摘要
伴血管母细胞瘤成分透明细胞肾细胞癌(clear cell renal cell carcinoma with hemangioblastoma like features,ccRCC-HBc)是一种罕见的特殊肾细胞癌类型,单纯从影像学表现无法与透明细胞肾细胞癌鉴别,诊断主要依靠病理学.本文报告1例ccRCC-HBc患者,女,56岁,外院上腹部CT发现右肾占位10 d入院,实验室检查示:天冬氨酸氨基转移酶为53.8 U/L,血糖为8.62 mmol/L.肾磁共振平扫影像表现为右肾下极内见结节状长T1、长T2异常信号,磁共振高分辨率T2WI抑制序列(fat saturation T2-weighted imaging,FS-T2WI)呈高信号,磁共振弥散加权成像(diffusion weighted imaging,DWI)呈略高信号,病灶边界清,直径约为1.5 cm,患者于泌尿外科接受手术切除肿物.低倍镜下肿瘤有2种区域,即透明细胞肾细胞癌(clear cell renal cell carcinoma,ccRCC)区域和血管母细胞瘤(hemangioblastoma,HB)区域.免疫组织化学染色示:配对盒基因8抗体(paired box protein 8,Pax-8)(+),碳酸酐酶IX(carbonic anhydrase IX,CAIX)(+),琥珀酸脱氢酶复合物铁硫亚基B(succinate dehydrogenase complex iron-sulfur subunit B,SDHB)(+),上皮膜抗原(epithelial membrane antigen,EMA)(个别+),证实为ccRCC-HBc.通过对其临床病理特征的分析,可加强临床医师对该类肾罕见肿瘤的认识.
Abstract
Clear cell renal cell carcinoma with hemangioblastoma like features(ccRCC-HBc)is a rare and distinct subtype of renal cell carcinoma that cannot be distinguished from conventional clear cell renal cell carcinoma(ccRCC)based solely on imaging.The diagnosis primarily relies on pathological examination.This paper reports a case of ccRCC-HBc in a 56-year-old female patient.A mass in the right kidney was detected by abdominal CT at an outside hospital 10 days prior to admission.Laboratory tests showed an aspartate aminotransferase level of 53.8 U/L and a blood glucose level of 8.62 mmol/L.Renal magnetic resonance(MRI)of the kidney revealed a nodular lesion in the lower pole of the right kidney,showing long T1 and long T2 signals.The lesion exhibited high signal intensity on high-resolution fat-saturation T2-weighted imaging(FS-T2WI)and slightly high signal intensity on diffusion-weighted imaging(DWI).The lesion had clear boundaries with a diameter of approximately 1.5 cm.The patient underwent surgical resection of the tumor in the urology department.Histologically,the tumor displayed two distinct regions:a ccRCC region and a hemangioblastoma region.Immunohistochemical staining revealed positive markers for paired box protein 8(Pax-8),carbonic anhydrase IX(CAIX),and succinate dehydrogenase complex iron-sulfur subunit B(SDHB),with epithelial membrane antigen(EMA)being sporadically positive,confirming the diagnosis of ccRCC-HBc.Analyzing the clinicopathological features of this case can help clinicians better understand this rare type of renal tumor.
维普
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