Yu Yong-wei, Hou Jian-guo, Chen Hai-tang, Cao Guang-wen, Wang Lin-hui, Xu Chuan-liang, Sun Ying-hao
Department of Pathology, Changhai Hospital, the Second Military Medical University, Shanghai 200433, China.
Zhonghua Bing Li Xue Za Zhi. 2008 Nov;37(11):721-5.
To assess the clinicopathological features and molecular genetic changes of multilocular cystic renal cell carcinoma (MCRCC).
All the data reviewed were from the files of pathology department of Changhai hospital collected from 1990 to 2006. In totally 706 cases of renal cell carcinoma studied, there were 21 MCRCC cases identified. The clinical and pathological features were assessed, immunohistochemical staining was performed, and loss of heterozygosity (LOH) and microsatellite instability (MSI) were assessed using four microsatellite markers on chromosomes 3, 9 and 14.
Of the 21 patients, the age ranged from 34 to 72 years (mean 50 years), 19 were male and two female. Tumors were found incidentally in 18 patients during physical examination, three patients had anemia or microhematuria. Among the 21 patients, 10 tumors were in the left kidney and 11 in the right. Eighteen patients were stage T1, two stage T2, and one stage T3 with perinephric tissue involvement. Follow up information was available in 20 patients, all showed no evidence of tumor recurrence or metastasis. Grossly, the tumor size ranged from 0.3 cm to 10.0 cm in the greatest dimension, consisting of multilocular cysts with variable sizes which contained light yellow, colloid or hemorrhagic fluid. The septae varied in thickness (ranged 0.1 cm to 0.5 cm, mean 0.2 cm). Microscopically the cysts were lined by single to multilayered epithelial cells with clear or lightly eosinophilic cytoplasm. There were clusters of clear cells seen in the septae stroma. Sixteen tumors were of Fuhrman grade 1, and five were of Fuhrman grade 2. Immunohistochemically, the clear cells were positive for vimentin, ABC, CAM5.2 and EMA. Six samples were positive for CD10, and 16 were positive for NSE. Among 21 patients, PCR amplification was successful in 11 patients. Microsatellite alterations were found in five patients. LOH was observed in 3 of 11 MCRCC (27%), two were at D3S1560 locus, and one at D14S617 locus. MSI frequency was identified in 2 of 11 MCRCC (18%), locating at D9S168 or D14S617 locus, respectively.
MCRCC is an uncommon tumor of kidney, constituting 2.9% of all RCC enrolled into the study. It has distinctive clinical and pathological characteristics with an excellent outcome. Results indicated that MCRCC is a rare entity with low malignant potential.
评估多房囊性肾细胞癌（MCRCC）的临床病理特征及分子遗传学改变。
回顾性分析1990年至2006年长海医院病理科存档的所有数据。在研究的706例肾细胞癌病例中，共确诊21例MCRCC。评估其临床和病理特征，进行免疫组化染色，并使用位于3号、9号和14号染色体上的四个微卫星标记评估杂合性缺失（LOH）和微卫星不稳定性（MSI）。
21例患者年龄在34至72岁之间（平均50岁），男性19例，女性2例。18例患者在体检时偶然发现肿瘤，3例患者有贫血或镜下血尿。21例患者中，10例肿瘤位于左肾，11例位于右肾。18例患者为T1期，2例为T2期，1例为T3期且伴有肾周组织受累。20例患者有随访信息，均未发现肿瘤复发或转移迹象。大体上，肿瘤最大径为0.3至10.0 cm，由大小不等的多房囊肿组成，囊内含淡黄色、胶冻样或血性液体。分隔厚度不一（范围为0.1至0.5 cm，平均0.2 cm）。镜下囊肿内衬单层至多层上皮细胞，胞质清亮或轻度嗜酸性。分隔间质中有散在的透明细胞团。16例肿瘤为Fuhrman 1级，5例为Fuhrman 2级。免疫组化显示，透明细胞波形蛋白、ABC、CAM5.2和EMA阳性。6例样本CD10阳性，16例NSE阳性。21例患者中，11例成功进行了PCR扩增。5例患者发现微卫星改变。11例MCRCC中有3例（27%）观察到LOH，2例位于D3S1560位点，1例位于D14S617位点。11例MCRCC中有2例（18%）检测到MSI，分别位于D9S168或D14S617位点。
MCRCC是一种少见的肾脏肿瘤，占本研究纳入的所有肾细胞癌的2.9%。它具有独特的临床和病理特征，预后良好。结果表明，MCRCC是一种罕见的实体瘤，恶性潜能低。
