透明细胞乳头状肾细胞癌9例临床病理分析

Clear cell papillary renal cell carcinoma:a clinicopathologic analysis of 9 cases

目的探讨透明细胞乳头状肾细胞癌（CCPRCC）的临床病理特征、鉴别诊断与预后。方法复习9例CCPRCC的临床资料，进行组织病理学形态和免疫组化观察。结果9例中男性7例，女性2例，年龄38～68岁，平均年龄51．9岁。肿瘤直径1．2～7em，平均3．5121／1；8例为单发病例。9例均为查体时发现，无终末期。肾病及VHL病家族史。镜下肿瘤由数量不等的腺泡状、乳头状、囊状和实性巢状结构组成，细胞核形态温和，远离基膜并朝向管腔、腺泡、乳头表面呈整齐的单层线状排列，间质内可见数量不等的平滑肌组织。免疫组化：CK、vimentin、CAIX和CK7均强（＋），CD10、P504S和RCC灶性弱（＋），CD117和TFE3均（-）。随访6个月～5年，均无复发和远处转移。结论CCPRCC为一种少见的惰性肾肿瘤，典型的组织学表现结合免疫组化结果通常可以明确诊断。手术切除是首选治疗方法，总体预后良好。

Objective To investigate the clinical pathological characteristics, diagnosis, differential diagnosis and prognosis of clear cell papillary renal cell carcinoma （CCPRCC）. Methods We reviewed nine patients with CCPRCC and studied by histopathological observation and immunohistochemical staining. Results Nine males and two female patients were reviewed with an average age of 51.9 years. The average diameter of tumors was 3.5 cm （ range from 1.2 to 7 cm） and 8 of them were single. They were found by medical examination and no end-stage renal disease and family history of VHL disease. Histologically, all of the 11 cases of renal cell carcinoma showed acinar, papillary, cystic and solid nest structures; the nuclei were bland, away from the basement membrane and toward the surface of lumen, acini and papillae, which were arranged in a single line of arrangement. Smooth muscle tissue was noted in the stroma. Immunohistochemical study showed strongly positive staining for CK, vimentin, CAIX and CK7 in all cases; CD10, P504S and RCC were focal weakly positive. All the cases were negative for CDll7 and TFE3. These patients were followed up for 6 months to 5 years, and they had no recurrence and metastasis. Conclusions CCPRCC is a rare inert renal tumor which can usually be diagnosed by typical histological changes and the immunohistochemical findings. Surgical resection is curative for these patients and the prognosis is relatively good.