多房囊性肾细胞癌1例报告

<正>多房囊性肾细胞癌(multilocular cystic renal cell carcinoma,MCRCC)是肾癌的一种特殊类型,恶性程度低,易与复杂性肾囊肿、囊性肾瘤、肾结核、肾脓肿、坏死性囊性肾癌等疾病相混淆,临床上较难鉴别。我科收治1例,报告如下。     

多房囊性肾细胞癌的临床分析及其与vonHippel-Lindau基因突变的相关性研究

目的 提高对多房囊性肾细胞癌(MCRCC)的认识及诊治水平,分析MCRCC中von Hippel-Lindau(VHL)基因的作用.方法 回顾性分析2000-2010年17例MCRCC患者资料,占同期收治512例肾癌的3.32%.男11例,女6例.年龄37～61岁,平均46岁.术前常规行B超、CT等检查,误诊为肾盂旁囊肿1例.应用PCR、PCR产物直接测序等方法分析11例MCRCC组织和相应远离病灶的正常组织中VHL基因突变的情况.结果 17例患者行根治性肾切除术14例,其中1例先行囊肿去顶术,术中囊壁组织冰冻病理检查提示透明细胞癌,改行根治性肾切除术;行肾部分切除术3例.肿瘤直径2.2～6.0 cm,平均(3.6±1.2)cm.术后病理均为MCRCC,镜下主要表现为纤维囊壁组织内衬单层或数层肿瘤细胞,核小致密,胞质透亮.TNM分期均为T1N0M0,病理分级G1 14例,G2 3例.术后随访9～36个月,平均12个月,均未见复发及转移.11例MCRCC组织中7例(64%)存在VHL基因突变,而正常组织标本均未发现VHL基因突变.结论 MCRCC作为肾细胞癌的一种少见的独立亚型,病理及临床上易漏诊或误诊.CT等影像学检查有助于术前诊断,因其预后良好,手术方式推荐保留肾单位手术.VHL基因突变与MCRCC的发生存在一定关系.

Abstract：

Objective To discuss the diagnosis and surgical management of multilocular cystic renal cell carcinoma (MCRCC) and to evaluate the gene function of the mutation of von Hippel-Lindau (VHL) gene in MCRCC. Methods Seventeen MCRCC cases (11 men and 6 women) out of 512 cases of renal cell carcinoma from 2000 to 2010 were retrospectively analyzed. The mean age of the 17 patients was 46 years (37-61 years). Ultrasonography and CT were available in all 17 cases, and 1 case was misdiagnosed as parapelvic renal cyst. The mutation of VHL gene was detected by PCR in the specimens of can-cerous tissue and adjacent normal tissue from 11 cases of MCRCC. Results Three of 17 cases underwent nephron sparing surgery, the others underwent radical nephrectomy. One case underwent unroofing of parapelvic renal cyst, but the rapid frozen pathology of the cyst wall showed renal cell carcinoma of clear type. As a result, radical nephrectomy was eventually performed. All 17 cases were confirmed as MCRCC by eva-luating pathological characteristics, such as the cyst wall lined by single or several layers of clear tumor cells and the nuclei which were small and anachromasis. Clinical stages of all cases were T1N0M0, in which there were 14 cases with pathological T1G1 and 3 cases with pathological T1G2. All patients underwent a follow-up of 9 to 36 months (mean, 12 months) without recurrence or metastasis. Mutation of VHL gene was detected in 7 of 11 cases (64%), but all adjacent normal tissues were negative. Conclusions As a rare subtype of renal cell carcinoma, MCRCC is difficult to diagnose. CT is an essential measure in diagnosis of MCRCC preoperatively. Because of the good prognosis of reported cases, nephron sparing surgery for the treatment of MCRCC is recommended. VHL gene mutations may play an important role in the carcinogenesis of MCRCC.     

多房囊性肾细胞癌影像学特征与病理学关系的研究

目的 探讨多房囊性肾细胞癌(Multilocular Cystic Renal Cell Carcinoma, MCRCC)的影像学特征与病理学特点的关系,提高诊断水平.方法 回顾性分析24例MCRCC患者的临床资料,分析影像学及病理学的特点,探讨两者之间存在的关系.结果 所有患者术前诊断肾癌18例,肾癌坏死3例,肾复杂囊肿3例;行肾部分切除术18例,肾根治性切除术6例,术后病理20例提示MCRCC,4例提示肾透明细胞癌合并MCRCC,术后随访14~120个月,平均36个月,均未见明确的肿瘤复发和转移.结论 MCRCC是一种病因不明、恶性程度低、预后良好的疾患,提高影像学检查技术并结合病理学特点,能有效提高术前确诊率.     

多房囊性肾细胞癌手术治疗的预后特点分析

目的探讨多房囊性肾细胞癌患者手术治疗的预后特点。方法回顾性分析482例肾癌患者中22例多房囊性肾细胞癌患者资料，分析其手术治疗的预后特点。多房囊性肾细胞癌占同期肾癌病例的4．56％，男女比例为2．67：1．00，年龄32～74岁，平均47岁。结果22例患者中行肾癌根治术18例，行肾部分切除术4例。肿瘤直径1．8～11．0cm，平均4．8cm。其中透明细胞癌21例，透明细胞癌与颗粒细胞癌混合型1例；pT1N0M019例，pT2N0M02例；pT3bN0M01例；G15例，G217例。失访2例，20例获随访，随访时间9～56个月，平均27．6个月。其中1例因肝硬化、上消化道出血死亡，无瘤存活21例。结论多房囊性肾癌是肾癌的一种特殊类型，多为肾偶发癌，病理分期分级低，预后与肿瘤大小无关，手术治疗效果满意，预后佳。     

多房囊性肾细胞癌的诊断与治疗

目的：探讨多房囊性肾癌的诊断和治疗,以提高术前诊断率及选择合理的治疗方法。方法：回顾性分析本院4例患者的临床资料：2例行肾切除术,另2例行肿物切除术。结果：4例均痊愈,术前误诊2例。结论：术前诊断主要依靠影像学检查,术中诊断也很重要。肾切除术是主要治疗方法,但保留肾单位手术可能更适合部分患者。     

多房囊性肾细胞癌的临床特点及预后

目的评价多房囊性肾细胞癌的诊疗特点及预后。方法回顾性分析6例多房囊性肾细胞癌的临床特点、影像学表现、外科手术方法、病理特点,随访其预后。站果男3例,女3例,肿瘤最大直径3．0～7．5cm。CT示多囊性肿物,实性部位平扫CT值30～36Hu,皮质期88～130Hu,排泄期75～96Hu,为隔膜型增强型。腹腔镜肾部分切除术1例,根治性肾切除5例。病理TNM分期,pT1aN0M0 2例,pT1bN0M03例,pT2N0M01例,癌细胞核分级均为G1。中位随访24个月,随访期内未发现远处转移及肿瘤复发,无瘤存活100％。结论多房囊性肾细胞癌为一种特殊的病理类型,病理分期、分级低,预后良好,术前怀疑或术中证实为多房囊性肾细胞癌应考虑行腔镜保留肾单位的肾部分切除术。     

囊性肾细胞癌的临床分析

1992年 1月～ 2 0 0 1年 4月 ,我院共收治肾细胞癌患者 181例 ,其中囊性肾细胞癌 12例 ,现报告如下。一、临床资料1 病例资料及诊断 :本组 12例 ,男10例 ,女 2例。年龄 14～ 76岁 ,平均 5 0岁。患者中 ,肿瘤位于左侧 8例 ,右侧 4例 ;上极 2例 ,下极 3例 ,中份 3例 ,占据肾大部分 4例。血尿 3例 ,腰痛 1例 ,1例行颅骨肿瘤切除后病理诊断为透明细胞癌 ,进一步检查发现肾原发病灶。体检 :Ｂ超发现 7例。根据Ｂｏｓｎｉａｋ[1] 肾囊性肿块分类 :Ⅰ类为单纯性囊肿 ;Ⅱ类为有少许并发症的囊性病变 ,属良性 ;Ⅲ类为有较多并发症的囊性病变 ,一些属…     

多房囊性肾细胞癌与复杂性肾囊肿的CT鉴别

目的:探讨多房囊性肾细胞癌(MCRCC)与复杂性肾囊肿(CRC)的CT特点,为疾病的鉴别诊断提供依据。方法:回顾性分析66例MCRCC与CRC患者的CT特征,包括肿物直径、分隔、钙化、结节等。结果:17.4%的MCRCC、46.5%的CRC显示钙化(P=0.038);69.5%的MCRCC、21.1%的CRC有分隔(P=0.000);26.1%的MCRCC、4.7%的CRC有结节(P=0.032);38.1%的MCRCC、10.5%的CRC有囊壁强化(P=0.029);MCRCC组肿物直径CRC组(P=0.007);logistic回归显示多分隔(P=0.011)、囊壁强化(P=0.039)为独立预测因子。结论:钙化、分隔、结节、肿物直径在两组鉴别上具有意义;多分隔、囊壁强化是两组鉴别最有价值的特征。     

成人囊性肾细胞癌——术前诊断多房囊性肾细胞癌可能吗？

作者分析了多房囊性肾细胞癌（MCRCC）临床病理和影像学特点，探讨术前诊断的标准。选取1993—2002年24例囊性成分占肿瘤主要成分的肾癌患者，复习每例组织学切片和影像学资料，依据Hartman分类标准。通过肿瘤内在生长方式和坏死变质程度将其分为MCRCC和有囊性改变的肾细胞癌（CRCC）2组，影像学上用凸出的结节、囊壁和间隔厚度作为CT和MRI诊断标准。结果显示：影像学上MCRCC表现为多房囊性占位，     

多囊性肾细胞癌

多囊性肾细胞癌 (multilocularcysticrenalcellcarcinoma ,MCRCC)是肾细胞癌的一个少见的亚类。其发病率低 ,是一种境界清晰的多囊性病变 ,囊壁为致密的纤维组织 ,囊壁内层覆以具有透明胞质的肿瘤性上皮细胞。核分级 1～ 2级。多无特征性的症状和体征 ,B超和CT能为诊断提供最直接的依据。它可以经外科手术治愈且预后良好     

Asymptomatic multilocular cystic renal cell carcinoma]

From January 1982 to December 1989, we experienced seven multilocular cystic renal cell carcinomas (MLCRCCs) in 36 asymptomatic renal cancers incidentally diagnosed, and none in 23 symptomatic renal cancers (p less than 0.05). No multiocular cystic nephromas (MLCNs) appeared in either group. Though MLCRCCs have been considered to be, in general, extremely rare, they do occur in asymptomatic renal cancers. A multiloculated renal mass with thick septum discovered in an adult by ultrasonography or CT scan should be suspected as being a MLCRCC rather than a MLCN.     

Bilateral multilocular cystic renal cell carcinoma]

A case of bilateral renal cell carcinoma with multilocular cysts is described. A 42-year-old female was admitted to our hospital for the purpose of further examination or right renal cystic mass discovered incidentally by ultrasonography. After she was examined by CT-scan and angiography, right nephrectomy was performed under the diagnosis of right renal cancer. Histological diagnosis was multilocular cystic renal cell carcinoma. One year later a small cystic legion was found in the left kidney. Under the diagnosis of left renal cancer, left partial nephrectomy was performed. Histological diagnosis was multilocular cystic renal cell carcinoma same as the right kidney. Multilocular renal cyst is classified into several classes by its style of development. This case of bilateral renal cell carcinoma which grew to be multilocular cysts is the first case in Japan as far as we can search.     

多房性囊性肾癌与肾癌出血坏死囊性变的临床比较

目的:探讨多房性囊性肾癌(MCRCC)与肾癌出血坏死囊性变(NCRCC)的临床特点,提高两种疾病的诊治水平。方法:对MCRCC及NCRCC各10例的临床资料进行对比研究,并对其囊壁增厚、强化特点等CT征象以及病理分级进行统计学分析。结果:两组患者无特异性,NCRCC组最重要的CT征象为囊壁及分隔局部结节或肿块,与MCRCC组相比差异有统计学意义(P<0.01)。MCRCC组病理分级低,与NCRCC组相比差异有统计学意义(P<0.05)。结论:MCRCC与NCRCC是两种生物学行为不同的肾癌类型,应注意鉴别,鉴别主要依靠影像学检查。MCRCC恶性程度低,预后较好。     

A case of renal cell carcinoma suspected of multilocular cystic nephroma

A 52-year-old, healthy looking woman was referred for further evaluation of left renal mass, She had had a history of hematuria for ten years. Admission laboratory studies were within normal limits except for slight elevation of white blood cell count, c-reactive protein, blood sedimentation rate and immunosuppressive acidic protein. Retrograde pyelography demonstrated a large mass with ringed calcification in the middle lobe of the left kidney. The multiloculated mass was also confirmed by computed tomography and ultrasonography. Selective left renal arteriography showed stretched arteries and irregularity and tortuosity of the smaller vessels. Under a presumptive diagnosis of multilocular cystic nephroma or renal cell carcinoma, left radical nephrectomy was performed. In surgical specimen, many lobules were filled with serous fluid and clotted blood. Microscopic examination revealed that the lining of the cyst wall consisted of renal cell carcinoma cell. At present, 5 months after the operation, she is well without any signs or symptoms of recurrence.     

Synchronous bilateral multilocular renal cell carcinoma

A rare case of multilocular primary renal cell carcinoma (two sites in each kidney) in a 48-year-old woman is presented. The preoperative diagnosis was based on the computerized tomography findings. The patient underwent bilateral total nephrectomy and subsequently maintained renal dialysis.     

Cystic renal cell carcinoma arising from multilocular cystic nephroma of the same kidney

Multilocular cystic nephroma is an uncommon benign entity grouped among the cystic non-genetic diseases. It is characterized by variable-sized, non-communicating cysts separated by irregular thin walled septa. Though multilocular cystic nephroma is usually considered a benign lesion, malignant changes in the cysts should not be overlooked.     

Renal cell carcinoma presenting as multilocular cystic mass

We report a case of a multilocular cystic mass that was erroneously labeled multilocular cystic nephroma at ultrasound and computerized tomography, thus leading to a partial nephrectomy; however the final histologic diagnosis was a multilocular cystic renal cell carcinoma. Only 1 similar case has been reported previously. The radiologic and surgical implications are discussed.