伴有乳头状特征的卵巢透明细胞癌

卵巢透明细胞癌（CCC）虽然占卵巢所有恶性肿瘤不到5％，但在妇科病理鉴别诊断中却占有相当比例。与卵巢CCC相关的鉴别诊断包括原始生殖细胞肿瘤（特别是卵黄囊瘤和无性细胞瘤）、性索间质肿瘤（类固醇细胞瘤）和转移性肿瘤（Krukenbegr瘤和肾细胞癌）。卵巢CCC误诊为卵巢低度恶性潜能浆液性肿瘤（L-LMP）尚未充分认识，作者分析了13例伴有明显乳头状特征的卵巢CCC，年龄39～65岁，平均53．8岁，所有病人临床表现为盆腔包块。     

透明细胞乳头状肾细胞癌1例

<正>患者女性,60岁,因右侧输尿管结石入院,自诉偶有右腰部酸胀感,无发热,无尿频、尿急、尿痛,无肉眼血尿;查体:心肺听诊无殊,腹软,未及包块,未及压痛、反跳痛,右肾区轻叩痛,左肾区无叩痛;腹部CT示右肾实质内见直径1. 6 cm等低密度结节影突出肾轮廓,增强后动脉期强化类似肾皮质,后期密度减低,呈相对略低密度,报告提示右肾占位,肾癌可能。行右肾部分切除术,送病理检查。     

透明细胞乳头状肾细胞癌1例

正患者女性,65岁。近年偶有腰酸、腰痛伴尿频、尿急、夜尿4～5次,于2017年6月收治我院。入院后B超示左肾中上部混合回声包块,CT示左肾占位,术前考虑肾癌可能。血尿常规、肝肾功能等检查均未见异常;行腹腔镜下手术切除。病理检查眼观:肿瘤位于肾中上极,大小3.5 cm×3cm×3 cm,切面多房囊实性,局部钙化,未见坏死出血(图1)。镜检:肿瘤以囊性及乳头状生长方式为主,囊性结构约     

卵巢透明细胞癌6例

卵巢透明细胞癌是上皮性肿瘤中较少见的一种,国内报道尚不很多。我院近年遇到6例,按WHO卵巢肿瘤组织学分类标准,均经病理确诊,现作一简要报告。临床资料发病率国外报告本瘤在卵巢肿瘤中的发病率为     

卵巢交界性透明细胞腺纤维瘤局灶合并透明细胞腺癌一例

患者女,54岁.因腰部酸胀1年,查体发现盆腔包块1个月于2007年10月入院.患者1991年因"子宫肌瘤+子宫内膜异位症"行子宫全切术.近1年感腰部酸胀,未就诊,近2个月加重,1个月前当地医院B超提示盆腔包块后来本院检查.     

肝内胆管巨大乳头状囊腺瘤癌变一例

患者女，４２岁。因上腹部撞伤后疼痛伴呕吐１天，于１９９５年３月２８日入院。平时常感腹胀，偶有上腹疼痛。体检：腹部膨隆，上腹部广泛压痛、反跳痛、下腹部轻压痛，肝脾未触及。腹腔穿刺：抽出不凝固性血性混浊液体。行急诊手术。术中所见：腹腔内血性混浊液体４５０...     

妊娠合并巨大卵巢粘液性囊腺瘤一例

病例:患者,24岁,孕1产0,既往月经规律,末次月经2001年11月03日,于6+个月前在外院诊断为早孕,于入院前1d在外院行超声检查发现巨大卵巢囊肿,于2002年5月7日转入我院。患者孕期无腹痛,腹胀及其它不适。查体:腹部明显膨隆,于脐上2指可扪及宫底,无宫缩,胎心144次/min,规律。妇科检查:外阴、阴道无异常,宫颈光滑,宫体如     

单细胞水平检测von Hippel-Lindau病基因突变的实验研究

目的建立单细胞水平检测vonHippel-Lindau病基因(VHL)突变的实验方法。方法单个淋巴细胞基于多重置换扩增(multiple displacement amplification,MDA)的全基因组扩增后进行常规PCR后测序和实时荧光定量PCR,结合各荧光的终点变化判断对应的等位基因存在与否。结果MDA后单细胞扩增效率为90.91%,污染率为0;通过测序,患者VHL等位基因的脱扣率为26.67%,诊断正确率为73.33%;通过结合相应荧光的终点变化判断患者VHL基因的等位基因的脱扣率为16.67%,正确率为83.33%。结论单细胞MDA后常规PCR后测序及实时荧光定量PCR能够特异、准确地检测单个淋巴细胞的VHL基因型,两者的联合应用有助于提高检测的准确性。     

甲状腺透明细胞型微小乳头状癌1例

正患者女性,41岁。于1年前体检发现甲状腺结节,近期自觉右侧颈部肿大明显遂住院治疗。患者自述无畏寒、发热;无手抖、出汗、突眼不适。精神及体力无明显减退。专科查体:右侧甲状腺稍肿大,甲状腺下极可扪及一约直径1 cm包块,质韧,随吞咽上下活动,无触痛,颈部淋巴结未触及肿大。实验室检查:促甲状腺激素(TSH)14.27μIU/ml,抗甲状腺球蛋白抗体(Anti-Tg)52.28 IU/ml,抗甲状腺微粒体抗     

卵巢交界性透明细胞肿瘤1例

<正>患者女性,43岁,入院10 h前无明显诱因下腹部胀痛,持续性加重,伴潮热、头晕、头痛,妇科检查双侧附件轻压痛,以左侧附件区明显,无反跳痛。B超示:子宫前位,大小、形态正常,内膜厚约9 mm。左侧附件见一囊性包块,大小41mm×38 mm×35 mm,边界清,壁薄,内透声好,内见多个稍强回声光带。右侧附件区未见明显异常包块。病理检查眼观:送检腹腔镜手术切除囊壁样组织2块,其中一块组织颜色灰白,大小6. 5 cm×5. 3 cm,囊壁厚     

An analysis of five clear cell papillary cystadenomas of mesosalpinx and broad ligament: four associated with von Hippel-Lindau disease and one aggressive sporadic type

Nogales F F, Goyenaga P, Preda O, Nicolae A, Vieites B, Ruiz‐Marcellan M C, Pedrosa A & Merino M J
(2012) Histopathology  60, 748–757
An analysis of five clear cell papillary cystadenomas of mesosalpinx and broad ligament: four associated with von Hippel‐Lindau disease and one aggressive sporadic type Aims: Clear cell papillary cystadenoma (CCPC) is associated with von Hippel‐Lindau disease (VHLD), but rarely involves mesosalpinx and broad ligament (M/BL). This study provides new data about its behaviour and immunophenotype. Methods and results: We performed an analysis of four benign cases of CCPC of M/BL with either characteristic clinical features or genetic markers [loss of heterozygosity (LOH)] of VHLD in patients ranging from 24 to 36 years and a sporadic case in a 52‐year‐old presenting with peritoneal metastases. All CCPCs were papillary but had solid and tubular areas. Haemorrhage, thrombosis and scarring were constant features and related to an unusual pattern of sub‐epithelial vascularity. All clear or oxyphilic cells co‐expressed cytokeratin 7 (CK7), CAM5.2 and vimentin, with strong apical CD10 and nuclear paired box gene 2 (PAX2) immunoreactivity. Three cases also showed positivity for VHL40, epithelial membrane antigen (EMA), Wilms’ tumour suppressor gene (WT‐1) and cancer antigen 125 (CA125) but only one expressed renal cell carcinoma (RCC) antigen. Vascular plexus overexpressed nuclear and cytoplasmic WT‐1. Conclusion: The VHLD‐associated cases appeared to be benign, but the sporadic case exhibited a low malignant potential. CCPCs show histological and immunophenotypical similarities with the recently reported clear cell papillary RCC, although the previously unreported apical CD10 and nuclear PAX2 expression may be related to their mesonephric origin. CCPC has a distinctive sub‐epithelial vascular pattern that is consistent with its pathogenesis.     

Clear cell carcinoid tumor of the gallbladder. A case without von Hippel-Lindau disease

A golden yellow polyp was detected in the gallbladder of a 64-year-old man who presented with epigastric pain. The lesion was composed of clear polygonal cells arranged in a trabecular and glandular pattern. The tumor invaded through the wall into the perimuscular subserosal layer. Immunohistochemical stains showed that neoplastic cells were positive for chromogranin A, synaptophysin, somatostatin, gastrin, and pancreatic polypeptide and negative for glucagon, serotonin, insulin, S100 protein, and inhibin. This tumor resembles the recently described clear cell endocrine tumors of the gallbladder and pancreas that are associated with von Hippel-Lindau disease. Our patient, however, had neither personal nor family history indicative of von Hippel-Lindau disease. Furthermore, published accounts of clear cell endocrine tumors in von Hippel-Lindau disease describe immunoreactivity for inhibin; the current case was negative for the disease. There may be a subtype of clear cell carcinoid tumor not associated with von Hippel-Lindau disease, which is characterized by its lack of immunoreactivity against inhibin.     

Cytogenetics of renal cell carcinomas associated with von Hippel-Lindau disease

To establish the chromosome pattern, we have analyzed short-term cultures of 24 renal cell carcinomas (RCC) from four patients with von Hippel-Lindau disease (VHL). We evaluated the results together with those for 16 RCCs from two VHL patients karyotyped previously in our laboratory and those of 6 tumors published by others. In all 46 RCCs, the cells had lost the shortest overlapping region of the 3pl3-pter chromosome segment. The rearrangement of 3p was the only karyotype change in 20 tumors. In more than 50% of the tumors, a gain of the shortest overlapping region of the 5122-qter segment was detected. Comparative analysis showed that the chromosome aberrations in RCCs associated with VHL are similar to those found in sporadic RCCs. These results indicate that non-papillary sporadic and VHL-RCCs have common genetic mechanisms that result in the loss of the 3p13-pter region containing one or more putative suppressor genes.     

Von Hippel-Lindau disease: a case report

Von Hippel Lindau (VHL) disease is a rare autosomal dominant condition manifested by central nervous system hemangioblastoma, retinal angiomas, cysts of pancreas, kidney and epididymis, pheochromocytomas and renal cell carcinoma. We present such a case in a 45 years old male patient.     

T-cell lymphoma with von Hippel-Lindau disease: a rare case report and review of literature

von Hippel-Lindau disease (VHLD) comprises a series of complicated clinical manifestations. We hereby described a unique case of co-existing T-cell lymphoma (TCL) and confirmed VHLD. The symptoms in this 42-year-old male included fever and pancytopenia. Overall tests and examination made an infectious process unlikely. The results of bone marrow biopsy confirmed the diagnosis. The purposes we described this case were to probe into the relationship between TCL and VHLD, which was not mentioned in previously literature. Combination of clinical, radiological, immunophenotypic, pathological, and genetic data plays an important role in improving the rate of diagnosis, particularly in the challenge for diagnosis of T cell non-Hodgkin lymphoma.     

Clear cell carcinoma of the breast with solid papillary pattern: a case report with immunohistochemical profile

Glycogen rich clear cell carcinoma of the breast is a rare neoplasm with different morphological characteristics to ordinary breast carcinomas. However, it has some common features with clear cell carcinomas of other organs. This report describes a case of clear cell carcinoma of the breast with a solid papillary pattern centrally localised in the left breast of a 45 year old woman. Antibodies directed against cytokeratin 7 (CK7), CK10, CK14, CK17, CK18, CK19, CK20, CK5/6/18, CK8/18, high molecular weight cytokeratin AE3, high molecular weight cytokeratin 34betaE12, the oestrogen receptor, the progesterone receptor, chromogranin, S-100 protein, smooth muscle actin, vimentin, and carcinoembryogenic antigen were applied to analyse the immunophenotypical profile of this rare neoplasm.     

Suprasellar hemangioblastoma without von Hippel-Lindau disease: a case report and literature review

Suprasellar hemangioblastoma (HBL) without von Hippel-Lindau (VHL) disease is extremely rare. A 51-year-old woman presented with headache and progressively deteriorating bilateral visual disturbance for 4 months. Magnetic resonance imaging (MRI) revealed a 2.5-cm solid mass in the suprasellar region with homogeneous contrast enhancement. Our preoperative presumptive diagnosis was meningioma. Resection of the tumor was achieved via a left pterional craniotomy. The tumor was reddish in appearance and relatively firm, and was extremely vascularized, which might provide extensive blood supply through small branches of the internal carotid artery. There was a clear border between the tumor and the pituitary stalk and optic nerves. Histopathologic examination showed that the tumor was well vascularized, consisting of a reticular mesh of numerous thin-walled capillaries and abundant stromal cells. Immunohistochemistry demonstrated the positive staining for CD34, vimentin (VIM), and neuron specific enolase (NSE) in the intratumoral capillaries, while negative staining of epithelial membrane antigen (EMA) and glial fibrillary acidic protein (GFAP) was observed. Based on these results, the patient was diagnosed as HBL. After the resection, the visual field defect in the left eye was markedly improved, and no tumor recurrence was noted in 1 year follow-up. When solid lesions are highly vascularized in the suprasellar region of patients, even though no VHL disease is present, the possibility of HBL should be taken into consideration. Moreover, craniotomy is a better treatment option for suprasellar HBL without VHL disease.     

Ovarian serous cystadenoma associated with Sertoli-Leydig cell tumor--a case report.

We Describe a case of ovarian serous cystadenoma having Sertoli-Leydig cell tumor, well differentiated, in the cystic septum. Well differentiated Sertoli-Leydig cell tumor coexisting with other tumor, including serous tumor, has not yet been described. In all cases of Sertoli-Leydig cell tumor with heterologous components or other tumors, the androblastomatous components are intermediately or poorly differentiated. The present case revealed a well differentiated Sertoli-Leydig cell tumor arising in a septum of serous cystadenoma, as a circumscribed nodule. With these findings, we discuss the possibility of this Sertoli-Leydig cell tumor, considered a mural nodule, which is well established in cystic common epithelial tumors of the ovary.