Sarcomatoid urothelial carcinoma with choriocarcinomatous features: first report of an unusual case

A 61-year-old man presented with gross hematuria and a polypoid right lateral bladder mass. The tumor was composed of conventional urothelial carcinoma with sarcomatoid and choriocarcinomatous features, both positive for epithelial markers (pancytokeratin, AE1/AE3, and CAM 5.2). In addition, choriocarcinomatous tumor cells were positive for beta human chorionic gonadotropin and placental alkaline phosphatase. Treatments included surgery, chemotherapy, and radiation therapy. The clinical course was aggressive, with liver, lung, and distant lymph node metastases and a postdiagnosis survival of 6 months. This is the first report, to our knowledge, indicating both sarcomatoid and choriocarcinomatous features in a conventional urothelial carcinoma of the bladder.     

Sarcomatoid carcinoma of the urinary bladder in a hemodialysis patient: a case report

The following is a case report bladder of sarcomatoid carcinoma in a Japanese 65-year old female patient treated with hemodialysis. She developed chronic renal failure due to chronic glomerulonephritis. Fifteen months after the beginning of the hemodialysis, continuous gross hematuria was noticed, and cystoscopy revealed a broad-based bladder tumor spreading from the right lateral wall to the posterior wall. The histopathologic diagnosis of the TUR-Bt specimens was sarcoma. Radical cystectomy was performed under the diagnosis clinical stage III, T3bN0M0. The post-operative histopathologic diagnosis of the tumor was sarcomatoid carcinoma, composed of nests of transitional cell carcinoma (G 3) and predominant areas of spindle cell sarcomatoid transformation. Sarcomatoid carcinoma of the bladder in a hemodialysis patient is extremely rare, and to date this may be only the second case in Japanese medical literature.     

Utility of a comprehensive immunohistochemical panel in the differential diagnosis of spindle cell lesions of the urinary bladder

Spindle cell lesions of the urinary bladder are uncommon, but when encountered in clinical practice, pose a difficult diagnostic challenge as the differential diagnostic considerations are vast. Pseudosarcomatous processes significantly overlap with malignant tumors (sarcomatoid urothelial carcinoma and leiomyosarcoma) in their morphology and published immunohistochemical profile [pancytokeratin pan (CK), smooth muscle actin (SMA), and desmin]. p63 has been studied rarely and CK 5/6 and CK 34betaE12 have not been analyzed in the bladder in this diagnostic context. In the current study, 45 typical examples of spindle cell lesions [10 pseudosarcomatous myofibroblastic proliferations (PMP), 22 sarcomatoid urothelial carcinomas, and 13 smooth muscle tumors] of the urinary bladder were immunostained with a panel containing broad spectrum anticytokeratin antibodies (OSCAR or AE1/AE3), as well as antibodies to CK 34betaE12, CK 5/6, p63, SMA, and anaplastic lymphoma kinase (ALK). The immunoreactivity was as follows: PMP-CK (OSCAR) 7/10 (70%), CK (AE1/AE3) 7/9 (78%), CK 34betaE12 0/10 (0%), CK 5/6 0/9 (0%), p63 0/9 (0%), SMA 10/10 (100%), ALK 2/10 (20%); sarcomatoid urothelial carcinoma-CK (OSCAR) 15/22 (68%), CK (AE1/AE3) 14/20 (70%), CK 34betaE12 5/20 (25%), CK5/6 6/22 (27%), p63 11/22 (50%), SMA 16/22 (73%), ALK 0/22 (0%); and smooth muscle tumors-CK (OSCAR) 7/13 (54%), CK (AE1/AE3) 7/12 (58%), CK 34betaE12 0/12 (0%), CK 5/6 0/12 (0%), p63 3/13 (23%), SMA 11/13 (85%), ALK 0/13 (0%). Positivity for keratin was typically focal to moderate in smooth muscle tumors and more commonly moderate to diffuse in sarcomatoid carcinomas and PMP. Our data indicate that there is significant immunohistochemical overlap between the different spindle cell lesions, each of which has unique clinicopathologic, prognostic, and therapeutic ramifications. Within the context of morphology, an immunohistochemical panel composed of broad-spectrum antibodies to cytokeratin as well as antibodies to SMA, ALK, p63, and CK 5/6 will be a useful diagnostic adjunct: a combination of pankeratin, SMA, and ALK positivity favors PMP; expression of several cytokeratin and especially CK 34betaE12 and CK 5/6 with p63 favors sarcomatoid carcinoma and SMA positivity with overall absence of other markers favors leiomyosarcoma.     

Primary intracranial sarcomatoid carcinoma arising from a recurrent/residual epidermoid cyst of the cerebellopontine angle: a case report

Primary intracranial squamous cell carcinomas (SCCs) are rare and mostly associated with an intracranial epidermoid or dermoid cyst. Sarcomatoid carcinoma is a rare biphasic tumor composed of both carcinomatous and sarcomatous components and has not previously been reported as a primary intracranial tumor. Here, we present a case of a 60-year-old man with a primary intracranial sarcomatoid carcinoma, arising from the remnants of the previously resected epidermoid cyst in the cerebellopontine angle. The resected material had portions of an epidermoid cyst lined by normal and dysplastic squamous epithelia and invasive keratinizing SCC. This area was in continuity with areas of highly pleomorphic, anaplastic sarcomatoid cells. Brisk mitotic activity and extensive areas of necrosis were found. On immunohistochemical staining, the cells of the conventional SCC were positive for cytokeratin 5/6, pancytokeratin, epithelial membrane antigen, p63, and p53. The sarcomatoid cells were diffusely and strongly positive for vimentin, p53, smooth muscle actin, and, focally, muscle-specific actin. Occasional sarcomatoid cells coexpressed cytokeratin 5/6, pancytokeratin, p63, and S100 protein. The patient subsequently developed leptomeningeal spread and died 4 months after the second surgery. This rare entity expands the morphologic spectrum encountered in primary intracranial carcinoma.     

A case report of sarcomatoid carcinoma of the bladder with metastasis to small intestine

A 67-year-old male presented to our clinic with gross hematuria. Cystoscopic examination revealed a broad-based tumor of 2.5 cm in diameter on the lateral side of the right ureteral orifice. Under the clinical diagnosis of TCC G2 > G3, T3bNOM0, radical cystectomy with orthotopic bladder substitution was performed. Pathological diagnosis was TCC G3 with sarcomatoid carcinoma, pT2pR0pL1 pVlpN0. Adjuvant chemotherapy was not performed because of his transient poor conditions. Lung metastasis was observed 6 months postoperatively. Despite of M-VAC therapy and radiation therapy, additional metastases to brain and liver were observed. One month later, partial ileectomy specimen for occlusive ileum revealed the same histologic findings, TCC G3 with sarcomatoid carcinoma. He died 9 months postoperatively. To our knowledge, this is the first case of sarcomatoid carcinoma of the bladder with metastasis to small intestine, although 6 cases of transitional cell carcinoma of the bladder with metastasis to small intestine has been reported in Japan.     

前列腺肉瘤样癌二例报告并文献复习

目的 探讨前列腺肉瘤样癌的临床表现、病理特点和诊治方法. 方法 前列腺肉瘤样癌患者2例.例1,51岁.因排尿困难、会阴部不适2个月,急性尿潴留入院.实验室检查PSA值2.31 ng/ml,CT检查示前列腺密度不均,左叶弥漫性增大、浸润膀胱.经直肠穿刺活检诊断为前列腺肉瘤样癌.行全膀胱、前列腺切除加尿流改道(Bricker手术),术后行局部放射治疗和内分泌治疗.例2,54岁,因排尿困难伴间歇性肉眼血尿1个月入院.实验室检查PSA 2.61 ng/ml.B超检查示低回声块.CT检查示前列腺密度不均.经直肠穿刺活检诊断为前列腺肉瘤.行全膀胱、前列腺切除加尿流改道(Bricker手术). 结果 2例术后病理均诊断为前列腺肉瘤样癌.镜下肿瘤组织由上皮癌细胞和肉瘤样间质2种成分组成,之间可见移行区过渡.免疫组化:2种成分中细胞角蛋白、上皮膜抗原均呈阳性表达.癌细胞波形蛋白阴性,肉瘤样细胞阳性.例1术后41个月出现广泛转移,2个月后死亡.实验室检查PSA正常.例2术后16个月出现骨转移并有局部复发,手术去势联合比卡鲁胺最大限度雄激素阻断治疗3个月无效,术后19个月死亡.实验室检查PSA<4.0 ng/ml.结论前列腺肉瘤样癌是一种罕见、高度恶性的肿瘤,预后不良,确诊需依赖病理表现及免疫组织化学检查.根治性切除辅以局部放射治疗和内分泌治疗可行.     

膀胱肉瘤样癌与癌肉瘤

目的探讨膀胱肉瘤样癌及膀胱癌肉瘤组织学特性。提高对膀胱肉瘤样癌和膀胱癌肉瘤的病理学和临床特征的认识。方法报告1例膀胱肉瘤样癌和1例膀胱癌肉瘤的病例资料。2例均为男性。年龄分别为60岁、66岁。1例以肉眼血尿就诊,膀胱镜、CT和B超检查均诊断为膀胱肿瘤,肿瘤呈侵润性生长,术前活检提示为移行上皮细胞癌。行膀胱部分切除术,术后行全身化疗和膀胱灌注化疗;病理检查为癌肉瘤。另1例以膀胱血块填塞就诊,急诊手术行血块清除、止血及肿瘤姑息性切除,术后均行全身化疗和膀胱灌注化疗,术后病理检查为肉瘤样癌。结果1例行膀胱部分切除者,术后病理可见上皮和肉瘤样间质2种恶性成分,且可见到横纹肌肉瘤成分,诊断为膀胱癌肉瘤,3个月后复查局部肿瘤复发。行膀胱全切-回肠新膀胱术,目前仍在随访中。另1例术后病理可见上皮和肉瘤样间质2种恶性成分,癌与肉瘤样区有移行,诊断为膀胱肉瘤样癌,1个月后死于全身衰竭、多处转移。结论膀胱肉瘤样癌和癌肉瘤具有浸润性生长的生物学特性,恶性程度高,预后不良;化疗、放疗都不太敏感,手术仍是首选治疗方式。     

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.     

膀胱癌肉瘤与肉瘤样癌诊治再讨论

目的：探讨膀胱癌肉瘤和肉瘤样癌的组织学特性、临床表现、治疗以及预后.方法：回顾性分析1例膀胱癌肉瘤及2例肉瘤样痛患者的临床、病理和随访资料,并复习相关文献进行讨论.结果：3例患者术后病理检查可见上皮源性和间质源性两种恶性成分.1例癌组织CK和EMA阳性,但Vimentin阴性,肉瘤样组织Vi-mentin阳性,诊断为膀胱癌肉瘤,术后给予M-VAC方案化疗,于10个月后死于肿瘤肺部转移.2例癌组织与肉瘤样区有移行,癌组织CK和EMA阳性,但Vimentin阴性,肉瘤样组织CK、EMA及Vimentin阳性,诊断为膀胱肉瘤样癌,术后分别给予GC和M-VAC方案化疗,于8个月和11个月后死于肿瘤肺部转移和心力衰竭.结论：膀胱癌肉瘤和肉瘤样癌具有浸润性生长的生物学特性,恶性程度高,预后不良.其确诊依赖病理学及免疫组织化学检查,手术仍是首选治疗方式.     

A case of inflammatory myofibroblastic tumor of the urinary bladder finally diagnosed by anaplastic lymphoma kinase (ALK) immunostaining

A 52-year-old house wife presented with pain on urination. Cystoscopy and magnetic resonance imaging revealed solid and sessile tumor of 3 cm in diameter invading the bladder wall. Pathological examination of the transurethral resection specimen showed proliferation of spindle cells and epithelial cells. Since both types of cells were positive for cytokeratin immunostaining, sarcomatoid carcinoma was highly suspected. She underwent anterior pelvic exenteration and construction of continent reservoir (Penn Pouch). Since the tumor cells showed spindle cell proliferation alone without epithelial growth and positive staining for anaplastic lymphoma kinase, we corrected the final diagnosis as an inflammatory myofibroblastic tumor of the urinary bladder. She has been doing well without recurrence for 1 year.     

膀胱肉瘤样癌二例报告

目的探讨膀胱肉瘤样癌组织学特性，提高对膀胱肉瘤样癌和膀胱癌肉瘤病理学上区别的认识。方法报告2例膀胱肉瘤样癌病例资料。2例均为男性，年龄分别为76、59岁。均以肉眼血尿就诊，膀胱镜、CT和B超检查均诊断为膀胱肿瘤，肿瘤呈浸润性生长。术前活检均提示为移行上皮细胞癌。结果2例均行膀胱部分切除术。1例术前行新辅助化疗。术后病理可见上皮和肉瘤样问质2种恶性成分，但癌与肉瘤样区有移行，诊断为膀胱肉瘤样癌。术后均行全身化疗和膀胱灌注化疗，3个月后复查局部肿瘤复发。1例5个月后死于心脏疾病，另1例仍在随访中。结论膀胱肉瘤样癌为尿路上皮癌的一种亚型，具有呈浸润性生长的生物学特性，恶性程度高，预后不良，在制定治疗方案尤其是化、放疗方案时应充分考虑。     

A case of sarcomatoid carcinoma of the urinary bladder with brain metastasis: a case report

A 69-year-old female visited our hospital with a chief complaint of macroscopic hematuria. A non-papillary broad-based tumor was found by cystoscopy. Excretory urography and computerized tomography revealed a large invasive tumor in the left wall of the urinary bladder. The histopathological diagnosis of bladder tumor obtained by transuretheral resection (TUR-Bt) was a sarcomatoid carcinoma composed of a urothelial cell carcinoma. One month after TUR-Bt, abdominal wall metastases were found. Thus total 50Gy of irradiation therapy was performed. However brain metastasis was also found and she died 3 months after TUR-Bt.     

膀胱肉瘤样癌的诊断与治疗（附2例报告）

目的 提高对膀胱肉瘤样癌的认识和诊治水平。方法 分析2例膀胱肉瘤样癌患者的临床资料并结合文献进行讨论。2例患者均有肉眼血尿或伴有膀胱刺激症，膀胱镜检查为实体瘤，表面有坏死组织；病理表现，肿瘤主要由移行上皮癌细胞和恶性间叶细胞（梭形或多形性细胞）组成，之间可见移行过渡；免疫组化CK（＋）、CEA（＋）、SMA（＋）。结果 2例Ⅰ期治疗均行经尿道膀胱肿瘤电切，术后常规膀胱灌注，1例术后5个月死于肿瘤复发及多处转移；1例半年后复发改行部分膀胱切除，至今健在。结论 膀胱肉瘤样癌是一种高度恶性、预后差的肿瘤。确诊需依赖病理和免疫组织化学检查，早期诊断和采用膀胱部分切除或根治性切除，是改善预后的关键。     

Malignant non-urothelial neoplasms of the urinary bladder: a review

OBJECTIVES: Non-urothelial bladder tumors frequently present a diagnostic and therapeutic challenge. We review the peer-reviewed literature to summarize the available evidence on the etiology, diagnosis and optimal management of malignant non-urothelial bladder tumors. METHODS: A comprehensive MEDLINE database search was performed. In addition, the proceedings of recent national and international urological and cancer society meetings were reviewed. RESULTS: Primary non-urothelial bladder tumors are rare in Europe and North America representing less than 5% of all bladder lesions combined. A large number of risk factors have been implicated in the etiology of non-schistosomiasis-related squamous cell carcinoma, yet their exact pathomechanism remains poorly defined. Squamous cell carcinoma, adenocarcinoma, small cell carcinoma, sarcoma and carcinosarcoma/sarcomatoid tumors share an unfavorable prognosis despite aggressive surgical management that relates both to an aggressive biological behaviour as well as to an often times advanced stage at the time of diagnosis. Inflammatory pseudotumors are benign tumors of uncertain histogenesis that may mimic sarcomas. Paraganglioma, primary melanoma and lymphoma represent additional, exceedingly rare bladder tumors. CONCLUSIONS: The systematic investigation of most non-urothelial bladder tumors is limited by the rarity of these lesions. A concerted effort of multiple institutions linked together in a national or international tumor registry will be necessary to advance our understanding of these tumors, evaluate treatment strategies and optimize patient outcome in the future.     

Empleo de M-VAC en el tratamiento adyuvante del carcinoma sarcomatoide de vejiga

Sarcomatoid bladder carcinoma is a high-grade neoplasm and accounts for approximately 0,3% of all bladder malignancies. sarcomatoid carcinoma originates from transitional cells of the bladder. sarcomatoid carcinoma is charactericed by a epithelial component and a sarcomatoid component, consisting of spindle cells, that is only epithelial marker-positive. we report a 26 year-old woman diagnosed of stage iii sarcomatoid bladder carcinoma (t3an0m0) treated with partial cistectomy followed by 4 cycles of adjuvant chemotherapy with methotrexate, vinblastine, doxorubicin and cisplatin.     

Inflammatory pseudotumor of the bladder: a new case report

Inflammatory pseudotumor is a reactive benign lesion which can be very difficult to distinguish from some malignant bladder tumors like sarcomas or sarcomatoid carcinoma. Inflammatory pseudotumour (IPT) of the urinary bladder is a benign proliferate lesion which can simulate clinically and histologically a sarcoma. A case of an inflammatory pseudotumor of the urinary bladder in a 18-years-old man is presented. This patient presented with sudden onset of gross painless hematurial related to large polypoid and ulcerated bladder masses found on endoscopy. Initial pathological analysis was interpreted as rhabdomyosarcoma but subsequent reviews were consistent with a benign process resembling nodular fasciitis. This rare, benign and presumed non-neoplastic, reactive lesion must be differentiated from sarcomas of the urinary bladder. Immunohistochemistry seems to be the method of election in differentiating inflammatory pseudo-tumor from other spindle cell proliferations of the bladder. When diagnosis is certain, complete transurethral resection is the treatment of choice. However, if there is no total pathologic confirmation, if it is a very wide lesion or if it is recurring after endoscopic resection, a partial cystectomy is suggested.     

从膀胱癌患者分离并培养人膀胱平滑肌细胞

目的：建立从膀胱癌患者的分离并培养膀胱平滑肌细胞的实验技术.方法：取一小块无明显肿瘤生长的膀胱组织,分离并培养膀胱平滑肌细胞;动态观察细胞形态变化、生长增殖情况以及平滑肌肌动蛋白（SMA）、结蛋白（Desmin）和广谱细胞角蛋白（AE1/AE3）的表达.结果：接种24 h后即有长梭形细胞贴壁生长,10天后长至80%融合,呈典型的＂峰谷＂样形态;传代后1天为潜伏期,2～6天为指数生长期,然后进入融合平台期,需再次传代.第2代细胞的SMA和Desmin表达阳性率分别高达（99.0±0.8）%和（97.0±2.1）%,不表达AE1/AE3.随着传代次数的增加,细胞去分化,细胞形态变成短梭状或椭圆形;SMA和Desmin的表达开始下降,传至第5代时,SMA和Desmin阳性率分别降至（78.0±3.3）%和（74.0±2.6）%;至第7代时,SMA和Desmin阳性率降至（51.0±3.0）%和（49.0±2.6）%.第7代细胞经血清饥饿培养48 h后,细胞又能再分化,形态转变成长梭状,SMA和Desmin阳性率可分别升至（90.0±3.5）%和（88.0±2.5）%,具有显著性差异.结论：本研究所培养的人膀胱平滑肌细胞具有较高的纯度,血清饥饿能促进去分化的细胞再分化,能为构建组织工程膀胱提供种子细胞.     

'True' sarcomatoid carcinoma of the renal pelvis. First case report with immunocytochemical study

A case of sarcomatoid carcinoma of the renal pelvis is reported. The neoplasm showed polypoid configuration and was composed exclusively of plump, spindle and pleomorphic cells. Light microscopy did not reveal any epithelial differentiation of the neoplastic cells. Immunoperoxidase staining for keratins and for epithelial membrane antigen was strongly positive in the spindle elements and showed the epithelial nature of the proliferation. The present case suggests that pleomorphic tumors, when occurring in visceral organs, should be carefully sampled and immunocytochemical markers for epithelial and sarcomatous differentiation studied, before diagnosis of carcinosarcoma or sarcoma may be accepted.     

Sarcomatoid carcinoma with osseous differentiation in the bladder

INTRODUCTION: Bladder sarcomatoid carcinoma is a very rare variant of transitional cell carcinoma. With disputed nomenclature, the tumor has been described previously under a variety of names such as sarcomatoid carcinoma, pseudosarcoma, malignant mixed mesodermal/Müllerian tumor, metaplastic carcinoma and spindle cell carcinoma. This malignancy represents 0.3% of all bladder tumors and has an aggressive behavior yielding a poor prognosis despite radio and chemotherapy. CASE REPORT: An 81 y/o man presented with a transitional cell carcinoma and underwent a transurethral resection. Adjuvant onco-BCG was introduced. After 9 months of follow-up, a local tumoral recurrence occurred and a new transurethral resection revealed sarcomatoid carcinoma with osseous elements. A radical cystoprostatectomy was then carried out.