【摘要】：目的探討Von-Hippel-Lindau(VHL)病并發(fā)腎癌等疾病的臨床特點(diǎn)。方法回顧分析山東大學(xué)第二醫(yī)院收治的2例VHL病并發(fā)腎癌的患者,并結(jié)合文獻(xiàn)復(fù)習(xí)。結(jié)果 2例中1例有家族史,行右腎癌根治術(shù),病理顯示為腎透明細(xì)胞癌,術(shù)后隨訪1年,未見復(fù)發(fā);1例無(wú)相關(guān)疾病家族史,先行右腎癌根治術(shù),病理顯示右腎多房性囊性腎細(xì)胞癌,右腎上腺嗜鉻細(xì)胞瘤,術(shù)后4年左腎及左側(cè)腎上腺出現(xiàn)腫瘤復(fù)發(fā),左腎腫瘤穿刺病理提示腎透明細(xì)胞癌,給予分子靶向藥物索拉菲尼治療,現(xiàn)規(guī)律透析。結(jié)論 VHL病是涉及多個(gè)系統(tǒng)的腫瘤綜合征,臨床較為少見,其并發(fā)腎癌的特征不同于散發(fā)性腎癌;早診斷、早進(jìn)行個(gè)體化治療可改善患者晚期生存質(zhì)量。
[Abstract]:Objective to investigate the clinical features of Von-Hippel-Lindau disease (VHL) complicated with renal cell carcinoma (RCC). Methods two patients with VHL complicated with renal cell carcinoma were reviewed retrospectively. Results one case with family history underwent radical resection of right renal cell carcinoma (RRCC), and the pathological findings were clear cell carcinoma of kidney (RCC). After one year of follow-up, no recurrence was found in 1 case without any family history of related diseases. Radical resection of RRCC was performed first. Pathological findings showed that right renal multilocular cystic renal cell carcinoma, right adrenal pheochromocytoma, left kidney and left adrenal gland recurred 4 years after operation, left renal tumor puncture pathology suggested renal clear cell carcinoma, and was treated with molecular targeted drug Solafenil. Now regular dialysis. Conclusion VHL disease is a tumor syndrome involving multiple systems, and its clinical features are different from those of sporadic renal cell carcinoma. Early diagnosis and early individualized treatment can improve the quality of life of patients with advanced stage.
【作者單位】： 山東大學(xué)第二醫(yī)院泌尿外科;
【基金】：山東省科技發(fā)展計(jì)劃(2014GSF118150)
【分類號(hào)】：R737.11

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