Dundr Pavel, Pesl Michael, Povýsil Ctibor, Tvrdík Daniel, Pavlík Ivan, Soukup Viktor, Dvorácek Jan
Institute of Pathology, 1st Medical Faculty and General Faculty Hospital, Charles University, Studnickova 2, Prague 128 00, Czech Republic.
Pathol Res Pract. 2007;203(8):593-7. doi: 10.1016/j.prp.2007.05.005. Epub 2007 Jul 20.
We report a case of a 60-year-old female with a pigmented microcystic chromophobe renal cell carcinoma (PMCRCC). The tumor was 4.5 cm in diameter, and was located in the right kidney. Grossly, on cross section, the tumor was light gray with multiple small brown to black pigmented foci up to 0.2 cm in diameter. Histologically, the tumor showed a microcystic arrangement with cribriform areas and formation of adenomatous structures. The microcystic and cribriform areas were composed of larger pale cells and smaller eosinophilic cells, with cytological features of conventional chromophobe renal cell carcinoma (CRCC). The cytological features of the cells within the adenomatous structures were different. These cells were mostly columnar with nuclei at the base, and had a variable amount of pale to eosinophilic cytoplasm. There were foci of ample brown pigmentation located in the cytoplasm of the tumor cells and extracellularly. In addition, microscopic calcifications were present. Immunohistochemically, the tumor cells were positive for EMA, E-cadherin, cytokeratin CAM5.2, and cytokeratin AE1/AE3. Cytokeratin 7 was positive only focally. S-100 protein, melan A, HMB 45, vimentin, and CD117 were negative. PMCRCC is a rare tumor. To the best of our knowledge, only one series containing 20 cases of this variant of CRCC has been described to date. The important feature is that PMCRCC seems to have a relatively benign biological behavior, and distant metastases and sarcomatoid transformation are absent.
我们报告一例60岁女性色素性微囊性嫌色肾细胞癌（PMCRCC）。肿瘤直径4.5 cm，位于右肾。大体上，肿瘤横切面呈浅灰色，有多个直径达0.2 cm的小的棕色至黑色色素沉着灶。组织学上，肿瘤呈微囊性排列，有筛状区域并形成腺瘤样结构。微囊性和筛状区域由较大的淡染细胞和较小的嗜酸性细胞组成，具有传统嫌色肾细胞癌（CRCC）的细胞学特征。腺瘤样结构内细胞的细胞学特征不同。这些细胞大多呈柱状，核位于基部，有数量不等的淡染至嗜酸性细胞质。肿瘤细胞胞质内及细胞外有大量棕色色素沉着灶。此外，可见微小钙化。免疫组化显示，肿瘤细胞EMA、E-钙黏蛋白、细胞角蛋白CAM5.2和细胞角蛋白AE1/AE3呈阳性。细胞角蛋白7仅局灶阳性。S-100蛋白、Melan A、HMB 45、波形蛋白和CD117均为阴性。PMCRCC是一种罕见肿瘤。据我们所知，迄今为止仅描述过一个包含20例该CRCC变异型的系列病例。重要的是，PMCRCC似乎具有相对良性的生物学行为，无远处转移和肉瘤样转化。
