多房囊性肾细胞癌18例临床病理分析

目的：探讨多房囊性肾细胞癌（ mu1ti1ocu1ar cystic rena1 ce11 carcinoma,MCRCC）的临床病理学特征、诊断及鉴别诊断。方法回顾性分析18例MCRCC的临床病理学及免疫表型特征,并复习相关文献。结果18例中男性12例,女性6例,年龄26~68岁（平均55.6岁）。影像学检查均示多囊性占位,边界清楚。眼观：肿瘤切面见大小不等的多房囊腔,内含浆液性或血性液体。镜检：肿瘤囊内壁被覆单层透明细胞、复层上皮或上皮缺如,囊内成分大部分流失,纤维性囊壁或囊腔间隔中见灶状透明细胞,呈Ⅰ级细胞核,其为形态学诊断线索。免疫表型：透明细胞CD10、vimentin、EMA均阳性,Ki-67增殖指数低。18例患者平均随访43个月,均无复发或转移,预后良好。结论 MCRCC是一种罕见的肾细胞癌组织学亚型,预后良好,需与肾透明细胞癌囊性变及良性肾囊性病变相鉴别。     

多房囊性肾细胞癌一例

患者女 ,5 4岁。两年前无明显诱因突发肉眼血尿伴肾区绞痛 ,经抗炎、止血等对症治疗好转。此后 ,类似情况发生过 3次。多次彩超检查均提示左肾多囊肾。患者于 2 0 0 0年12月 5日在外院住院。ＣＴ示左肾内有一类圆形呈膨胀性生长且边界尚清之低密度灶 ,其密度不均 ,可见小点状钙化灶 ,ＣＴ值 3～ 98Ｈｕ不等。经增强扫描 ,病灶内可见增强之条索状影 ,条索交织成蜂窝状 ,有多个分隔的低密度灶 ,ＣＴ诊断考虑为多囊肾合并感染 ,但不除外其他占位性病变。专科检查 :双肾区无叩痛 ,不饱满 ,双输尿管行程无压痛 ,膀胱区无压痛 ,未扪及肿块。手术所…     

多囊性肾细胞癌1例报道及文献复习

目的：阐明多囊性肾细胞癌的病理形态学特征及鉴别诊断要点。方法：报道1例多囊性肾细胞癌,结合文献对本病的临床病理特征进行探讨。结果：肿瘤由厚的假纤维被膜环绕,瘤体全部由大小不等的囊腔及无膨胀性实性结节的薄的间隔构成,囊腔被覆透明细胞,异形性小,间隔内含聚集的透明细胞上皮巢,结论：多囊性肾细胞癌是一种罕见的有低度恶性倾向的肿瘤,术前影像学,细胞学检查及术后的肉眼特征均与囊性肾瘤,囊性部分分化性肾母细胞瘤相似,最重要的是本瘤缺乏肉眼所见的膨胀性实性结节,显微镜下的关键特征是间隔内可见聚集的透明细胞巢。     

多房囊性肾细胞癌一例

患者男,62岁。因体检发现右肾占位于2005年12月9日入院。患者无发热、腰痛、血尿等异常症状。B 超检查发现右肾体积增大,上极实质内见一囊实性包块,向外突起,大小约6.5cm×5.5cm,边界尚清,内可见多条分隔。CT 显示右肾上极有一6.5cm×5.8cm 的类圆形低密度影,CT 值17Hu,加强 CT 值71Hu,内见明显网状分隔。体检:腹部未扪及包块,双肾区无叩痛,双侧输尿管区无压痛。临床诊断为肾肿瘤,行右肾癌根治术,完整切除右肾送病理科检查。病理检查:切除肾脏组织一件,12.5cm×7.0cm×4.0cm,切面肾上极见一5.5cm×4.5cm 肿物,多囊性,内含淡     

乳头状肾细胞癌32例临床病理分析

目的：探讨乳头状肾细胞癌( papillary renal cell carcinoma, PRCC)的临床病理特征、免疫表型、鉴别诊断和预后。方法回顾性分析32例PRCC患者的临床和病理资料,采用免疫组化EnVision法染色,并对患者进行随访。其中21例行根治性肾切除术,11例行肾部分切除术。结果770例肾上皮性肿瘤中32例为PRCC(4.2%)。镜下见PRCC主要由多少不等的乳头状和管状结构组成,被覆单层立方或多层柱状肿瘤细胞,乳头轴心及间质内可见泡沫细胞、砂砾体沉积,部分肿瘤细胞胞质内可见含铁血黄素。Ⅰ型18例,细胞呈立方形,胞质少,嗜碱性,淡染,Fuhrman分级低级别16例;Ⅱ型14例,细胞呈高柱状,胞质丰富,嗜酸性,Fuhrman分级高级别12例。Ⅰ型和Ⅱ型PRCC不同程度地表达vimentin、EMA、CK(AE1/AE3)、CK7、CD10和AMACR,均不表达CK(34βE12)和TFE-3。31例患者获得随访,1例术后肝、肺转移,4个月后死亡,3例术后1年分别出现骨、肺、肝等处转移,2年后死亡;死亡患者中Ⅱ型3例、Ⅰ型1例。其余27例均无瘤生存。高核分级、血管内癌栓、淋巴结转移、高临床分期提示患者预后较差。结论 PRCC国内少见,具有独特的病理形态特征,Ⅱ型PRCC较Ⅰ型患者预后差。 PRCC细胞核分级高、出现肉瘤样成分或有透明细胞癌结构可能提示肿瘤具有侵袭性,预后不良。诊断时需结合病理组织学特征、免疫表型和细胞遗传学分析。     

管状囊性肾细胞癌3例并文献复习

目的 探讨管状囊性肾细胞癌(tubulocystic renal cell carcinoma, TCRCC)的临床病理特征、免疫表型、治疗和预后。方法 回顾性分析3例TCRCC的临床资料、组织学特点、免疫表型、随访和预后情况,并复习相关文献。结果 3例TCRCC中,2例男性,1例女性,平均发病年龄58.3岁。肿瘤均位于左肾,无明显临床症状。2例肿瘤分期为pT1,1例为pT3。CT或MRI均表现为多房囊性病变。镜下肿瘤完全由大小不等的小管或囊腔组成,囊壁菲薄,仅少量纤维间质分隔。肿瘤细胞呈单层排列,可扁平、立方状、柱状或鞋钉样,胞质丰富嗜酸性,细胞核大,核仁明显(WHO/ISUP核分级：3级),核分裂象少见。肿瘤细胞FH(3/3)均无表达缺失,PAX8(3/3)和P504s(3/3)强阳性,CD10(2/3)、CK7(2/3)、CK19(1/2)和EMA(1/3)部分阳性。2例患者行左肾根治性切除术,1例行肾部分切除术,患者术后随访均未出现复发及转移。结论 TCRCC罕见,大部分预后良好,其诊断标准严格,需与其他具有管状囊性结构的肾肿瘤鉴别,确诊需组织学结合免疫组化,必要时可行分子遗传...     

多囊性肾细胞癌

多囊性肾细胞癌（MCRCC）,也叫多囊性透明细胞肾细胞癌（RCC）和多囊性透明细胞癌,是一种预后较佳的少见的肾脏囊性肿瘤。2004年WHO肾肿瘤分类中将MCRCC作为透明细胞肾细胞癌的一种罕见亚型,其预后较佳。虽然文献报道囊性肾细胞癌比实体型透明细胞RCC预后好,但对于诊断MCRCC真正的临床意义和其预后还不清楚。作者根据WHO2004年的诊断标准收集了45例MCRCC,年龄30～80岁,平均54．3岁。男性28例,年龄36～80岁,平均56．3岁;女性17例,年龄30～78岁,平均51．1岁。50％的患者年龄小于50岁。肿瘤直径1—14cm,平均4．9cm,     

多房性囊性肾细胞癌2例

例1,患者女性,52岁,因无明显诱因出现右腰部不适、活动时加重半年入院.查体:一般情况好,BP:120/80 mg,右侧肾区扣击痛(±),未扪及明显肿块.B超:右肾切面形态稍大,下段背侧稍隆起,其内可见多个形态不规则的囊性暗区,内见多条强光带分隔.CT示:⑴右肾盂旁囊肿;⑵右肾下极皮质囊肿.既往有高血压病史,术中见右肾下极及肾盂输尿管旁各有1囊肿,剔除囊肿送病检.术后给予抗炎对症治疗,未行特殊处理,患者痊愈出院.随访7年未见复发或转移.     

乳头状肾细胞癌30例临床病理分析

目的：探讨乳头状肾细胞癌( papillary renal cell carci-noma, PRCC)的临床病理学特征。方法回顾性分析30例PRCC的临床资料、病理组织学及免疫表型特征,并进行随访。结果男性24例,女性6例,平均年龄55．8岁(22~82岁)。肿瘤平均直径5．2 cm(1．7~11．5 cm)。17例行根治性肾切除术,2例行肾输尿管全长切除术,11例行保留肾单位手术,病理分期：pT1aN0M0者10例,pT1bN0M0者8例, pT2aN0M0者9例, pT2bN0M0者1例, pT3bN0M0者1例, pT2aN1M0者1例;Fuhrman分级：Ⅰ级7例,Ⅱ级12例,Ⅲ级9例,Ⅳ级2例。免疫表型：26例弥漫性表达CK7,21例表达P504s,19例表达vimentin。30例PRCC均获得随访,随访时间为7~72个月,5例因病变转移死亡;1例肺转移,行靶向治疗,存活至今;其余24例均无瘤生存。结论 PRCC是一种特殊形态的少见肾癌类型,主要通过病理检查进行诊断,治疗首选手术切除,Ⅰ型预后较好。     

多房性囊性肾细胞癌的临床病理学特征和分子遗传学观察

目的 探讨多房性囊性肾细胞癌(MCRCC)的临床病理及分子遗传学改变特征.方法 对1990年1月至2006年12月间第二军医大学附属长海医院病理科病理诊断为肾细胞癌(706例)中的21例MCRCC进行了光镜、免疫表型观察和微卫星不稳定性(MSI)及杂合性缺失(LOH)检测.结果 21例MCRCC患者年龄34～72岁(平均年龄50岁),男19例,女2例.肉眼或镜下血尿3例,18例因体检而偶然发现.10例肿瘤位于左肾,11例位于右肾.T1期18例,T2期2例,T3期1例.其中1例侵犯肾包膜,均无淋巴结转移.随访20例,无一例死于肾癌,未发现肾癌转移或复发病例.组织学观察,肿块最大直径0.3～10.0 cm(平均3.6 cm).切面呈多房性囊性,囊腔大小不等,囊腔间隔厚薄不均(0.1～0.5 cm.平均0.2 cm),内含淡黄色液体或胶冻样或血性液体.囊腔内衬单层或多层上皮细胞,胞质透明或淡嗜酸性.囊腔间隔内也可见呈巢状或腺泡状排列的透明细胞.Fuhrman 1级16例,Fuhrman 2级5例.免疫组织化学观察,瘤细胞波形蛋白、ABC、CAM5.2、上皮细胞膜抗原(EMA)均呈阳性表达,CD10阳性表达6例.神经元特异性烯醇化酶(NSE)阳性表达16例.分子遗传学观察,21例标本中,PCR扩增成功的有11例,5例发现微卫星变异,3例有LOH,其中D3S1560基因座2例,D14S617基因座1例;2例有MSI,分别为D9S168和D14S617各1例.结论 MCRCC是一种少见的肾囊性肿瘤,具有独特的临床和病理学特征,肿瘤预后良好,可通过手术切除而治愈.     

229例肾细胞肿瘤的临床病理学分析

目的研究肾细胞肿瘤的临床表现、组织学分型、形态学特征及预后。方法复习229例连续肾细胞肿瘤的临床资料、大体标本、HE切片、免疫组化染色结果,按2004年WHO肾细胞肿瘤分类标准重新分类,并随访。结果229例肾细胞肿瘤占同期肾实质肿瘤89.1%(229/257),其中223例(97.4%)为肾细胞癌,6例(2.6%)为肾嗜酸细胞腺瘤。肾细胞癌中,透明细胞癌180例(78.6%),乳头状肾细胞癌19例(8.3%),嫌色细胞癌13例(5.7%),1例嫌色细胞癌伴肉瘤变,多房性囊性肾细胞癌6例(2.6%),Xp11.2易位/TFE3基因融合相关肾细胞癌2例(0.9%),未能分类的肾细胞癌2例(0.9%),集合管癌1例(0.4%)。透明细胞癌5年生存率为75.5%;乳头状肾细胞癌5年生存率为80%;嫌色细胞癌随访6例,随访期8~57个月,1例于57个月后死于肿瘤转移;多房性囊性肾细胞癌随访2~40个月,无复发和转移。结论肾细胞肿瘤是一组形态学上各有特征的异质性肿瘤,以恶性为主,其中透明细胞癌、乳头状肾细胞癌、嫌色细胞癌最常见。不同类型肾细胞癌预后不同。WHO肾细胞癌的组织学分型有其实用性和先进性。     

透明细胞（管状）乳头状肾细胞癌临床病理学分析

目的：探讨透明细胞（管状）乳头状肾细胞癌（clear cell tubulopapillary renal cell carcinoma,CCTPRCC）临床病理学特征。方法：复习2012年至2014年肾细胞癌病理学切片,筛选CCTP-RCC病例。观察CCTP-RCC巨检及镜检特征,行免疫组化染色以及分子遗传学检测。结果：肾细胞癌中CCTP-RCC占2.0%（4/201）。肿瘤平均直径2.8 cm（范围1.2~4.5 cm）。囊性、乳头状、管状以及实性生长方式出现频率分别是100%（4/4）、100%（4/4）、100%（4/4）以及75%（3/4）。4例（100%）细胞轻度异型性。Fuhrman细胞核分级2级3例（75%）,1级1例（25%）。细胞核远离基底膜的细胞占17.5%（范围10%~20%）,其他位置占82.5%（范围80%~90%）。4例（100%）见平滑肌组织,3例（75%）见发育不全血管。3例（75%）T1a,1例（25%）T1b,所有病例均为I期肿瘤。免疫组化CK7 4例（100%）弥漫强阳性;CD10 3例（75%）阴性反应,1例（25%）局灶弱阳性。分子遗传学4例FISH检测17号染色体为二倍体。平均随访时间15.5（3~23）个月,所有病例没有局部复发、淋巴结转移以及远处转移。结论：CCTP-RCC是一种少见的RCC亚型;病理诊断应结合生长方式、细胞学以及间质表现三方面综合分析;典型免疫表型是CK7弥漫强阳性,CD10阴性或局灶阳性。     

Cytologic findings of acquired cystic disease-associated renal cell carcinoma: a report of two cases

Renal tumors may arise in the setting of end-stage renal cell disease. The risk is 100 times that of the normal population with an incidence ranging from 3-7%. The most common malignant tumor to arise in the setting of acquired cystic disease of the kidney is the acquired cystic disease-associated renal cell carcinoma (ACD-associated RCC). The cytomorphologic features of ACD-associated RCC, which has not been described previously, show moderately cellular specimens composed of clusters of cells with papillary configuration. The cells ranged from polygonal to columnar and contained abundant eosinophilic granular cytoplasm. The nuclei were round and centrally located, and the chromatin was finely granular with prominent central nucleoli that corresponded to Fuhrman's grade 3 nucleolar size. The main differential diagnosis is type 2 papillary renal cell carcinoma, from which it can be distinguished based on clinical findings only at this moment.     

Uretheral invagination of multilocular cystic nephroma; a case report of a new pathologic variant

Background: The multilocular cystic nephroma (MLCN) is a unilateral cystic neoplasm of the kidney exhibiting benign biological behavior. The etiology and histopathogenesis of the disease is controversial (dysplastic/hamartomous/neoplastic). MLCNs show bimodal age distribution, with peak incidence occurring at 2-4 years of age and between the fourth and sixth decades. The male to female ratio in patients aged below 4 years is 3:1, which reverses to 1:8 between the fourth and sixth decades. Patients and methods: A 59-year-old female patient presented with left flank pain and abdominal pain. Ultrasound (US) revealed 220×109×82 mm multiple septated hyperechoic kidney cysts with a semi-solid appearance. MRI showed a 245×119×98 mm multilocular cystic renal mass in the left kidney with hypointense appearance in T1-weighted images and hyperintense in T2-weighted images, and multicystic appearance in ureter projection, the largest portion measuring 17 mm in diameter. Radical nephrectomy was planned with the pre-diagnosis of multilocular cystic nephroma or multicystic renal cell carcinoma. Results: The patient underwent transperitoneal radical nephroureterectomy. The immunohistopathological examination revealed MLCN with ureteral invagination. Conclusion: The etiology, pathogenesis, and genetic basis of multilocular cystic nephroma are currently unknown. This tumor is confused with cystic partially differentiated nephroblastoma and cystic Wilms tumor in childhood, and multilocular cystic renal cell carcinoma, clear cell papillary renal cell carcinoma, and tubulocystic carcinoma in adults. The association of this tumor with pleuropulmonary blastoma in children exhibits genetic inheritance. US control is particularly recommended in siblings of these children. Albeit rare, the disease can occur as a bilateral synchronous or metachronous lesion. There are four reports of cases with recurrence in the literature. The laparoscopic partial nephrectomy is the recommended treatment method in patients with sufficient renal reserve that are found to be free of malignancy in the frozen section examination. The symptoms of hematuria and flank pain can be associated with invagination of the cysts into the pelvis and intrarenal rupture of the cysts. The invagination of cysts into the pelvis has been previously described. The authors consider that this was the first case of MLCN in the literature exhibiting invagination into the ureter.     

Acquired cystic disease-associated renal cell carcinoma with sarcomatoid change and rhabdoid features

Acquired cystic disease (ACD)–associated renal cell carcinoma (RCC) is a recently described entity. To the best of our knowledge, there are no reports of ACD-associated RCC with sarcomatoid and rhabdoid changes. In this article, we present the first case of such a tumor. A 56-year-old Japanese man has received long-term hemodialysis and had a history of right renal cancer. Following the discovery of metastatic cancer in the thoracic wall, detailed imaging studies revealed a mass in the left kidney. The histologic examination of the left renal tumor showed ACD-associated RCC with sarcomatoid change and rhabdoid features. Immunohistochemically, intracytoplasmic globular inclusions in rhabdoid cells were positive for vimentin and cytokeratin CAM5.2. The G-band karyotype showed the following changes: 46, X, +X. −Y[1]/43, idem, add(2)(q31), −6, −9, −14, −15, +16, −22, +mar1[6]/46, XY[2]/abnormal cell[11]. In conclusion, pathologists and urologists should be aware that rhabdoid features may occur in ACD-associated RCC and that the loss of chromosomes 9 and 14 may occur during the process of sarcomatoid change in ACD-associated RCC.     

Morphological spectrum of renal cell carcinoma,unclassified: an analysis of 136 cases

Aims

Renal cell carcinoma, unclassified (RCCU) is a category that includes a morphologically and biologically heterogeneous group of tumours that are unable to be diagnosed as other well‐defined entities. We aim to describe the morphological findings of tumours within this category and to determine the most frequent morphological features leading to classification difficulty.

Methods and results

One hundred and thirty‐six cases of RCCU were examined. Patients ranged in age from 23 to 87 years. Seventy‐seven patients were men and 59 were women. International Society of Urological Pathology (ISUP) grade was most commonly 3 (n = 66), followed by 2 (n = 42) and 4 (n = 28). Tumour size ranged from 0.6 to 24.9 cm. The American Joint Committee on Cancer (AJCC) pathological T categories included pT1a (n = 50), pT1b (n = 14), pT2a (n = 7), pT2b (n = 4), pT3a (n = 50) and pT4 (n = 9). Forty‐four cases included lymph node(s), 41% of which (n = 18) had metastases. Tumours were assessed for a variety of histological features and assigned to the following morphological groups: predominantly oncocytoma/chromophobe RCC‐like; clear cell RCC‐like; papillary RCC‐like; collecting duct‐like; and pure sarcomatoid differentiation. The majority of the oncocytoma/chromophobe and clear cell RCC‐like phenotypes were low stage (pT1 or pT2). The papillary RCC‐like, collecting duct‐like and pure sarcomatoid phenotypes were mainly high stage (pT3 or pT4).

Conclusions

Renal cell carcinoma, unclassified is a term that encompasses tumours with a variety of morphological features and a wide biological spectrum. The most common source of diagnostic difficulty was tumours composed of predominantly eosinophilic cells.