目的:探讨儿童肾透明细胞肉瘤(clear cell sarcoma of the kidney,CCSK)的临床病理特点与鉴别诊断。方法:观察1例儿童肾透明细胞肉瘤的病理学形态、特殊染色及免疫表型特征并复习有关文献。结果:片内显示为小细胞肿瘤,呈弥漫性分布,部...目的:探讨儿童肾透明细胞肉瘤(clear cell sarcoma of the kidney,CCSK)的临床病理特点与鉴别诊断。方法:观察1例儿童肾透明细胞肉瘤的病理学形态、特殊染色及免疫表型特征并复习有关文献。结果:片内显示为小细胞肿瘤,呈弥漫性分布,部分细胞丰富,密集,部分区域细胞排列较松散,以分枝状纤维血管间质分隔肿瘤细胞呈不规则索状和小团状为结构特征,细胞呈圆形、多角形或梭形,细胞核呈圆形、卵圆形或棒状,核染色质呈细颗粒状,核膜较薄,核仁不明显。网状纤维染色:网状纤维密布于单个或小团肿瘤细胞之间。Masson三色:肿瘤间质小血管周纤维组织呈绿色。免疫组化染色:Vim、Bcl2和CD99(+),其他(-)。结论:CCSK是少见的小儿恶性肾肿瘤,诊断主要依赖于病理学,熟悉CCSK典型形态学特征以及合理应用特殊染色和免疫组化有助于同肾母细胞瘤、PNET和先天性中胚叶肾瘤等鉴别。展开更多
Background:We previously showed that the expression of follistatin-like protein 1(FSTL1)was significantly down-regulated in metastatic clear-cell renal cell carcinoma(ccRCC).In this study,we aimed to characterize the ...Background:We previously showed that the expression of follistatin-like protein 1(FSTL1)was significantly down-regulated in metastatic clear-cell renal cell carcinoma(ccRCC).In this study,we aimed to characterize the role of FSTL1 in the development of ccRCC.Methods:The effects of FSTL1 on cell activity and cell cycle were investigated in ccRCC cell lines with altered FSTL1 expression.Gene expression microarray assays were performed to identify the major signaling pathways affected by FSTL1 knockdown.The expression of FSTL1 in ccRCC and its effect on postoperative prognosis were estimated in a cohort with 89 patients.Results:FSTL1 knockdown promoted anchorage-independent growth,migration,invasion,and cell cycle of ccRCC cell lines,whereas FSTL1 overexpression attenuated cell migration.FSTL1 knockdown up-regulated nuclear factor-κB(NF-κB)and hypoxia-inducible factor(HIF)signaling pathways,increased epithelial-to-mesenchymal transition,up-regulated interleukin-6 expression,and promoted tumor necrosis factor-α-induced degradation of NF-κB inhibitor(IκBα)in ccRCC cell lines.FSTL1 immunostaining was selectively positive in epithelial cytoplasm in the loop of Henle,and positive rate of FSTL1 was significantly lower in ccRCC tissues than in adjacent renal tissues(P<0.001).The mul-tivariate Cox regression analysis showed that the intratumoral FSTL1 expression conferred a favorable independent prognosis with a hazard ratio of 0.325(95%confidence interval 0.118-0.894).HIF-2αexpression was negatively cor-related with FSTL1 expression in ccRCC specimens(r=−0.229,P=0.044).Intratumoral expression of HIF-2α,rather than HIF-1α,significantly predicted an unfavorable prognosis in ccRCC(log-rank,P=0.038).Conclusions:FSTL1 plays a tumor suppression role possibly via repressing the NF-κB and HIF-2αsignaling pathways.To increase FSTL1 expression might be a candidate therapeutic strategy for metastatic ccRCC.展开更多
We present an uncommon case （female patient aged 59 years） of the clear-cell variant of calcifying epithelial odontogenic tumor （CEOT） （also known as Pindborg tumor） in the mandible. The clinical characteristics...We present an uncommon case （female patient aged 59 years） of the clear-cell variant of calcifying epithelial odontogenic tumor （CEOT） （also known as Pindborg tumor） in the mandible. The clinical characteristics and probable origins of the clear tumor cells of previously reported cases of clear-cell variant of intraosseous CEOT are also summarized and discussed.展开更多
A 17-year-old female presented with rectal bleeding from an ulcerated sigmoid mass in 1994.Initial pathological evaluation revealed a rare clear cell neoplasm of the colon,possibly originating from kidneys,adrenals,lu...A 17-year-old female presented with rectal bleeding from an ulcerated sigmoid mass in 1994.Initial pathological evaluation revealed a rare clear cell neoplasm of the colon,possibly originating from kidneys,adrenals,lung or a gynecologic source as a metastatic lesion.Extensive imaging studies were negative,and over the next 15 years,she remained well with no recurrence.The original resected neoplasm was reviewed and reclassified as a perivascular epithelioid cell neoplasm (PEComa).Although the long-term natural history of PEComas requires definition,increased clinical and pathological awareness should lead to increased recognition of an apparently rare type of colonic neoplasm that likely occurs more often than is currently appreciated.展开更多
Introduction:?Ovarian borderline clear cell tumor is a very rare tumor accounting for less than 1% of borderline ovarian tumors. Currently, only 30 cases of borderline clear cell tumors have been reported.?This border...Introduction:?Ovarian borderline clear cell tumor is a very rare tumor accounting for less than 1% of borderline ovarian tumors. Currently, only 30 cases of borderline clear cell tumors have been reported.?This borderline ovarian clear cell tumor often arises from endometriosis. But there are no definitive guidelines for the management of endometriosis with malignant transformation due to a lack of understanding of the mechanistic details that mediate the transformation of endometriosis into ovarian cancer. Case: A?52-year-old woman (G2 P2) who periodically underwent ultraso-nography over the course of 12 years in order to follow the endometrioma present in her left ovary. The diameter of the lesion was 46 mm and it remained largely unchanged. However, a solid region within the ovarian cyst was detected. After the surgery, the final pathological diagnosis was cystic clear cell tumor of borderline malignancy.?Conclusion: It reported that risk factors of malignant transformation are older age, postmenopausal status, and larger tumor diameters were associated with a higher prevalence of cancer development. But it is recommended that patients with endometriosis, regardless of patient age or tumor size, undergo careful monitoring to detect changes in growth or the formation of solid regions for as long as the lesion is present.展开更多
文摘目的:探讨儿童肾透明细胞肉瘤(clear cell sarcoma of the kidney,CCSK)的临床病理特点与鉴别诊断。方法:观察1例儿童肾透明细胞肉瘤的病理学形态、特殊染色及免疫表型特征并复习有关文献。结果:片内显示为小细胞肿瘤,呈弥漫性分布,部分细胞丰富,密集,部分区域细胞排列较松散,以分枝状纤维血管间质分隔肿瘤细胞呈不规则索状和小团状为结构特征,细胞呈圆形、多角形或梭形,细胞核呈圆形、卵圆形或棒状,核染色质呈细颗粒状,核膜较薄,核仁不明显。网状纤维染色:网状纤维密布于单个或小团肿瘤细胞之间。Masson三色:肿瘤间质小血管周纤维组织呈绿色。免疫组化染色:Vim、Bcl2和CD99(+),其他(-)。结论:CCSK是少见的小儿恶性肾肿瘤,诊断主要依赖于病理学,熟悉CCSK典型形态学特征以及合理应用特殊染色和免疫组化有助于同肾母细胞瘤、PNET和先天性中胚叶肾瘤等鉴别。
基金supported by the National Key Basic Research Program(973 program)(2015CB554000 to GC)the National Natural Science Foundation of China(81520108021 and 91529305 to G.C.,81672518 and 81101928 to X.T.)the Three-year Action Plan on Public Health,Phase IV,Shanghai,China(15GWZK0801 to GC).
文摘Background:We previously showed that the expression of follistatin-like protein 1(FSTL1)was significantly down-regulated in metastatic clear-cell renal cell carcinoma(ccRCC).In this study,we aimed to characterize the role of FSTL1 in the development of ccRCC.Methods:The effects of FSTL1 on cell activity and cell cycle were investigated in ccRCC cell lines with altered FSTL1 expression.Gene expression microarray assays were performed to identify the major signaling pathways affected by FSTL1 knockdown.The expression of FSTL1 in ccRCC and its effect on postoperative prognosis were estimated in a cohort with 89 patients.Results:FSTL1 knockdown promoted anchorage-independent growth,migration,invasion,and cell cycle of ccRCC cell lines,whereas FSTL1 overexpression attenuated cell migration.FSTL1 knockdown up-regulated nuclear factor-κB(NF-κB)and hypoxia-inducible factor(HIF)signaling pathways,increased epithelial-to-mesenchymal transition,up-regulated interleukin-6 expression,and promoted tumor necrosis factor-α-induced degradation of NF-κB inhibitor(IκBα)in ccRCC cell lines.FSTL1 immunostaining was selectively positive in epithelial cytoplasm in the loop of Henle,and positive rate of FSTL1 was significantly lower in ccRCC tissues than in adjacent renal tissues(P<0.001).The mul-tivariate Cox regression analysis showed that the intratumoral FSTL1 expression conferred a favorable independent prognosis with a hazard ratio of 0.325(95%confidence interval 0.118-0.894).HIF-2αexpression was negatively cor-related with FSTL1 expression in ccRCC specimens(r=−0.229,P=0.044).Intratumoral expression of HIF-2α,rather than HIF-1α,significantly predicted an unfavorable prognosis in ccRCC(log-rank,P=0.038).Conclusions:FSTL1 plays a tumor suppression role possibly via repressing the NF-κB and HIF-2αsignaling pathways.To increase FSTL1 expression might be a candidate therapeutic strategy for metastatic ccRCC.
文摘We present an uncommon case （female patient aged 59 years） of the clear-cell variant of calcifying epithelial odontogenic tumor （CEOT） （also known as Pindborg tumor） in the mandible. The clinical characteristics and probable origins of the clear tumor cells of previously reported cases of clear-cell variant of intraosseous CEOT are also summarized and discussed.
文摘A 17-year-old female presented with rectal bleeding from an ulcerated sigmoid mass in 1994.Initial pathological evaluation revealed a rare clear cell neoplasm of the colon,possibly originating from kidneys,adrenals,lung or a gynecologic source as a metastatic lesion.Extensive imaging studies were negative,and over the next 15 years,she remained well with no recurrence.The original resected neoplasm was reviewed and reclassified as a perivascular epithelioid cell neoplasm (PEComa).Although the long-term natural history of PEComas requires definition,increased clinical and pathological awareness should lead to increased recognition of an apparently rare type of colonic neoplasm that likely occurs more often than is currently appreciated.
文摘Introduction:?Ovarian borderline clear cell tumor is a very rare tumor accounting for less than 1% of borderline ovarian tumors. Currently, only 30 cases of borderline clear cell tumors have been reported.?This borderline ovarian clear cell tumor often arises from endometriosis. But there are no definitive guidelines for the management of endometriosis with malignant transformation due to a lack of understanding of the mechanistic details that mediate the transformation of endometriosis into ovarian cancer. Case: A?52-year-old woman (G2 P2) who periodically underwent ultraso-nography over the course of 12 years in order to follow the endometrioma present in her left ovary. The diameter of the lesion was 46 mm and it remained largely unchanged. However, a solid region within the ovarian cyst was detected. After the surgery, the final pathological diagnosis was cystic clear cell tumor of borderline malignancy.?Conclusion: It reported that risk factors of malignant transformation are older age, postmenopausal status, and larger tumor diameters were associated with a higher prevalence of cancer development. But it is recommended that patients with endometriosis, regardless of patient age or tumor size, undergo careful monitoring to detect changes in growth or the formation of solid regions for as long as the lesion is present.
