多房性囊性肾细胞癌的临床病理特点

目的 探讨多房性囊性肾细胞癌(MCRCC)的临床病理特点及与其他肾囊性疾病的鉴别诊断。方法 收集19例MCRCC的临床病理资料,并与同期诊断的其他肾囊性疾病46例(包括透明细胞肾细胞癌伴广泛囊性变12例、透明细胞乳头状肾细胞癌6例、管状囊性癌2例、单纯性囊肿22例、囊性肾瘤1例和多囊肾3例)进行形态学和免疫组织化学比较。结果 19例患者中男性14例,女性5例,平均年龄50岁。临床上多因体检发现,偶有血尿或腰痛等症状。17例获随访,时间6～170个月,均未见复发和转移。病理检查：肿瘤直径1.5～7.0 cm,边界大多清楚,切面均呈多房囊性或蜂窝状,囊内含清亮液体、血液或浑浊液体,内壁光滑,不见实性区域或大体可见的壁结节。镜下观察：囊腔衬覆单层胞质透明、核小、无核仁、Fuhrman核分级Ⅰ级瘤细胞,少数(5例)为Ⅱ级,部分囊壁上皮增生为多层,个别囊内有小乳头形成。大多数病例纤维间隔内出现与囊腔衬覆细胞相似的透明细胞团,14例肌纤维母细胞增生显著。免疫组织化学染色：囊腔内衬上皮及间隔内透明细胞团对上皮性标志物广谱角蛋白、上皮细胞膜抗原和CK7均高表达,阳性比例分别为19/19、16/19和15/19;碳酸酐酶Ⅸ(CAⅨ)和PAX8分别在17/19和15/19的病例表达;CD10、肾细胞癌标志物(RCC)和α-甲酰基辅酶A消旋酶较少表达,阳性比例分别为7/19、6/19和2/19;所有病例对34βE12、CD117和CD68均阴性。结论 多房性、纤维间隔内核分级低的透明细胞团并肌纤维母细胞及囊壁上皮下微血管增生是MCRCC的重要形态学特征;CAⅨ、CK7阳性,CD10、RCC多阴性是MCRCC的重要免疫表型,CAⅨ有助于肾脏各种良恶性囊性病变的鉴别诊断;MCRCC是一种预后极好的低度恶性囊性肾肿瘤。     

获得性囊性肾病相关性肾细胞癌临床病理学观察

目的 探讨获得性囊性肾病相关性肾细胞癌(acquired cystic kidney disease-associated renal cell carcinoma,ACKD-RCC)的临床病理特点、免疫表型、组织发生及预后。 方法 收集2010年至2017年解放军第四一医院2例和青岛大学附属医院1例ACKD-RCC患者的临床资料,进行组织形态学观察和免疫组织化学分析,并复习相关文献。 结果 3例均为男性,年龄46～78岁,均有慢性肾功能衰竭病史,2例曾行血液透析分别9年和11年。大体上2例瘤组织边界清楚,直径2.5和3.5 cm,1例肾脏广泛出血,肿瘤不明显。镜下观察：瘤组织主要呈筛状、微囊状或腺泡状排列,1例局部可见多量乳头状结构,3例均有程度不等的出血,2例坏死明显,瘤细胞胞质丰富、嗜酸性,边界不清,核圆形或椭圆形,核仁明显[WHO/国际泌尿病理协会(ISUP)核分级3级],核分裂象少见,2例肿瘤内可见多量草酸盐结晶。3例肿瘤周围肾实质均显示萎缩,肾小管不同程度囊状扩张,部分衬覆异型增生的肾小管上皮细胞。免疫组织化学染色：3例肿瘤均弥漫强阳性表达CD10、肾细胞癌标志物(RCC)、P504s、波形蛋白、CAM5.2和mitochondria;上皮细胞膜抗原(EMA,2/3)、细胞角蛋白7(CK7,1/3)、碳酸酐酶Ⅸ(CA9,1/3)和PAX8(3/3)等不同程度表达;而CD117、HMB45、Melan A和TFE3均阴性。3例随访3～14个月,除1例术后6个月死于肾功能衰竭外,另2例均存活,无复发和转移。 结论 ACKD-RCC是一种非常少见的肾细胞癌,依据患者有ACKD的病史以及形态学上独特的筛状结构、胞质嗜酸性、核仁明显(WHO/ISUP核分级3级)的癌细胞以及癌组织内出现特征性的草酸盐结晶沉积,可明确诊断。该肿瘤组织起源于肾近段小管,其发生可能与ACKD不典型囊肿上皮细胞增生癌变有关,其生物学行为大多属低度恶性,但有肉瘤样分化时预后较差。     

肾混合性上皮间质肿瘤和成人囊性肾瘤的临床病理学观察

目的 探讨肾混合性上皮间质肿瘤(MEST)和成人囊性肾瘤的临床病理学特点、免疫表型和鉴别诊断.方法 通过HE和免疫组织化学染色(EnVision法)分析5例MEST和4例囊性肾瘤,并复习有关文献.结果 5例MEST均为女性,中位年龄45岁;囊性肾瘤中男性3例,女性1例,中位年龄41岁;临床表现为腰痛或血尿.大体观察:MEST界清无包膜,切面未见明显出血坏死,其中3例旱实性,1例呈囊实性,另1例呈多囊性其间为厚的纤维分隔;囊性肾瘤有包膜,切面呈多囊性,囊壁薄,无实区和坏死厌.镜下观察:MEST由不等量增生、囊性扩张的腺上皮与不同排列方式的梭形间质细胞混合组成,两种细胞成分无明显异形,2例局部衬覆子宫内膜样或输卵管样上皮;囊性肾瘤为多房囊腔组织,囊壁薄,内衬单层上皮.免疫表型:9例上皮细胞CKpan、上皮细胞膜抗原(EMA)均呈阳性表达;MEST间质梭形细胞波形蛋白(5/5)、平滑肌肌动蛋白(SMA,3/5)、结蛋白(4/5)、CD10(5/5)、ER(4/5)和PR(4/5)呈阳性表达,HMB45、CD34、CD117和S-100蛋白呈阴性;囊性肾瘤间质成分波形蛋白(4/4)、SMA(4/4)、结蛋白(1/4)阳性,ER(3/4)和PR(1/4)少量细胞阳性,CDIO、HMB45、CD34、CDll7和S-100蛋白呈阴性.结论 (1)MEST和囊性肾瘤均是少见的肾脏肿瘤,大多为良性.(2)MEST间叶细胞呈不同程度的平滑肌或肌纤维母细胞分化;2例有MaUerian管上皮分化特征.(3)MEST和成人囊性肾瘤在形态学和免疫表型上有很多相似性,可能为位于同一肿瘤谱系两端的肿瘤.     

肾混合性上皮间质肿瘤和成人囊性肾瘤的临床病理学观察

Objective To study the clinicopathologie features,immunophenotype and differential diagnosis of mixed epithelial and stromal tumor of kidney ( MEST) and adult cystic nephroma ( CN).Methods Five cases of MEST and 4 cases of CN were retrospectively analyzed.Immunohistochemical study was carried out and the literature was reviewed.Results All of the five patients with MEST were females.Their median age was 45 years.For CN,there were 3 males and 1 female and their median age was 41 years.All patients presented with loin pain and hematuria.On gross examination,MEST was well-circumscribed but non-encapsulated.There was no evidence of haemorrhage or necrosis.Three of the cases were solid in nature.One was composed of a mixture of solid and cystic elements,while the remaining case showed a multicystic cut surface bridged by thick fibrous septa.On the other hand,CN were well-circumscribed and encapsulated.They were multiloculated cystic in nature.The cystic spaces were separated by thin septa and there was no significant solid or necrotic component.Histologically,MEST consisted of proliferation of cystically dilated glands admixed with spindly stromal cells with various cellularity and growth patterns.Both the glandular and stromal elements were well-differentiated with no cytologic atypia identified.The glandular structures in 2 of the cases were partially lined by endometrial or tubal epithelium.In contrast,the thin-walled cystic spaces in CN were lined by a single layer of epithelium.Immunohistochemical　study showed that the epithelial cells were positive for pan-cytokeratin and epithelial membrane antigen.The spindle cells in MEST expressed vimentin (5/5 ) ,smooth muscle actin (3/5 ),desmin (4/5 ),CD10 (5/5),estrogen receptor (4/5) and progesterone receptor (4/5).They were negative for HMB45,CD34,CD117 and S-100 protein.On the other hand,the spindle cells in CN were variably positive for vimentin (4/4),smooth muscle actin (4/4),desmin (1/4),estrogen receptor (3/4) and progesterone receptor (1/4).They were negative for CD10,HMB45,CD34,CD117 and S-100 protein.Conclusions Both MEST and CN are uncommon renal neoplasm.Most of them run a benign clinical course.The stromal cells in MEST show smooth muscle or myofibroblastic differentiation.Areas demonstrating Miillerian features also existed in some cases.MEST and CN share overlapping histological and immunohistochemical features,and may represent spectrum of the same group of lesions.     

肾混合性上皮间质肿瘤和成人囊性肾瘤的临床病理学观察

Objective To study the clinicopathologie features,immunophenotype and differential diagnosis of mixed epithelial and stromal tumor of kidney ( MEST) and adult cystic nephroma ( CN).Methods Five cases of MEST and 4 cases of CN were retrospectively analyzed.Immunohistochemical study was carried out and the literature was reviewed.Results All of the five patients with MEST were females.Their median age was 45 years.For CN,there were 3 males and 1 female and their median age was 41 years.All patients presented with loin pain and hematuria.On gross examination,MEST was well-circumscribed but non-encapsulated.There was no evidence of haemorrhage or necrosis.Three of the cases were solid in nature.One was composed of a mixture of solid and cystic elements,while the remaining case showed a multicystic cut surface bridged by thick fibrous septa.On the other hand,CN were well-circumscribed and encapsulated.They were multiloculated cystic in nature.The cystic spaces were separated by thin septa and there was no significant solid or necrotic component.Histologically,MEST consisted of proliferation of cystically dilated glands admixed with spindly stromal cells with various cellularity and growth patterns.Both the glandular and stromal elements were well-differentiated with no cytologic atypia identified.The glandular structures in 2 of the cases were partially lined by endometrial or tubal epithelium.In contrast,the thin-walled cystic spaces in CN were lined by a single layer of epithelium.Immunohistochemical　study showed that the epithelial cells were positive for pan-cytokeratin and epithelial membrane antigen.The spindle cells in MEST expressed vimentin (5/5 ) ,smooth muscle actin (3/5 ),desmin (4/5 ),CD10 (5/5),estrogen receptor (4/5) and progesterone receptor (4/5).They were negative for HMB45,CD34,CD117 and S-100 protein.On the other hand,the spindle cells in CN were variably positive for vimentin (4/4),smooth muscle actin (4/4),desmin (1/4),estrogen receptor (3/4) and progesterone receptor (1/4).They were negative for CD10,HMB45,CD34,CD117 and S-100 protein.Conclusions Both MEST and CN are uncommon renal neoplasm.Most of them run a benign clinical course.The stromal cells in MEST show smooth muscle or myofibroblastic differentiation.Areas demonstrating Miillerian features also existed in some cases.MEST and CN share overlapping histological and immunohistochemical features,and may represent spectrum of the same group of lesions.     

肾混合性上皮间质肿瘤和成人囊性肾瘤的临床病理学观察

Objective To study the clinicopathologie features,immunophenotype and differential diagnosis of mixed epithelial and stromal tumor of kidney ( MEST) and adult cystic nephroma ( CN).Methods Five cases of MEST and 4 cases of CN were retrospectively analyzed.Immunohistochemical study was carried out and the literature was reviewed.Results All of the five patients with MEST were females.Their median age was 45 years.For CN,there were 3 males and 1 female and their median age was 41 years.All patients presented with loin pain and hematuria.On gross examination,MEST was well-circumscribed but non-encapsulated.There was no evidence of haemorrhage or necrosis.Three of the cases were solid in nature.One was composed of a mixture of solid and cystic elements,while the remaining case showed a multicystic cut surface bridged by thick fibrous septa.On the other hand,CN were well-circumscribed and encapsulated.They were multiloculated cystic in nature.The cystic spaces were separated by thin septa and there was no significant solid or necrotic component.Histologically,MEST consisted of proliferation of cystically dilated glands admixed with spindly stromal cells with various cellularity and growth patterns.Both the glandular and stromal elements were well-differentiated with no cytologic atypia identified.The glandular structures in 2 of the cases were partially lined by endometrial or tubal epithelium.In contrast,the thin-walled cystic spaces in CN were lined by a single layer of epithelium.Immunohistochemical　study showed that the epithelial cells were positive for pan-cytokeratin and epithelial membrane antigen.The spindle cells in MEST expressed vimentin (5/5 ) ,smooth muscle actin (3/5 ),desmin (4/5 ),CD10 (5/5),estrogen receptor (4/5) and progesterone receptor (4/5).They were negative for HMB45,CD34,CD117 and S-100 protein.On the other hand,the spindle cells in CN were variably positive for vimentin (4/4),smooth muscle actin (4/4),desmin (1/4),estrogen receptor (3/4) and progesterone receptor (1/4).They were negative for CD10,HMB45,CD34,CD117 and S-100 protein.Conclusions Both MEST and CN are uncommon renal neoplasm.Most of them run a benign clinical course.The stromal cells in MEST show smooth muscle or myofibroblastic differentiation.Areas demonstrating Miillerian features also existed in some cases.MEST and CN share overlapping histological and immunohistochemical features,and may represent spectrum of the same group of lesions.     

肾混合性上皮间质肿瘤和成人囊性肾瘤的临床病理学观察

Objective To study the clinicopathologie features,immunophenotype and differential diagnosis of mixed epithelial and stromal tumor of kidney ( MEST) and adult cystic nephroma ( CN).Methods Five cases of MEST and 4 cases of CN were retrospectively analyzed.Immunohistochemical study was carried out and the literature was reviewed.Results All of the five patients with MEST were females.Their median age was 45 years.For CN,there were 3 males and 1 female and their median age was 41 years.All patients presented with loin pain and hematuria.On gross examination,MEST was well-circumscribed but non-encapsulated.There was no evidence of haemorrhage or necrosis.Three of the cases were solid in nature.One was composed of a mixture of solid and cystic elements,while the remaining case showed a multicystic cut surface bridged by thick fibrous septa.On the other hand,CN were well-circumscribed and encapsulated.They were multiloculated cystic in nature.The cystic spaces were separated by thin septa and there was no significant solid or necrotic component.Histologically,MEST consisted of proliferation of cystically dilated glands admixed with spindly stromal cells with various cellularity and growth patterns.Both the glandular and stromal elements were well-differentiated with no cytologic atypia identified.The glandular structures in 2 of the cases were partially lined by endometrial or tubal epithelium.In contrast,the thin-walled cystic spaces in CN were lined by a single layer of epithelium.Immunohistochemical　study showed that the epithelial cells were positive for pan-cytokeratin and epithelial membrane antigen.The spindle cells in MEST expressed vimentin (5/5 ) ,smooth muscle actin (3/5 ),desmin (4/5 ),CD10 (5/5),estrogen receptor (4/5) and progesterone receptor (4/5).They were negative for HMB45,CD34,CD117 and S-100 protein.On the other hand,the spindle cells in CN were variably positive for vimentin (4/4),smooth muscle actin (4/4),desmin (1/4),estrogen receptor (3/4) and progesterone receptor (1/4).They were negative for CD10,HMB45,CD34,CD117 and S-100 protein.Conclusions Both MEST and CN are uncommon renal neoplasm.Most of them run a benign clinical course.The stromal cells in MEST show smooth muscle or myofibroblastic differentiation.Areas demonstrating Miillerian features also existed in some cases.MEST and CN share overlapping histological and immunohistochemical features,and may represent spectrum of the same group of lesions.     

肾混合性上皮间质肿瘤和成人囊性肾瘤的临床病理学观察

Objective To study the clinicopathologie features,immunophenotype and differential diagnosis of mixed epithelial and stromal tumor of kidney ( MEST) and adult cystic nephroma ( CN).Methods Five cases of MEST and 4 cases of CN were retrospectively analyzed.Immunohistochemical study was carried out and the literature was reviewed.Results All of the five patients with MEST were females.Their median age was 45 years.For CN,there were 3 males and 1 female and their median age was 41 years.All patients presented with loin pain and hematuria.On gross examination,MEST was well-circumscribed but non-encapsulated.There was no evidence of haemorrhage or necrosis.Three of the cases were solid in nature.One was composed of a mixture of solid and cystic elements,while the remaining case showed a multicystic cut surface bridged by thick fibrous septa.On the other hand,CN were well-circumscribed and encapsulated.They were multiloculated cystic in nature.The cystic spaces were separated by thin septa and there was no significant solid or necrotic component.Histologically,MEST consisted of proliferation of cystically dilated glands admixed with spindly stromal cells with various cellularity and growth patterns.Both the glandular and stromal elements were well-differentiated with no cytologic atypia identified.The glandular structures in 2 of the cases were partially lined by endometrial or tubal epithelium.In contrast,the thin-walled cystic spaces in CN were lined by a single layer of epithelium.Immunohistochemical　study showed that the epithelial cells were positive for pan-cytokeratin and epithelial membrane antigen.The spindle cells in MEST expressed vimentin (5/5 ) ,smooth muscle actin (3/5 ),desmin (4/5 ),CD10 (5/5),estrogen receptor (4/5) and progesterone receptor (4/5).They were negative for HMB45,CD34,CD117 and S-100 protein.On the other hand,the spindle cells in CN were variably positive for vimentin (4/4),smooth muscle actin (4/4),desmin (1/4),estrogen receptor (3/4) and progesterone receptor (1/4).They were negative for CD10,HMB45,CD34,CD117 and S-100 protein.Conclusions Both MEST and CN are uncommon renal neoplasm.Most of them run a benign clinical course.The stromal cells in MEST show smooth muscle or myofibroblastic differentiation.Areas demonstrating Miillerian features also existed in some cases.MEST and CN share overlapping histological and immunohistochemical features,and may represent spectrum of the same group of lesions.     

肾混合性上皮间质肿瘤和成人囊性肾瘤的临床病理学观察

Objective To study the clinicopathologie features,immunophenotype and differential diagnosis of mixed epithelial and stromal tumor of kidney ( MEST) and adult cystic nephroma ( CN).Methods Five cases of MEST and 4 cases of CN were retrospectively analyzed.Immunohistochemical study was carried out and the literature was reviewed.Results All of the five patients with MEST were females.Their median age was 45 years.For CN,there were 3 males and 1 female and their median age was 41 years.All patients presented with loin pain and hematuria.On gross examination,MEST was well-circumscribed but non-encapsulated.There was no evidence of haemorrhage or necrosis.Three of the cases were solid in nature.One was composed of a mixture of solid and cystic elements,while the remaining case showed a multicystic cut surface bridged by thick fibrous septa.On the other hand,CN were well-circumscribed and encapsulated.They were multiloculated cystic in nature.The cystic spaces were separated by thin septa and there was no significant solid or necrotic component.Histologically,MEST consisted of proliferation of cystically dilated glands admixed with spindly stromal cells with various cellularity and growth patterns.Both the glandular and stromal elements were well-differentiated with no cytologic atypia identified.The glandular structures in 2 of the cases were partially lined by endometrial or tubal epithelium.In contrast,the thin-walled cystic spaces in CN were lined by a single layer of epithelium.Immunohistochemical　study showed that the epithelial cells were positive for pan-cytokeratin and epithelial membrane antigen.The spindle cells in MEST expressed vimentin (5/5 ) ,smooth muscle actin (3/5 ),desmin (4/5 ),CD10 (5/5),estrogen receptor (4/5) and progesterone receptor (4/5).They were negative for HMB45,CD34,CD117 and S-100 protein.On the other hand,the spindle cells in CN were variably positive for vimentin (4/4),smooth muscle actin (4/4),desmin (1/4),estrogen receptor (3/4) and progesterone receptor (1/4).They were negative for CD10,HMB45,CD34,CD117 and S-100 protein.Conclusions Both MEST and CN are uncommon renal neoplasm.Most of them run a benign clinical course.The stromal cells in MEST show smooth muscle or myofibroblastic differentiation.Areas demonstrating Miillerian features also existed in some cases.MEST and CN share overlapping histological and immunohistochemical features,and may represent spectrum of the same group of lesions.     

肾混合性上皮间质肿瘤和成人囊性肾瘤的临床病理学观察

Objective To study the clinicopathologie features,immunophenotype and differential diagnosis of mixed epithelial and stromal tumor of kidney ( MEST) and adult cystic nephroma ( CN).Methods Five cases of MEST and 4 cases of CN were retrospectively analyzed.Immunohistochemical study was carried out and the literature was reviewed.Results All of the five patients with MEST were females.Their median age was 45 years.For CN,there were 3 males and 1 female and their median age was 41 years.All patients presented with loin pain and hematuria.On gross examination,MEST was well-circumscribed but non-encapsulated.There was no evidence of haemorrhage or necrosis.Three of the cases were solid in nature.One was composed of a mixture of solid and cystic elements,while the remaining case showed a multicystic cut surface bridged by thick fibrous septa.On the other hand,CN were well-circumscribed and encapsulated.They were multiloculated cystic in nature.The cystic spaces were separated by thin septa and there was no significant solid or necrotic component.Histologically,MEST consisted of proliferation of cystically dilated glands admixed with spindly stromal cells with various cellularity and growth patterns.Both the glandular and stromal elements were well-differentiated with no cytologic atypia identified.The glandular structures in 2 of the cases were partially lined by endometrial or tubal epithelium.In contrast,the thin-walled cystic spaces in CN were lined by a single layer of epithelium.Immunohistochemical　study showed that the epithelial cells were positive for pan-cytokeratin and epithelial membrane antigen.The spindle cells in MEST expressed vimentin (5/5 ) ,smooth muscle actin (3/5 ),desmin (4/5 ),CD10 (5/5),estrogen receptor (4/5) and progesterone receptor (4/5).They were negative for HMB45,CD34,CD117 and S-100 protein.On the other hand,the spindle cells in CN were variably positive for vimentin (4/4),smooth muscle actin (4/4),desmin (1/4),estrogen receptor (3/4) and progesterone receptor (1/4).They were negative for CD10,HMB45,CD34,CD117 and S-100 protein.Conclusions Both MEST and CN are uncommon renal neoplasm.Most of them run a benign clinical course.The stromal cells in MEST show smooth muscle or myofibroblastic differentiation.Areas demonstrating Miillerian features also existed in some cases.MEST and CN share overlapping histological and immunohistochemical features,and may represent spectrum of the same group of lesions.     

伴横纹肌样特征的肾细胞癌的临床病理特征

目的探讨伴横纹肌样特征的肾细胞癌（RCC）的临床病理特征。方法对1995—2005年南京军区南京总医院常规外检档案352例RCC中的10例伴横纹肌样特征的RCC进行了光镜、免疫表型检测和超微结构观察,并结合临床病理特征进行分析。结果10例伴横纹肌样特征的RCC患者年龄33．69岁（平均年龄52岁）,男9例,女1例。5例肿瘤侵犯肾包膜,2例伴淋巴结转移,1例伴肺转移。组织学观察,10例伴横纹肌样特征的RCC中透明细胞型9例,乳头型1例,均可见不同程度的具横纹肌样特征的瘤细胞区域。横纹肌样特征的瘤细胞排列成梁索状、腺泡样、器官样或团片状,瘤细胞散在分布,细胞间无黏附或黏附性差,呈类圆形或多角形,核泡状、偏位,核仁突出,胞质内见红染均质包涵体样物,瘤组织常伴明显坏死。免疫组织化学观察,具横纹肌样特征的瘤细胞CD10、CK（AE1／AE3）、上皮细胞膜抗原（EMA）、波形蛋白均阳性表达,CK7、CK20、结蛋白、肌细胞生成素、α-平滑肌肌动蛋白（α-SMA）、肌肉特异性肌动蛋白（MSA）均阴性表达,神经元特异性烯醇化酶（NSE）、S-100蛋白灶性阳性表达。横纹肌样瘤细胞区Ki-67阳性表达较周围经典型瘤细胞区增高,两者比较差异有统计学意义（P〈0．05）。随访8例,2例于术后6个月及29个月死亡,6例存活。结论伴横纹肌样特征的RCC主要见于透明细胞型,应与胞质嗜酸的肾细胞肿瘤和肾恶性横纹肌样瘤等相鉴别。RCC中伴横纹肌样特征的瘤细胞较周围经典型瘤细胞Ki-67表达明显增高,可能与此类肿瘤临床生物学行为更具侵袭性有关。     

多房囊性肾细胞癌32例临床病理分析

目的：探讨多房囊性肾细胞癌( multilocular cystic renal cell carcinoma, MCRCC)的临床病理特征,提高对该肿瘤的认识。方法复习32例MCRCC的临床资料,观察其病理学形态和免疫表型特征,结合随访资料评价其预后。结果32例中13例位于左肾,18例位于右肾,1例双肾;肿瘤平均最大径4.6 cm(1.0~8.0 cm);男女比为2.2：1。11例行根治性肾全切术,21例行肾部分切除术。肿瘤均为多房囊性,缺少实体成分,囊壁内衬单层(偶为多层或小乳头状)胞质透明或淡粉染、Fuhrman核1级的瘤细胞,腔面富于薄壁血管。瘤细胞表达CK(32/32)、CK7(25/32)、EMA(32/32)、CD10(23/32)、vimentin(20/32),均不表达CD68。术后随访5~140个月,均未见复发和转移,无肿瘤相关死亡病例。结论 MCRCC的瘤细胞核级低、无实性瘤巢;囊壁腔面衬覆胞质透亮或淡粉染细胞伴丰富薄壁血管是诊断线索,免疫表型有助于诊断,患者一般预后良好。     

伴血管母细胞瘤成分肾透明细胞癌临床病理学分析

目的:探讨伴血管母细胞瘤成分肾透明细胞癌［clear cell renal cell carcinoma （ccRCC） with hemangioblastoma（HB） component, ccRCC-HBc］的临床病理及分子学特征。方法:收集2015年9月和2016年3月福建省立医院手术切除标本及会诊病例共2例...     

间变性淋巴瘤激酶易位性肾细胞癌临床病理分析

目的探讨间变性淋巴瘤激酶(ALK)易位性肾细胞癌的临床病理学、分子遗传学、鉴别诊断及预后。方法回顾性分析解放军东部战区总医院2011年1月至2020年12月收集的2例ALK易位性肾细胞癌的组织形态学特征、免疫组织化学表达以及相关预后信息, 采用荧光原位杂交(FISH)技术、逆转录聚合酶链式反应(RT-PCR)、高通量靶向测序等多项分子检测分析其分子病理特征。结果男女各1例, 年龄分别为59岁和57岁。形态学上, 例1类似于肾集合管癌或髓质癌, 呈小管状、微囊网状结构, 具有显著的黏液背景及淋巴细胞浸润;例2则类似于Xp11.2易位性肾细胞癌或Ⅱ型乳头状肾细胞癌, 呈管状乳头状、局部实性结构, 肿瘤细胞胞质呈絮状, 间质内见多量泡沫样组织细胞, 未见黏液背景及淋巴细胞浸润。免疫表型方面, 2例均强阳性表达ALK蛋白, 此外细胞角蛋白7、E-cadherin、波形蛋白、PAX8和CD10呈现不同程度的表达, 其余标志物均为阴性。多种分子检测技术均明确显示ALK基因易位, 例1为罕见的VCL-ALK融合基因, 融合位点为VCL基因的第16号外显子和ALK基因的第20号外显子;例2为EML4-...     

肾细胞癌的临床病理与免疫表型研究

目的 研究肾细胞癌的临床病理特征、预后及免疫表型特点.方法 复习114例肾细胞癌的临床病理资料、HE切片,按2004年WHO肾肿瘤分类标准重新分类、随访并进行免疫组织化学染色.结果 114例.肾细胞癌包括5个类型,肾透明细胞癌77例(67.5%)、乳头状肾癌11例(9.6%)、肾嫌色细胞癌14例(12.3%)、Xp11.2易位_/TFE3基因融合相关性肾癌10例(8.8%)、未能分类肾肿瘤2例(1.8%).免疫组织化学结果,肾透明细胞癌主要表达CK(93.5%,72/77)、CD10(93.5%,72/77)、波形蛋白(75.3%,58/77),乳头状肾癌主要表达α-甲酰基辅酶A消旋酶(AMACR,11/11),肾嫌色细胞癌主要表达CD117(11/14),Xp11.2易位/TFE3基因融合相关性肾癌TFE3、AMACR、CD10和CK的阳性率分别为10/10、10/10、9/10和7/10.结论 肾癌是一组形态学上各有特征的异质性肿瘤,在形态学基础上,CD10、波形蛋白、CD117、AMACR、CK7、TFE3有助于亚型的诊断.     

Immunohistochemical and molecular genetic profiling of acquired cystic disease-associated renal cell carcinoma

Aims:  Acquired cystic disease-associated renal cell carcinoma (ACD-associated RCC) is a unique neoplasm that specifically develops in the background of acquired cystic disease of the kidney. The aim was to analyse nine ACD-associated RCCs from three patients to determine their immunohistochemical and molecular characteristics using immunohistochemistry, comparative genomic hybridization (CGH) and fluorescence in situ hybridization (FISH) .
Methods and results:  ACD-associated RCC preferentially expressed proximal nephron phenotype (CD10+/RCC marker+/α-methylacyl-CoA racemase+/glutathione S-transferase-α+/BerEP4+/cytokeratin 7–/E-cadherin–/high-molecular-weight cytokeratin−/MOC31−). CGH combined with FISH demonstrated non-random chromosomal gains clustering on chromosomes 3 (8/9), 7 (6/9), 16 (7/9), 17 (4/9) and Y (5/9). Chromosomal losses were uncommon. The chromosomal aberrations in all multifocal tumours were not identical for the same kidney or for the same patient, indicating a 'field effect' that induces multiple independent clones.
Conclusions:  Although the genetic profiles of ACD-associated RCC showed some similarity to those of papillary RCC, ACD-associated RCC distinctly revealed frequent gains on chromosomes 3 and Y. ACD-associated RCC is characterized not only by its particular clinical setting and histology, but also by its unique immunohistochemical and molecular genetic profiles.     

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.     

肾脏杂合性嗜酸细胞/嫌色细胞肿瘤的临床病理学特征

目的:探讨肾脏杂合性嗜酸细胞/嫌色细胞肿瘤（HOCT）的临床病理、免疫表型特点以及与肾嗜酸细胞瘤（RO）、嫌色性肾细胞癌间的关联性。方法:收集2008年1月至2019年6月青岛大学附属医院（5例）和海军第九七一医院（3例）病理确诊为HOCT的临床病理资料,进行组织形态学观察、免疫组织化学染色及随访,并与同期诊断的27例...     

Oncocytic renal cell carcinoma with immunohistochemical properties of renal oncocytoma

Renal oncocytoma (RO) is a characteristic benign renal tumor. The existence of malignant RO is controversial and anecdotal, partly due to a lack of specific markers for RO. With recent advances in immunohistochemistry, RO can be distinguished from other renal neoplasms with routine stains and with the aid of immunostaining. We report two cases of renal neoplasms with similar histopathological appearances. They were characterized by oncocytic cytoplasm, numerous intra-cytoplasmic vacuoles, uniform round to oval hyperchromatic nuclei with remarkably thick nuclear membranes and prominent nucleoli. The tumor cells were closely packed and disposed in an alveolar pattern. The neoplastic cells were diffusely reactive for CD117 and progesterone receptor, and diffusely or focally reactive for cytokeratin AE1/AE3, and focally reactive for cytokeratin 7, CD10, and racemase. The cells were non-reactive for renal cell carcinoma (RCC) antigen, vimentin, S100, and neuroendocrine markers. One tumor showed lymph node metastasis. Due to the remarkable cytological atypia, lymph node metastasis, and similar immunological features of RO, these two tumors likely represent a distinct subtype of RCC related to RO.