膀胱炎性肌纤维母细胞瘤2例报告并文献复习

目的：探讨膀胱炎性肌纤维母细胞瘤的临床、病理、组织生化特性和诊治方法及预后。方法：报告2例肉眼血尿伴重度贫血的膀胱炎性肌纤维母细胞瘤患者的诊治资料,并结合文献进行探讨。结果：膀胱炎性肌纤维母细胞瘤以肉眼血尿和尿痛为最常见症状,肿瘤好发于膀胱底部、顶壁、侧壁;组织学由具有平滑肌细胞和纤维母细胞特征的梭形细胞、大量慢性炎性细胞及血管样黏液构成。1例随访3年,1例随访6个月,均无瘤存活。结论：膀胱炎性肌纤维母细胞瘤为低度恶性肿瘤,罕见。确诊依据病理学检查,应与平滑肌肉瘤、横纹肌肉瘤、梭形细胞癌等鉴别;治疗原则以切除肿瘤、保留膀胱功能为主,TUPKBT、TURBT或膀胱部分切除术,术后辅以化疗药物膀胱灌注,效果佳。     

膀胱炎性肌纤维母细胞瘤合并腺性膀胱炎1例报告

本文回顾性分析1例膀胱炎性肌纤维母细胞瘤合并腺性膀胱炎患者的临床资料。患者因肉眼血尿, 偶伴尿痛20余天入院。术前影像检查提示膀胱前壁占位性病变, 大小约7.7 cm×6.1 cm×4.4 cm, 考虑膀胱癌可能性大。行机器人辅助腹腔镜膀胱部分切除术, 术后病理诊断为膀胱炎性肌纤维母细胞瘤合并腺性膀胱炎。膀胱炎性肌纤维母细胞瘤是一种起源于膀胱间叶组织的真性肿瘤, 属于临床罕见病, 合并有腺性膀胱炎目前尚未见报道。由于本病的临床表现和影像学检查表现与膀胱恶性肿瘤难以鉴别, 易造成误诊, 确诊主要依靠病理检查和免疫组化染色检查。治疗方法为经尿道膀胱肿瘤切除术、膀胱部分切除术或根治性膀胱切除术。     

泌尿系炎性肌纤维母细胞瘤二例报告并文献复习

目的 提高对泌尿系炎性肌纤维母细胞瘤的认识.方法 分析2例泌尿系炎性肌纤维母细胞瘤患者资料并复习文献.例1,女,50岁.主诉尿痛、排尿困难2个月,B超、膀胱镜检示右侧输尿管口肿物直径约3 cm.例2,男,63岁.主诉无痛性肉眼血尿2 d,B超、膀胱镜检见膀胱三角区实性占位,直径约7 cm.结果 2例肿瘤均完整切除.例1标本光镜下见肿瘤细胞多呈长梭形,平行排列,核分裂象少见,未见病理性核分裂象,有散在炎细胞浸润,免疫组化染色示MyoD1(-),SMA(+),Vimentin(+),诊断为输尿管炎性肌纤维母细胞瘤.例2标本光镜下见肿瘤细胞呈长梭形,核仁嗜碱性,未见病理性核分裂象,间质中有散在的炎细胞浸润,少量梭形细胞明显侵蚀平滑肌束,免疫组化染色示CD34(+),MyoD1(+),符合膀胱炎性肌纤维母细胞瘤诊断.术后每3个月复查膀胱镜,例1随诊6个月,例2随诊2年,均未见复发.结论泌尿系炎性肌纤维母细胞瘤是一种特殊类型的炎性假瘤,本质上是一种良性肿瘤,临床诊断困难,治疗以手术切除为主,术后应加强随访.     

膀胱炎性肌纤维母细胞瘤一例报告并文献复习

目的探讨膀胱炎性肌纤维母细胞瘤的临床、病理、组织化学特性和诊治方法及预后。方法总结1例膀胱炎性肌纤维母细胞瘤患者的临床资料。患者,男,54岁,肉眼血尿10 d入院。盆腔B超及CT增强扫描示膀胱前壁占位性病变。膀胱镜检可见顶壁4 cm×4 cm×5 cm肿物,表面缺血呈暗红色,病理活检疑为膀胱尿路上皮恶性肿瘤。结果行膀胱部分切除术。组织学表现为增生的梭形或长梭形肿瘤细胞囊状分布,间质小血管增生,伴淋巴细胞、嗜酸性粒细胞及浆细胞浸润,诊断为膀胱炎性肌纤维母细胞瘤。免疫组织化学染色结果为瘤细胞平滑肌特异性肌动蛋白（＋）、波形蛋白（＋）、间变性淋巴瘤激酶（＋）。随访6个月,患者无瘤存活。结论膀胱炎性肌纤维母细胞瘤确诊依据病理学检查,应与横纹肌肉瘤、梭形细胞肉瘤等鉴别,治疗以膀胱部分切除或经尿道切除为主。     

膀胱炎性肌纤维母细胞瘤1例报告并文献复习

目的探讨膀胱炎性肌纤维母细胞瘤的临床特点和病理特征。方法报告1例肉眼血尿伴休克的膀胱炎性肌纤维母细胞瘤患者的诊治资料,并结合文献进行探讨。结果膀胱炎性肌纤维母细胞瘤（IMT）以血尿和尿痛为最常见症状,肿瘤好发于膀胱底部、顶壁、侧壁;TURBT或膀胱部分切除术效果佳;确诊需依靠病理学检查。结论发生于膀胱的IMT为低度恶性,治疗原则以切除肿瘤、保留膀胱功能为主。     

膀胱炎性肌纤维母细胞瘤一例报告

膀胱炎性肌纤维母细胞瘤临床罕见，易被误诊为梭形细胞肉瘤等恶性病变。2004年5月我院收治1例，现报告如下。     

膀胱炎性肌纤维母细胞瘤的临床特征与治疗(附5例报告)

目的:探讨膀胱炎性肌纤维母细胞瘤(IMTUB)的临床诊断及治疗。方法:回顾性分析我院病理检查确诊为IMTUB的5例患者临床资料:5例术前均诊断为膀胱肿瘤,临床表现均缺乏特异性。光镜下可见梭形或长梭形细胞增生,且呈囊状分布,伴有间质小血管增生及大量浆细胞、嗜酸性粒细胞、中性粒细胞及淋巴细胞浸润。免疫表型上,间变性淋巴瘤激酶阳性5例,Desmin阳性4例,3例行膀胱部分切除术,2例行经尿道膀胱肿瘤电切术。结果:5例均临床治愈。随访11~63个月,平均34个月,患者无明显的血尿及尿频、尿急等症状,B超检查未见肿物复发。结论:IMTUB是一种交界性并具恶性潜能的肿瘤,临床诊断困难,确诊依赖于病理组织学及免疫组织化学染色检查。治疗以手术为主。     

腹腔镜膀胱部分切除术治疗膀胱炎性肌纤维母细胞瘤1例并文献复习

目的:探讨腹腔镜膀胱部分切除术(LPC)治疗膀胱炎性肌纤维母细胞瘤(IMT)的临床特点病理特征及治疗方法。方法:回顾分析1例LPC治疗IMT患者的资料,肿瘤位于膀胱左侧顶部,行腹腔镜膀胱部分切除术,术后6个月未见复发。结合文献进行讨论。结果:IMT以血尿为最常见症状,肿瘤好发于膀胱底部、顶部、侧壁。TURBT或膀胱部分切除术效果最佳。确诊依靠病理检查,组织学表现为增生的梭形细胞,SMA(+)、Vimentin(+)、ALK(+)。结论:炎性纤维母细胞瘤是一种少见具有恶性潜能的肿瘤,治疗以切除肿瘤、保留膀胱功能为主。位于顶部或侧壁的炎性肌纤维母细胞瘤行腹腔镜膀胱部分切除术安全、有效。     

胃炎性肌纤维母细胞瘤的临床病理及腹腔镜手术治疗

本文报道1例胃炎性肌纤维母细胞瘤的临床资料,成功行腹腔镜下远端胃切除术。随访16个月,临床未见肿瘤复发和转移。组织病理学结果证实为梭形细胞密集型炎性肌纤维母细胞瘤,镜下显示肿瘤由梭形细胞和大量炎性细胞组成,免疫组化示Vimentin、SMA、ALK阳性,S-100、CDll7阴性。     

膀胱移行细胞癌术后再发膀胱小细胞癌1例

患者,男,74岁。因无痛性血尿1个月余,以膀胱肿瘤复发入院。患者于16年前因无痛性血尿,经膀胱镜检查诊断为膀胱左侧壁肿瘤,并行膀胱部分切除术,术后病理诊断为膀胱移行细胞癌Ⅲ级,术后膀胱灌注噻替哌60mg/次,共6次,定期复查膀胱镜无肿瘤复发。1个月前再次出现血尿。体检:一般情况可,心肺腹无异常,B超膀胱右前壁1.0cm×1.5cm肿瘤,双肾无异常。X线片:双肺未见肿块阴影,膀胱镜检查:膀胱右前壁1.5cm×1.5cm×1.0cm菜花状肿物,基底部宽,周围膀胱黏膜正常,于2004年10月18日在硬脊膜外阻滞麻醉下行TURBT,术中见膀胱右前壁1.5cm×1.5cm×1.0cm菜花…     

Inflammatory myofibroblastic tumors of the urinary tract: a clinicopathologic study of 46 cases, including a malignant example inflammatory fibrosarcoma and a subset associated with high-grade urothelial carcinoma

Inflammatory myofibroblastic tumor (IMT) of the urinary tract, also termed postoperative spindle cell nodule, inflammatory pseudotumor, and pseudosarcomatous fibromyxoid tumor, is rare and in the past was believed to reflect diverse entities. We reviewed a series of 46 IMTs arising in the ureter, bladder, and prostate, derived primarily from a large consultation practice. There were 30 male and 16 females aged 3 to 89 years (mean 53.6). Lesions were 1.2 to 12 cm (mean 4.2). There was a history of recent prior instrumentation in 8 cases. Morphology was similar to that previously described for IMT occurring in this region, with the exception of 1 case that focally appeared sarcomatous. Polypoid cystitis coexisted in 5 patients (11%). Mitoses were typically scant (0 to 20/10 hpf, mean 1). Necrosis was seen in 14 (30%) cases. Invasion of the muscularis propria was documented in 19 (41%). By immunohistochemistry (IHC), lesions at least focally expressed anaplastic lymphoma kinase (ALK) (20/35, 57%), AE1/3 (25/34, 73%), CAM5.2 (10/15, 67%), CK18 (6/6, 100%), actin (23/25, 92%), desmin (15/19, 79%), calponin (6/7, 86%), caldesmon (4/7, 57%, rare cells), p53 (10/13, 77%), and most lacked S100 (0/14), CD34 (0/13), CD117 (2/13, 15%), CD21 (0/5), and CD23 (0/3). ALK gene alterations were detected by fluorescence in situ hybridization (FISH) in 13/18 (72%) tested cases, including 2 with prior instrumentation; 13/18 (72%) showed agreement between FISH ALK results and ALK protein results by IHC. Most bladder IMTs were managed locally, but partial cystectomy was performed as the initial management in 7 cases and cystectomy in 1 (1 IMT was initially misinterpreted as carcinoma, 1 IMT was found incidentally as a separate lesion in a cystectomy specimen performed for urothelial carcinoma). Follow-up was available in 32 cases (range 3 to 120 mo; mean 33; median 24). There were 10 patients with recurrences (2 with 2 recurrences). Recurrences were unassociated with muscle invasion or with ALK alterations. In 2 cases, tumors of the urinary tract (TURs) showing IMT preceded (1 and 2 mo, respectively) TURs showing sarcomatoid carcinoma with high-grade invasive urothelial carcinoma accompanied with separate fragments of IMT. Even on re-review the IMT in these 2 cases were morphologically indistinguishable from other cases of IMT, with FISH demonstrating ALK alterations in the IMT areas in one of them. Both these patients died of their carcinomas. Lastly, there was 1 tumor with many morphological features of IMT and an ALK rearrangement, yet overtly sarcomatous. This case arose postirradiation for prostate cancer 4 years before the development of the lesion, with tumor recurrence at 4 months and death from intra-abdominal metastatic disease at 9 months. In summary, urinary tract IMTs are rare and share many features with counterparts in other sites, displaying similar morphology and immunogenotypic features whether de novo or postinstrumentation. Typical IMTs can be locally aggressive, sometimes requiring radical surgical resection, but none of our typical cases metastasized, although they can rarely arise contemporaneously with sarcomatoid urothelial carcinomas. For these reasons, close follow-up is warranted.     

膀胱小细胞癌(附五例报告)

目的 探讨膀胱小细胞癌的诊治疗效。方法 对5例膀胱小细胞癌的病理及临床资料进行回顾性分析。结果 5例患者,男4例,女1例,平均年龄67岁。分期T2N0M01例,T3N0M02例,T4N0M01例,T4N3M01例。根治性膀胱切除1例,根治性膀胱切除加放疖例,动脉导管化疗1例,动脉导管化疗、术前疗加姑息膀胱切除1例。3例死于肿瘤复发,平均生存时间17个月,2例存活7年。结论 膀胱小细胞癌预后差,手术加联合化疗可提高治愈率。     

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.     

Case series: Inflammatory myofibroblastic bladder tumor in regional Australia

Introduction and importanceInflammatory myofibroblastic tumor (IMT) is a rare tumor subtype that affects multiple organ systems. This case series adds a regional perspective to the literature, demonstrating rare urological cancers can be managed excellently in regional settings.Case presentationWe report a case series of two patients in regional Australia who were diagnosed with IMT of the urinary bladder. Both patients received computed tomography (CT) and ultrasound (US) imaging to investigate lower urinary tract symptoms (LUTS). Following initial diagnosis of a bladder tumor, both patients underwent surgery, one having a transurethral resection of bladder tumor (TURBT) with the other receiving a partial cystectomy. Histology from both surgeries revealed IMT of the urinary bladder, with the first case going on to receive a partial cystectomy to ensure clear surgical margins. Both patients recovered well post-operatively, with CT cystograms revealing water-tight bladders. Initial follow up reveals no recurrence of disease.Clinical discussionIMT of the urinary bladder is an exceedingly rare clinical entity which is fortunately benign and can be well managed with appropriate surgical intervention.ConclusionIMT can be well managed in regional hospitals equipped with appropriate surgical, pathological and oncological services.     

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.     

Cancer specific outcomes in patients with pT0 disease following radical cystectomy

PURPOSE: We assessed clinical outcomes in patients found to have no evidence of disease, ie pT0, in the cystectomy specimen following radical cystectomy for transitional cell carcinoma. MATERIALS AND METHODS: Between 1984 and 2003, 955 consecutive patients underwent bilateral pelvic lymphadenectomy and radical cystectomy for bladder cancer at 3 institutions, namely The Johns Hopkins Hospital, University of Texas Southwestern Medical Center and Baylor College of Medicine. Excluding nonTCC histology and patients with missing data resulted in 888 evaluable cases. Primary end points were recurrence-free survival and bladder cancer specific survival. RESULTS: Final pathological evaluation revealed absent transitional cell carcinoma in the cystectomy specimen, ie pT0, in 59 patients (7%), of whom 2 (3%) had pathologically positive lymph nodes. Transurethral resection stage or clinical stage data were available on 56 patients (95%), including Tis in 5 (9%), Ta in 2 (4%), T1 in 18 (32%), T2 in 29 (52%) and T3 in 2 (4%). Overall 6 recurrences (10%) were noted, including cTis in 1 case, cT1 in 1, cT2 in 3 and cT3 in 1. Median followup in patients with pT0 disease was 56 months (range 3 to 183). Three patients (5%) died of bladder cancer and another 4 (7%) died of other causes. Five and 10-year bladder cancer progression-free and cancer specific survival estimates in patients with pT0 disease were 90% and 81%, and 95% and 85%, respectively. CONCLUSIONS: Despite excellent clinical outcomes in the majority of patients with no evidence of tumor on final pathological evaluation not all patients with pT0 disease in the cystectomy specimen are cured of bladder cancer. These events may even occur in patients with nonmuscle invasive or muscle invasive organ confined pathology at staging transurethral resection. Further study is needed to identify prognostic factors in this population.     

The effects of neoadjuvant chemotherapy and chemo-radiation therapy on MRI staging in invasive bladder cancer: comparative study based on the pathological examination of whole layer bladder wall

Objective

We evaluated the correlation of radiological findings obtained by MRI study with pathological diagnosis in invasive bladder cancer treated with neoadjuvant chemotherapy, with or without radiation.

Design, Setting, and Participants

Twenty-seven patients, who underwent total or partial cystectomy for invasive bladder tumors, were enrolled into the present study. Eight cases had received neoadjuvant chemotherapy following the staging biopsy (group A), ten cases had received chemo-radiation therapy following the staging biopsy (group B), and nine cases had received preoperative staging biopsy alone (group C). As a final treatment, 12 of the 27 patients underwent total cystectomy and the other 15 patients underwent partial cystectomy. MRI was conducted prior to total or partial cystectomy in each case. The pathological stage was assessed by histological examination of the entire layer of the bladder wall.

Results and Limitations

Tumor stage assessed by MRI was consistent with pathological findings in 16 of the 27 cases (59.3%), while MRI produced over-staging in 7 cases and under-staging in 4 cases. The accuracy of staging was 75.0, 30.0, and 77.8% in groups A, B, and C, respectively. The accuracy of MRI staging in group B was lower than that in group C (P < 0.05). There was no difference in the accuracy of MRI staging between groups A and C.

Conclusion

MRI is useful for the staging of bladder cancer. However, care needs to be taken when staging invasive bladder tumors treated with neoadjuvant chemo-radiation therapy, because inflammatory infiltrations and/or fibrous changes caused by the chemotherapy or chemo-radiation therapy make precise staging with MRI difficult.     

先天性静脉畸形骨肥大综合征合并膀胱血管瘤一例报告并文献复习

目的 提高对先天性静脉畸形骨肥大综合征合并膀胱血管瘤患者的诊治水平. 方法 患者,女,23岁.主因间断无痛性全程肉眼血尿1个月,发现膀胱肿物20 d于2010年11月入院.外院膀胱活检病理提示血管瘤病.既往:左下肢血管瘤23年,分别于3个月、3岁、13岁时行左下肢血管瘤切除术.查体见左下肢膝关节以下明显增粗并长于右侧,左小腿及足踝部软组织增生.B超检查提示膀胱左前壁实性占位,范围4.1 cm ×3.0 cm ×2.5 cm.膀胱镜检可见膀胱顶部略偏左侧黏膜隆起并可见曲张静脉血管,范围3.0 cm x5.0 cm. 结果 患者诊断为膀胱血管瘤,先天性静脉畸形骨肥大综合征.行膀胱部分切除术.术后病理为膀胱肌间血管瘤.术后肉眼血尿消失.术后3个月复查B超,膀胱未见异常. 结论 先天性静脉畸形骨肥大综合征可能合并膀胱血管瘤,膀胱部分切除术可以作为治疗方法之一.     

A case of transitional cell carcinoma with squamous differentiation which developed squamous cell carcinoma in situ in the clinical course

In August 2000, a 62-year-old woman presented to another municipal hospital with macroscopic Transurethral resection of bladder tumor (TUR-Bt) was performed. The pathological hematuria. diagnosis was transitional cell carcinoma (TCC), G2 > squamous cell carcinoma (SCC). TUR-Bt repeated in July 2003 indicated recurrence. The pathological diagnosis was TCC, G2. She was referred to our hospital in August 2003 because she desired bladder preservation. After cystoscopy and random biopsy, pathological diagnosis was TCC with squamous differentiation, G1-G2, pTis. She received 7 weekly intravesical bacillus Calmette-Guerin (BCG) instillations. In April 2004, TUR-Bt was repeated and multiple recurrences were found. The pathological diagnosis was TCC with squamous differentiation, G1-G2, pTa. She received 10 weekly intravesical Pirarubicin hydrochroride instillations. In August cystoscopy and random biopsy were performed for evaluation of the intavesical instillation treatment. Pathological diagnosis was atypical squamous cells. In November, cystoscopy revealed recurrence of a bladder tumor. After admission, a small papillary tumor and multiple flat lesion biopsies demonstrated SCC without obvious invasion. The patient underwent cystectomy. There were widespread areas of full thickness squamous atypia. Most of the bladder did not show appearance of typical TCC, but the final pathological diagnosis was TCC because the case developed from TCC and could not be diagnosed as pure SCC. The diagnosis of SCC in situ of bladder is difficult, and this may contribute to its rarity.