原发性肾滑膜肉瘤一例报告并文献复习

目的 总结原发性肾滑膜肉瘤的临床表现、病理特点和诊治方法.方法 原发性肾滑膜肉瘤患者1例,男,55岁.因右腰背及上腹部疼痛5 h入院.无尿频、尿急、尿痛、血尿、发热等症状.CT检查示右肾中下极恶性占位可能,累及右肾门、肾动静脉及肝脏.行右肾肿瘤根治性切除术.结果 病理检查:肿瘤大小12.5 cm×11.0 cm×9.0 cm.镜下瘤细胞呈梭形、束状排列,细胞异型,可见核分裂相,部分区域见增生的薄壁血管,肿瘤呈浸润性生长,伴发炎症及大量坏死、出血.免疫组化染色示Vimentin、CD99、Bcl-2阳性,CK阴性.病理诊断为原发性肾滑膜肉瘤.患者术后2个月出现局部复发及肝肺转移,予异环磷酰胺、表阿霉素化疗1疗程,患者不能耐受.术后8个月死亡.结论 原发性肾滑膜肉瘤是一种罕见、高度恶性的肿瘤,预后不良,确诊需依赖病理检查、免疫组织化学及RT-PCR检测.根治性切除辅以化学治疗可行.     

原发性肾滑膜肉瘤一例报告并文献复习

Objective To discuss the presentations, pathologic features, diagnosis and treatment of primary synovial sarcoma of the kidney. Methods One case of primary synovial sarcoma of the kidney was reported and the relevant literature was reviewed. A 55-year-old man was admitted with complaint of right abdomen and flank pain for 5 h. Computerized tomography revealed a 12.5 cm × 11.0 cm × 9. 0 cm mass located at the middle and lower pole of the right kidney. The patient was taken radical nephrectomy. Results The diagnosis of primary synovial sarcoma of the kidney in the patient was confirmed by postoperative pathology. Under microscope, tumor was typically mitotically active, monomorphic spindle cells growing in intersecting fascicles or in solid sheets with epithelial differentiation. In some areas a haemangiopericytoma-like pattern was found. Immunohistochemical staining showed that the tumor cells were positive for the markers Vimentin, CD99 and Bcl-2, but CK was negative. The patient died of local recurrence and multi-metastasis at 8 months after surgery. Conclusions Primary synovial sarcoma of the kidney is extremely rare with a high grade of malignancy,and its prognosis is poor. The diagnosis depends on pathological features, Immunohistochemical studies and RT-PCR detection. Radical resection combined with chemicaltherapy is considered to be the most reliable treatment so far.     

原发性肾滑膜肉瘤一例报告并文献复习

Objective To discuss the presentations, pathologic features, diagnosis and treatment of primary synovial sarcoma of the kidney. Methods One case of primary synovial sarcoma of the kidney was reported and the relevant literature was reviewed. A 55-year-old man was admitted with complaint of right abdomen and flank pain for 5 h. Computerized tomography revealed a 12.5 cm × 11.0 cm × 9. 0 cm mass located at the middle and lower pole of the right kidney. The patient was taken radical nephrectomy. Results The diagnosis of primary synovial sarcoma of the kidney in the patient was confirmed by postoperative pathology. Under microscope, tumor was typically mitotically active, monomorphic spindle cells growing in intersecting fascicles or in solid sheets with epithelial differentiation. In some areas a haemangiopericytoma-like pattern was found. Immunohistochemical staining showed that the tumor cells were positive for the markers Vimentin, CD99 and Bcl-2, but CK was negative. The patient died of local recurrence and multi-metastasis at 8 months after surgery. Conclusions Primary synovial sarcoma of the kidney is extremely rare with a high grade of malignancy,and its prognosis is poor. The diagnosis depends on pathological features, Immunohistochemical studies and RT-PCR detection. Radical resection combined with chemicaltherapy is considered to be the most reliable treatment so far.     

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.     

肾平滑肌肉瘤1例报告并文献复习

目的：探讨肾平滑肌肉瘤（LMSK）的临床特点和诊治方法.方法：回顾性分析1例LMSK患者的临床资料,并结合文献复习讨论LMSK的临床表现、病理特点、诊治方法和预后.患者为女性,主要表现为腰部阵发性疼痛8天,B超和CT提示左肾占位性病变.行根治性肾切除术.结果：病理检查肿瘤直径6 cm,光镜下见肿瘤细胞主要为平行束状或交织束状排列的嗜酸性梭形细胞,胞核钝圆,胞浆嗜酸性.免疫组织化学染色α-平滑肌肌动蛋白（α-SMA）和结蛋白（Des）阳性,细胞角蛋白（CK）和黑色素瘤抗体（HMB-45）阴性.TNM分期为T2b N0M0,分级为G2.患者术后5个月死于局部肿瘤复发和肺转移.结论：LMSK临床少见,恶性程度高,预后差.临床表现和影像学检查无特异性表现,通常为术后病理检查确诊.手术为主要治疗手段,辅助化疗和放疗效果尚有争议.     

膀胱平滑肌肉瘤3例报告并文献复习

目的 探讨膀胱平滑肌肉瘤的临床病理和诊治方法.方法 患者3例,肉眼血尿,膀胱镜检见肿瘤分别发生于膀胱左侧壁、左前壁和右后壁,直径2.0～3.5 cm,以菜花样和乳头样为主,基底较宽,瘤体表面光滑.结果 3例均行全膀胱切除术,病理表现为上皮下间质富于黏液,黏液中可见散在或束状分布的细长梭形瘤细胞,其胞质淡染,核呈长梭形,有轻度异型,核分裂象易见.免疫组化SMA(+)3例、MSA(+)3例、Des(+)2例、S-100(-)2例、CD34(-)3例,均诊断为膀胱低级别平滑肌肉瘤.随访6个月～8年,1例术后2.5年死亡,2例存活.结论 膀胱平滑肌肉瘤临床罕见,确诊依赖病理及免疫组化检查,应与平滑肌瘤、横纹肌肉瘤、癌肉瘤、炎性肌纤维母细胞瘤等鉴别,治疗以手术为主,需根据肿瘤分级、分期决定手术方案及术后是否需行辅助治疗.     

原发性肾滑膜肉瘤一例报告并文献复习

目的探讨原发性肾滑膜肉瘤的临床特点及诊治方法。 方法回顾性分析我院原发性肾滑膜肉瘤1例的临床资料,并对国内外相关文献进行回顾。 结果患者接受根治性左肾切除术,术后病理回报为原发性肾滑膜肉瘤。 结论原发性肾滑膜肉瘤非常罕见,无特异性临床特点及影像表现,诊断主要依靠病理及免疫组化,SYT-SSX融合基因有提示作用,主要治疗方案为手术,化疗对生存率的影响还需进一步研究。     

附睾原发性平滑肌肉瘤1例报告并文献复习

患者 男,15岁.发现右侧阴囊无痛性肿块2个月在当地医院行附睾肿块切除术,术后病检报告为恶性肿瘤.术后20d到我院就诊,重新行标本切片检查和酶标测定诊断为平滑肌肉瘤.     

肾脏原发性淋巴瘤一例报告并文献复习

目的 提高肾脏原发性淋巴瘤的诊治水平.方法 回顾性分析1例肾脏原发性淋巴瘤患者的临床病理资料,结合文献复习讨论.患者,女,61岁.因左侧腰痛2周入院.查体:左上腹压痛,左肾区叩击痛.B超检查见左肾正常结构未显示,集合系统分离,最宽处约1.0 cm,左肾区可探及一巨大低回声肿物,大小9.7cm×5.3cm,边界模糊.CT检查见左肾中下极肿物,大小9.8 cm×8.9 cm ×8.8 cm,边界不清,平扫CT值为39 HU,增强后为61 HU,强化不明显.术前诊断:左肾恶性肿瘤.结果 患者行根治性左肾切除术.病理报告:左肾大部分被肿瘤组织占据,中下极见10.0cm×9.5 cm ×8.5 cm类圆形肿物,质硬,肿物切面呈灰白色,质地细腻.镜下瘤细胞呈弥漫性浸润,卵圆形或多边形,略大于正常淋巴细胞,核大深染呈不规则形.病理诊断:肾弥漫性大B细胞性淋巴瘤.骨髓穿刺检查未见异常.行环磷酰胺+吡喃阿霉素+长春新碱+泼尼松+利妥昔单抗方案化疗6周期.术后每3个月复查胸部X线片、腹部B超及CT等.随访20个月,未见肿瘤复发.结论 肾脏原发性淋巴瘤临床罕见,症状与肾癌相似,影像学检查无特征性改变,确诊需依靠病理学检查.本病预后不良,手术切除患肾联合规范的全身化疗,可延长患者的生存期.     

肾脏后肾腺瘤1例报告并文献复习

目的：探讨后肾腺瘤的临床病理学特征,提高对后肾腺瘤的认识.方法：分析1例29岁女性后肾腺瘤患者的临床病理资料,并结合相关文献进行讨论.结果：患者因腰部不适行B超检查发现右肾肿瘤,16层螺旋CT诊断为右肾肿瘤（良性可能大）,行肾肿瘤剜除术,术中见肿瘤被膜完整,切面色灰白,质地均匀,术中冷冻切片诊断为乳头状肾细胞癌,随即行肾癌根治术.镜下肿瘤细胞呈腺样或乳头状排列,并有砂粒体.免疫组化：NSE（＋）、Ki67、Vim为阳性.病理诊断：右肾后肾腺瘤.随访22个月,未见肿瘤复发.结论：后肾腺瘤术前难以依据影像学特征明确诊断,术前肿瘤穿刺活检结合免疫组化对明确诊断可能是一种可行的选择,更重要的是,了解其临床及病理特点有助于该肿瘤的准确诊断.后肾腺瘤的治疗多采取保留肾单位的手术,但由于其生物学行为及细胞起源的不确定性,术后长期随访观察是必需的.     

肾脏尤文氏肉瘤1例报告并文献复习

目的探讨肾脏尤文氏肉瘤的临床诊治及免疫组化特点。方法分析1例原发性肾脏尤文氏肉瘤的临床资料,复习文献总结肾脏尤文氏肉瘤的特点。结果在全身麻醉下进行右肾癌根治术。肿瘤免疫组化标记结果：Vimentin＋＋＋、CD99＋＋、CD56＋＋、Ki-67＋、Calponin＋/-;SMA、Desmi、CK7、myoylobin、S-100、NSE、CgA、Syn、CD20、HMB45等为阴性。术后进行化疗和放疗。结论肾脏尤文氏肉瘤源于神经外胚层恶性软组织肿瘤,有较高的侵袭性,预后较差,手术切除应为首选治疗方案,术后配合放疗和化疗。     

骨原发尤文肉瘤预后相关因素的分析

目的 明确骨原发尤文肉瘤患者的治疗结果,分析影响预后相关因素.方法 回顾性分析1998年7月至2007年7月就诊的78例骨原发尤文肉瘤患者的临床资料.通过随访了解肿瘤学预后,明确5年总体生存率及局部复发率.通过回顾病例,分析年龄、性别、肿瘤部位、肿瘤大小、初次诊断时有无转移、乳酸脱氢酶、碱性磷酸酶水平、治疗方式、外科边界等因素对总体生存率的影响.利用Kaplan-Meier生存分析确定对生存有影响的单个因素,并通过Cox回归分析明确影响预后的独立危险因素.结果 53例患者获得随访,中位随访时间37.6个月(8.0～101.0个月).患者5年总体生存率为33.7%,局部复发率20.8%,中位复发时间20个月.单因素分析结果显示:年龄＜20岁、初诊时元转移、病变位于四肢、肿瘤直径＜10 cm、取得广泛外科边界的患者预后较好(P＜0.05);多因素分析显示初诊时转移情况、肿瘤部位、肿瘤直径是影响预后的独立危险因素.结论 初诊时有无转移、肿瘤的部位和大小是影响骨尤文肉瘤预后的独立危险因素.     

儿童阴茎原始神经外胚层肿瘤/尤文氏瘤1例并文献复习

目的:探讨原发于阴茎的原始神经外胚层肿瘤/尤文氏瘤(PNET/Ewing's sarcoma)的临床表现、病理特点、治疗方法。方法:分析本院收治的1例5岁患儿原发于阴茎的PNET/EWS患者的病例资料进行文献复习。结果:病理表现为小圆细胞恶性肿瘤,免疫组化结果显示CD99(+),分子生物学检查:EWS荧光原位杂交可见易位,符合原始神经外胚层肿瘤/尤文氏肉瘤诊断,该患者经过病理确诊为阴茎PNET/EWS,经45周化疗和局部放疗,瘤灶缩小。目前停药3个月。结论:原发阴茎PNET/EWS极罕见,临床症状无特异性,以阴茎增大伴疼痛为主,免疫组化及分子生物学检查有助于对PNET/EWS的确诊。     

肾原发性滑膜肉瘤1例报告及文献复习

目的提高对肾原发性滑膜肉瘤的诊治水平。方法回顾性分析1例晚期肾原发性滑膜肉瘤的诊治经过,并报告采用索拉非尼治疗其肺转移灶的经验。结果本例临床和影像学表现无特异性,患肾切除术后常规病理检查误诊为肾母细胞瘤,经免疫组化检查及联合病理会诊,确诊为肾原发性滑膜肉瘤(梭形细胞型)。采用依托泊苷、异环磷酰胺、顺铂化疗联合索拉非尼靶向药物治疗其肺部转移灶,获得部分缓解,但索拉非尼单药治疗及免疫治疗效果不佳。疾病控制期为6个月,生存期11个月。结论肾原发性滑膜肉瘤诊断困难,对肿瘤标本进行免疫组化或分子学分析方可确诊。一旦发生转移,预后不佳,联合采用以高剂量异环磷酰胺为基础的化疗和索拉非尼靶向药物治疗可能获得一定疗效。     

Extraskeletal Ewing's sarcoma: a case report and review of the literature

STUDY DESIGN: Case report. OBJECTIVES: To report on the diagnosis and current treatment of a rare tumor about the cervical spine. SUMMARY OF BACKGROUND DATA: Extraskeletal Ewing's sarcoma (EES) is rare and has not been previously described about the cervical spine. We present a case of a 24-year-old man with a large mass in the posterior triangle of the neck extending through the vertebral foramens of the cervical vertebrae. This was identified as an extraskeletal Ewing's sarcoma. Traditional treatment paradigms have been associated with a poor prognosis. Since the recommendations of the Intergroup Rhabdomyosarcoma Study II study of multimodal chemotherapy and radiotherapy, this tumor has a significantly better prognosis. METHOD: Surgical debulking of the tumor was necessary to relieve cord compression. Histologic analysis was used to confirm both magnetic resonance imaging and computed tomography diagnosis. A chemoradiation therapy program was commenced in accordance with Intergroup Rhabdomyosarcoma Study II recommendations. RESULTS: Computed tomography and magnetic resonance imaging demonstrated a large lobulated mass extending through the exit foramens of C2/C3 and C3/C4. The mass was entirely extraskeletal and extradural. Histologic examination of the excised mass showed microscopy consistent with extraskeletal Ewing's sarcoma. After surgical debulking and chemoradiation, the patient made a complete recovery. CONCLUSION: A review of the literature confirms that extraskeletal Ewing's sarcoma is a rare tumor and particularly so in the region of the cervical spine. Early diagnosis and surgical debulking combined with current multimodality chemoradiation programs can produce a favorable outcome.     

成人原发性肾肉瘤的诊治及预后

目的探讨成人原发性肾肉瘤的诊断、治疗以及影响预后的因素。方法1996年至2005年收治成人原发性肾肉瘤15例，占同期收治794例肾肿瘤患者的1．9％。男6例，女9例。年龄36～68岁，平均50岁。临床表现为腹部肿块10例，腹部疼痛2例，健康体检发现后腹膜占位2例，肉眼血尿1例。肿瘤直径6．8～22．5cm，平均13．4cm，均为单发。临床分期Ⅱ期6例、Ⅲ期5例、Ⅳ期4例。15例影像学表现具有肾恶性肿瘤的特征，但与肾癌或错构瘤鉴别困难。结果11例行根治性肾切除术，4例因无法根治而行单纯肾切除术或肿瘤部分切除术。术后病理诊断为平滑肌肉瘤6例、脂肪肉瘤3例、低分化肉瘤3例、纤维肉瘤2例、滑膜肉瘤1例。13例随访3个月～8年，死亡10例，生存最长者为8年（脂肪肉瘤），最短者为3个月（术前已肝转移）。11例接受根治性肾切除术者平均生存期33个月，4例行单纯肾切除术或肿瘤部分切除术者平均生存期8个月；平滑肌肉瘤组平均生存期22个月，脂肪肉瘤组为46个月，低分化肉瘤组为15个月。结论腹部肿块是成人原发性肾肉瘤最常见的临床表现，是否可以进行根治性手术、病理类型是影响预后的重要因素。