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转移性肉瘤样肾嫌色细胞癌引起的淋巴管周围性腹膜后癌病

Lymphangitic retroperitoneal carcinomatosis occurring from metastatic sarcomatoid chromophobe renal cell carcinoma.

作者信息

Alimchandani Meghna, Lara Karlena, Tsokos Maria, Linehan W M, Merino Maria J

机构信息

Translational Surgical Pathology, Lab. Of Pathology, National Cancer Institute, National Institutes of Health, Bethesda, Maryland.

Urologic Oncology Branch, National Cancer Institute, National Institutes of Health, Bethesda, Maryland.

出版信息

Urol Case Rep. 2014 Mar 1;2(2):39-62. doi: 10.1016/j.eucr.2013.12.008.

Abstract

Forty -five year-old man with left renal mass underwent nephrectomy to reveal 20cm tumor diagnosed as sarcomatoid chromophobe renal cell carcinoma (CRCC). Lymph node metastasis of chromophobe and sarcomatoid components, disseminated tumor in retroperitoneal fat, lymphatic vessels, peri-renal adipose tissue in lymphangitic carcinomatosis pattern were identified. Chromophobe epithelial cells EMA, c-Kit, cytokeratin 7 positive; sarcomatoid cells were CD10, SMA positive with high proliferation index . Chromophobe epithelial cells had loss of heterozygosity (LOH) in chromosomes 1p, 1q while sarcomatoid cells had LOH in 3p,1p, 1q. In conclusion, sarcomatoid CRCC has aggressive biologic behavior and potential to metastasize in unusual patterns.

摘要

一名45岁的左侧肾脏肿物男性患者接受了肾切除术,术中发现一个20厘米的肿瘤,诊断为肉瘤样嫌色肾细胞癌(CRCC)。发现存在嫌色和肉瘤样成分的淋巴结转移,腹膜后脂肪、淋巴管、肾周脂肪组织中呈淋巴管癌病模式的播散性肿瘤。嫌色上皮细胞EMA、c-Kit、细胞角蛋白7阳性;肉瘤样细胞CD10、SMA阳性,增殖指数高。嫌色上皮细胞在1号染色体短臂、1号染色体长臂存在杂合性缺失(LOH),而肉瘤样细胞在3号染色体短臂、1号染色体短臂、1号染色体长臂存在LOH。总之,肉瘤样CRCC具有侵袭性生物学行为和以不寻常模式转移的潜能。

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