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.
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.
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DOI：
10.1111/j.1365-2559.2011.04151.x
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发表时间：
2012-04
期刊：
影响因子：
6.4
通讯作者：
Merino MJ
中科院分区：
文献类型：
--
作者：
Nogales FF;Goyenaga P;Preda O;Nicolae A;Vieites B;Ruiz-Marcellan MC;Pedrosa A;Merino MJ
透明细胞乳头状囊腺瘤(CCPC)与von Hippel-Lindau病(VHLD)有关，但很少累及输卵管系膜和阔韧带。这项研究提供了有关其行为和免疫表型的新数据。我们分析了四例良性的M/BLCCPC患者，其临床特征或VHLD的遗传标记[杂合性缺失(LOH)]的患者年龄从24岁到36岁不等，还有一例52岁的散发性腹膜转移患者。所有CCPC均为乳头状，但有实心区和管状区。出血、血栓形成和疤痕形成是常见的特征，与一种不寻常的上皮下血管模式有关。所有透明或嗜氧细胞共表达细胞角蛋白7(CK7)、CAM5.2和波形蛋白，顶端CD10和核对盒基因2(PAX2)免疫反应较强。3例VHL40、上皮膜抗原(EMA)、Wilms抑癌基因(WT-1)、癌抗原125(CA125)阳性，仅1例表达肾细胞癌(RCC)抗原。血管丛高表达胞核和胞浆WT-1。与VHLD相关的病例似乎是良性的，但零星病例显示出较低的恶性潜能。CCPC在组织学和免疫表型上与最近报道的透明细胞乳头状肾细胞癌相似，尽管先前未报道的顶端CD10和核PAX2的表达可能与它们的中肾起源有关。CCPC具有独特的亚上皮下血管模式，这与其发病机制一致。
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. 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 subepithelial vascularity. All clear or oxyphilic cells coexpressed 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. 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.
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影响因子：
7.2
作者：
Wagner M;Browne HN;Marston Linehan W;Merino M;Babar N;Stratton P
通讯作者：
Stratton P
影响因子：
5
作者：
FUNK, KC;HEIKEN, JP
通讯作者：
HEIKEN, JP
影响因子：
5.6
作者：
Aydin, H;Young, RH;Epstein, JI
通讯作者：
Epstein, JI
影响因子：
4.6
作者：
Truong, Luan D.;Shen, Steven S.
通讯作者：
Shen, Steven S.
影响因子：
5.6
作者：
Rabban, Joseph T.;McAlhany, Stephanie;Zaloudek, Charles J.
通讯作者：
Zaloudek, Charles J.