Deshmukh Mahesh, Shet Tanuja, Bakshi Ganesh, Desai Sangeeta
Department of Pathology, Tata Memorial Hospital, Parel, Mumbai 400 012, India.
Indian J Pathol Microbiol. 2011 Jan-Mar;54(1):127-30. doi: 10.4103/0377-4929.77363.
Tubulocystic renal cell carcinoma (TCRCC) is a rare variant of renal cell carcinoma, which has distinct histology but there is some controversy about its association with papillary renal cell carcinoma (PRCC) and cell of origin in literature. We report an 18-year-old girl with the rare TCRCC of kidney associated with PRCC with metastases to the para-aortic nodes. The patient presented with hematuria and a right renal mass with enlarged regional nodes for which a radical nephrectomy with retroperitoneal lymph node dissection was done. On gross examination, a solid cystic lesion involving the lower pole and middle pole of the kidney measuring 12x9x9 cm was seen along with an additional cystic lesion in upper pole of kidney. Microscopically the main tumor showed the typical histology of a tubulocystic carcinoma with multiple cysts filled with secretions lined by variably flattened epithelium with hobnailing of cells. The mass in the upper pole was a high-grade PRCC and the nodal metastases had morphology similar to this component. To conclude, at least a small but definite subset of TCRCC is associated with PRCC, and cases associated with PRCC do seem to have a higher propensity for nodal metastasis as in the case we report.
肾小管囊性肾细胞癌（TCRCC）是肾细胞癌的一种罕见变体，其组织学特征独特，但在文献中关于它与乳头状肾细胞癌（PRCC）的关联以及起源细胞存在一些争议。我们报告了一名18岁女孩，患有罕见的肾脏TCRCC并伴有PRCC，且已转移至主动脉旁淋巴结。患者因血尿和右肾肿块伴局部淋巴结肿大就诊，接受了根治性肾切除术及腹膜后淋巴结清扫术。大体检查可见一个实性囊性病变，累及肾脏下极和中极，大小为12×9×9 cm，同时肾脏上极还有一个额外的囊性病变。显微镜下，主要肿瘤呈现出典型的肾小管囊性癌组织学特征，有多个充满分泌物的囊肿，内衬不同程度扁平的上皮细胞，细胞有鞋钉样改变。上极的肿块为高级别PRCC，淋巴结转移灶的形态与该成分相似。总之，至少有一小部分但明确的TCRCC亚群与PRCC相关，并且与PRCC相关的病例似乎确实像我们报告的病例一样，有更高的淋巴结转移倾向。
