肾上皮样血管平滑肌脂肪瘤的研究进展

发布时间：2018-05-13 04:11

本文选题：肾脏肿瘤 + 上皮样血管平滑肌脂肪瘤　；参考：《河北医科大学》2017年硕士论文

【摘要】：肾上皮样血管平滑肌脂肪瘤(renal epithelioid angiomyolipoma,REAML)是一种临床极其罕见的肾脏实性肿瘤,具有恶变潜能的特性,属于血管周围上皮样细胞瘤肿瘤家族(PEComa),其在组织学上主要由上皮样细胞组成。在恶性REAML组织中可以发现P53基因突变,可能在REAML的恶性转化中起了关键作用。由于临床医师对其认识尚浅,临床表现无特异性,常规影像学检查不能明确诊断,因此临床诊断难度比较大,容易导致误诊为肾细胞癌,病理结果及免疫组化表型结果是诊断和鉴别诊断的有效依据,首选黑色素相关蛋白HMB-45和Melan-A。REAML目前尚无有效治疗方法,但临床上仍首选外科手术治疗,术后仍有可能出现复发及转移,则预后较差,生存期较短,死亡率较高,需长期密切随访观察。对于不能手术治疗的患者,可试用化疗、m TOR抑制剂或伊马替尼等新型靶向药物治疗,但临床疗效仍有待进一步验证。
[Abstract]:Renal epithelioid angiomyolipoma (REAMLR) is a rare clinical solid renal tumor with malignant potential. It belongs to the tumor family of perivascular epithelioid cell tumor (PEComaI), which is mainly composed of epithelioid cells in histology. P53 gene mutation can be found in malignant REAML tissues, which may play a key role in the malignant transformation of REAML. Because the clinicians know little about it, the clinical manifestation is not specific, and the routine imaging examination can not make a definite diagnosis, so the clinical diagnosis is difficult, and it is easy to be misdiagnosed as renal cell carcinoma (RCC). Pathological results and immunohistochemical phenotypic results are the effective basis for diagnosis and differential diagnosis. There is no effective treatment method for melanin associated protein HMB-45 and Melan-A.REAML, but surgery is still the first choice in clinic. Recurrence and metastasis may still occur after the operation, the prognosis is poor, the survival time is shorter, the mortality is higher, and it is necessary to follow up closely for a long time. New targeted drugs such as chemotherapeutic TOR inhibitor or imatinib can be used in patients who can not be treated surgically, but the clinical efficacy needs to be further verified.
【学位授予单位】：河北医科大学
【学位级别】：硕士
【学位授予年份】：2017
【分类号】：R737.11

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