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肾脏重度黑色素沉着性血管周上皮样透明细胞肿瘤

Heavily melanotic perivascular epithelioid clear cell tumor of the kidney.

作者信息

Adachi Shiro, Hanada Masato, Kobayashi Yoshiyuki, Tsutahara Kohichi, Fukuhara Shin-ichiro, Mori Naoki, Hara Tsuneo, Mukai Hideyo, Shimasaku Emi, Kawai Masaru, Kishikawa Naoto, Yamaguchi Seiji

机构信息

Department of Pathology and Laboratory Medicine, The City Hospital of Ikeda, Osaka, Japan.

出版信息

Pathol Int. 2004 Apr;54(4):261-5. doi: 10.1111/j.1440-1827.2004.01617.x.

DOI:10.1111/j.1440-1827.2004.01617.x
PMID:15028028
Abstract

A black-colored and well-circumscribed renal tumor in a 71-year-old woman is reported. The tumor was unique in that it was rich in vasculature and exclusively composed of perivascular epithelioid clear cells. Morphological features were reminiscent of conventional renal cell carcinoma (RCC). However, immunohistochemical examinations showed that the tumor cells did not express any epithelial markers, but diffusely and intensely expressed a melanocytic marker, gp-100/HMB-45. Another striking feature of the tumor was a large amount of cytoplasmic pigment that made the tumor wholly black. The pigment was not stained with Berlin-blue, completely bleached with potassium permanganate, and stained with Fontana-Masson staining, which suggests that the pigment was melanin. Morphological features and immunohistochemical findings indicated that the present tumor was an extreme example of a perivascular epithelioid clear cell tumor with a large amount of melanin, which has not been previously reported. One should be aware of the pure form of perivascular epithelioid clear cell tumor of the kidney because it is sometimes very difficult to differentiate this tumor from conventional RCC. Immunohistochemical examinations and the presence of cytoplasmic melanin can help the differentiation.

摘要

报告了一名71岁女性患者的一例黑色且边界清晰的肾肿瘤。该肿瘤的独特之处在于其血管丰富,且完全由血管周上皮样透明细胞组成。形态学特征使人联想到传统肾细胞癌(RCC)。然而,免疫组化检查显示肿瘤细胞不表达任何上皮标志物,而是弥漫性且强烈表达一种黑素细胞标志物gp-100/HMB-45。该肿瘤的另一个显著特征是大量的细胞质色素,使肿瘤整体呈黑色。该色素不被柏林蓝染色,用高锰酸钾完全漂白后,经Fontana-Masson染色呈阳性,这表明该色素为黑色素。形态学特征和免疫组化结果表明,本病例肿瘤是血管周上皮样透明细胞肿瘤伴有大量黑色素的一个极端例子,此前未见报道。应注意肾血管周上皮样透明细胞肿瘤的纯形式,因为有时很难将该肿瘤与传统RCC区分开来。免疫组化检查和细胞质黑色素的存在有助于鉴别诊断。

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引用本文的文献

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Pigmented perivascular epithelioid cell tumor (PEComa) of the kidney: a case report and review of the literature.肾色素性血管周上皮样细胞瘤(PEComa):1例病例报告并文献复习
Korean J Pathol. 2012 Oct;46(5):499-502. doi: 10.4132/KoreanJPathol.2012.46.5.499. Epub 2012 Oct 25.
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Perivascular epithelioid cell tumor of the rib.
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Virchows Arch. 2008 Jun;452(6):697-702. doi: 10.1007/s00428-008-0612-y. Epub 2008 Apr 24.
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Malignant clear-cell myomelanocytic tumor of broad ligament--a case report.阔韧带恶性透明细胞肌黑素细胞瘤——病例报告
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Perivascular epithelioid cell tumor (PEComa) of the uterine cervix associated with intraabdominal "PEComatosis": A clinicopathological study with comparative genomic hybridization analysis.子宫颈血管周上皮样细胞瘤(PEComa)伴腹腔内“PEComatosis”:一项采用比较基因组杂交分析的临床病理研究
World J Surg Oncol. 2004 Oct 19;2:35. doi: 10.1186/1477-7819-2-35.