早期上尿路上皮肿瘤的安全性及有效性。方法2002年4月～2010年5月,对10例不适合行根治性。肾输尿管切除术的早期上尿路上皮肿瘤患者行内镜下钬激光治疗,其中输尿管肿瘤7例（1例合并膀胱肿瘤）,肾盂肿瘤3例。单发7例,多发3例。术前肿瘤分期cTa～cT1。3例对侧已行肾输尿管全长切除,2例孤立肾,3例肾功能不全,1例2～3级心功能不全,1例肿瘤小（〈1cm,位于。肾盂,单发且表浅）。输尿管硬镜治疗7例,软镜1例,微通道经皮肾镜2例。术后行丝裂霉素上尿路及膀胱灌注化疗。结果10例术后随访2年,无肿瘤死亡。1例术后6个月输尿管狭窄,其余均未出现大出血、严重感染、周围脏器损伤及全身肿瘤转移。复发4例,其中1例输尿管合并膀胱肿瘤者膀胱内复发,1例为肾盂内单发肿瘤复发,2例为输尿管单发肿瘤复发。该4例随访5年,1例未见肿瘤复发与转移,3例复发3—4次,且为尿路多处复发,行肾盂输尿管癌根治术,其中2例术后血液透析1年内肿瘤转移死亡。结论对不适合行根治性肾输尿管切除术的早期上尿路上皮肿瘤,内镜下钬激光治疗短期内是安全有效的。     

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.     

泌尿男生殖系统淋巴瘤的临床特征及预后分析

目的探讨泌尿男生殖系统淋巴瘤患者的临床特征,并对其预后进行分析。方法回顾性分析2014年8月至2019年8月北京友谊医院收治的9例泌尿男生殖系统淋巴瘤患者的临床资料。男5例,女4例。平均年龄62(50~69)岁。肿瘤位于肾脏3例,膀胱2例,睾丸4例。首发症状:肾肿瘤3例中2例为发热(1例伴腹痛及体重减轻),1例为体检发现肾盂占位;膀胱肿瘤1例为肉眼血尿伴腹痛,1例为尿急、尿痛;4例睾丸肿瘤均为睾丸无痛性肿大。5例行CT检查见低密度肿物伴轻中度强化,4例睾丸肿瘤行B超检查提示不规则、不均质肿块内见血流信号。术前诊断均为泌尿男生殖系统肿瘤。6例接受手术,3例行病灶穿刺获取病理。9例中4例接受手术治疗联合化疗,2例仅接受化疗,2例仅接受手术治疗,1例未治疗。肾肿瘤3例中,1例行B超引导下病灶穿刺活检术,术后予环磷酰胺+阿霉素+长春新碱+泼尼松+利妥昔单抗(R-CHOP)方案静脉化疗;1例肾盂占位性病变者行腹腔镜肾输尿管全长切除术,术后予R-CHOP方案静脉化疗;1例行B超引导下病灶穿刺活检术,术后患者拒绝进一步治疗。2例膀胱肿瘤者均行TURBT,术后均拒绝放化疗。4例睾丸肿瘤者中,3例行患侧睾丸切除术,1例行B超引导下病灶穿刺活检术;3例行R-CHOP方案化疗,1例于外院行化疗及对侧睾丸放疗,具体方案不详;4例均行鞘内注射化疗药物(甲氨蝶呤15 mg+阿糖胞苷50 mg+地塞米松5 mg)预防中枢神经系统浸润。结果本组9例术后恢复顺利,无手术及穿刺相关并发症发生。术后病理诊断均为非霍奇金淋巴瘤,其中8例为弥漫性大B细胞淋巴瘤,1例睾丸淋巴瘤为间变性大细胞淋巴瘤。按照Ann Arbor分期标准进行临床分期,确诊时Ⅰ~ⅡE期4例,ⅢE~ⅣE期5例。国际预后指数(IPI)评分0~2分6例,≥3分3例。随访时间平均18(6~66)个月。7例存活,其中3例完成化疗者中,2例完全缓解(睾丸淋巴瘤ⅡE期IPI评分1分和ⅣE期IPI评分2分各1例),1例部分缓解(肾盂淋巴瘤ⅣE期IPI评分3分);3例拒绝化疗者未见肿瘤复发或进展(膀胱淋巴瘤2例和肾盂淋巴瘤1例,均为ⅠE期IPI评分1分);1例化疗2个周期后因出现细菌真菌混合感染性肺炎停止化疗(睾丸淋巴瘤ⅢE期IPI评分3分)。肾淋巴瘤ⅢE期IPI评分3分1例和睾丸淋巴瘤Ⅲ期IPI评分2分1例,分别于确诊后6个月、17个月因肿瘤进展死亡。结论泌尿男生殖系统淋巴瘤以弥漫大B细胞淋巴瘤为主,临床表现特异性差,诊断时应根据症状及影像学表现与其他常见肿瘤进行鉴别,避免误诊误治。组织病理学是诊断的金标准,R-CHOP方案化疗为首选治疗手段。     

成人肾母细胞瘤的诊断与治疗（附12例报告）

目的探讨成人肾母细胞瘤的诊断和治疗方法。方法回顾性分析12例成人肾母细胞瘤患者的临床资料。结果本组患者年龄16-63岁,表现为肉眼血尿2例,腰腹痛6例,4例为体检发现;术前诊断为肾肿瘤9例,肾盂肿瘤3例。12例均行手术治疗,术后病理均诊断为肾母细胞瘤。按照美国国家肾母细胞瘤研究组（NWTS）分期标准,本组分别为Ⅰ期5例、Ⅱ期3例、Ⅲ期3例、Ⅳ期1例。术后辅以放疗和化疗,平均随访41个月,3年生存率为48%。结论成人肾母细胞瘤是一种比较少见的恶性肿瘤,术前确诊困难,手术治疗并辅以放疗、化疗是其主要的治疗方法。     

Metastatic primitive neuroectodermal tumor of the kidney in adults.

OBJECTIVE: Primitive neuroectodermal tumors (PNET) of the kidney are rare and highly aggressive malignancies. The purpose of our study was to present information about the management of patients with metastatic disease. METHODS: The records of 2 patients (30-year-old female and 32-year-old male) with metastatic PNET of the kidney were reviewed and our data compared with the literature. RESULTS: Neither clinical evaluation nor radiological methods allowed to distinguish PNET from renal cell carcinoma. Immunohistochemistry revealed strong positivity for CD99 in tumor 1 and weak positivity for NSE and vimentin in both tumors. In tumor 2, EWS/FLI1 translocation was detected by RT-PCR. Patient 1 underwent nephrectomy, seven cycles of polychemotherapy, two cycles of high-dose chemotherapy, autologous bone marrow rescue, radiotherapy of suspicious skeletal foci and is without evidence of recurrent disease 28 months after therapy. Patient 2 underwent six cycles of polychemotherapy, nephrectomy, high-dose chemotherapy with cyclophosphamide and abdominal radiotherapy. Because of relapse high-dose chemotherapy with stem cell rescue was not performed. He underwent three further cycles of polychemotherapy and died one year after diagnosis due to cerebral metastasis. CONCLUSIONS: The diagnosis of renal PNET must be considered in young patients with renal neoplasm, particularly those with advanced disease at presentation. Achieving exact diagnosis has important clinical consequences because polychemotherapy and high-dose chemotherapy may lead to dramatic tumor reduction or even complete remission.     

原发性非脐尿管型膀胱黏液腺癌临床病理学特点分析

目的 探讨原发性非脐尿管型膀胱黏液腺癌的临床及病理学特点.方法 原发性非脐尿管型膀胱黏液腺癌患者9例.男6例,女3例.平均年龄62(55～78)岁.间歇性、无痛性全程肉眼血尿8例,其中黏液血尿4例;体检发现1例.B超及CT检查均提示膀胱占位性病变;膀胱镜检发现膀胱肿物9例,取活检5例.病理报告均为膀胱黏液腺癌.肿瘤直径平均3.1(1.9～4.7)cm.手术治疗8例,其中膀胱部分切除术4例,膀胱全切加尿流改道3例,TURBt 1例.TURBt 1例术后6个月肿瘤复发,再行膀胱全切加尿流改道.1例体弱患者未手术.结果 9例病理报告均为原发性非脐尿管型膀胱黏液腺癌,病理分期T_(2a)5例、T_(2b) 2例、T_(3a)2例.病理分级高分化5例、中分化4例.9例平均随访16.5(3～60)个月.术后1年内死亡4例,1例未手术者随访4个月死于心功能衰竭;1例行TURBt患者,再行根治术术后3个月死于肺转移;2例行膀胱部分切除术者分别于术后9、11个月死于肿瘤转移.其余5例未见肿瘤复发及转移.结论 原发性非脐尿管型膀胱黏液腺癌少见,预后差.手术为主要的治疗手段,对复发或转移患者,配合放疗或化疗等综合治疗可延长患者生存期.