多房囊性肾细胞癌32例临床病理分析

Clinicopathological features of multilocular cystic renal cell carcinoma:a series of 32 cases

目的探讨多房囊性肾细胞癌（multilocular cystic renal cell carcinoma,MCRCC）的临床病理特征,提高对该肿瘤的认识。方法复习32例MCRCC的临床资料,观察其病理学形态和免疫表型特征,结合随访资料评价其预后。结果 32例中13例位于左肾,18例位于右肾,1例双肾;肿瘤平均最大径4.6 cm（1.0-8.0 cm）;男女比为2.2∶1。11例行根治性肾全切术,21例行肾部分切除术。肿瘤均为多房囊性,缺少实体成分,囊壁内衬单层（偶为多层或小乳头状）胞质透明或淡粉染、Fuhrman核1级的瘤细胞,腔面富于薄壁血管。瘤细胞表达CK（32/32）、CK7（25/32）、EMA（32/32）、CD10（23/32）、vimentin（20/32）,均不表达CD68。术后随访5-140个月,均未见复发和转移,无肿瘤相关死亡病例。结论 MCRCC的瘤细胞核级低、无实性瘤巢;囊壁腔面衬覆胞质透亮或淡粉染细胞伴丰富薄壁血管是诊断线索,免疫表型有助于诊断,患者一般预后良好。

Purpose To investigate the clinicopathological features of multilocular cystic renal cell carcinoma（ MCRCC）,and to improve the understanding of this disease. Methods Thirty-two of MCRCC were studied by clinic data,pathological features and immunophenotype. All the thirty-two cases were followed up. Results In this study,thirty-two patients were diagnosed as MCRCC with a male-to-female ratio of 2. 2 ∶ 1,thirteen of whom had the tumor in the left kidney,eighteen in the right kidney,another in the double kidneys. The mean of tumor diameter was 4. 6 cm（ 1. 0 ~ 8. 0 cm）. Eleven patients underwent radical nephrectomy while the other 21 patients received nephron sparing surgery. Microscopically,all the cases were multiocular,lined in the cyst wall by a single layer of tumor cells with the clear or pale cytoplasm and Fuhrman grade 1 nuclei. Occasionally,the lining consisted of several layers of tumor cells or a few small papillae were present. The linings of the cyst wall were rich in thin-wall blood vessels. Immunohistochemically,the tumor cells were positive for CK（ 32 /32）,CK7（ 25 /32）,EMA（ 32 /32）,CD10（ 23 /32） and vimentin（ 20 /32）,while negative for CD68. Conclusions MCRCC is characteristic by low grade nuclei,lacking solid nodules,and in the cyst wall,and has a favourable prognosis. The rich in thin-wall blood vessels and the lining cells with the clear or pale cytoplasm are diagnosis clues.