本文選題：腎上皮樣血管平滑肌脂肪瘤 + 臨床特點��； 參考：《山東大學》2017年碩士論文

【摘要】：目的:探討腎上皮樣血管平滑肌脂肪瘤的臨床特點、病理特點、診斷方法、治療及預(yù)后判斷。提高對腎上皮樣血管平滑肌脂肪瘤(EAML)的認識及診療水平。材料及方法:本次研究我們回顧性分析了山東省立醫(yī)院泌尿外科于2017年3月3日收治的1例腎上皮樣血管平滑肌脂肪瘤患者的臨床資料�；颊吲�,37歲,因"間斷性肉眼血尿1月余"入院,當?shù)蒯t(yī)院B超檢查示:右腎實性占位(Ca?)。CT檢查示:右腎惡性腫瘤,腫瘤體積7.3cmx9.4cmx10.5cm,腫瘤形態(tài)不規(guī)則,動態(tài)增強掃描,動脈期腫瘤強化不明顯,靜脈期、腎實質(zhì)期病灶明顯強化,但強化程度較正常減低,病灶與正常腎臟對比輕度強化。病灶突破腎周脂肪囊,突破腎盂并擠壓下腔靜脈,邊界欠清晰。腹膜后未見腫大淋巴結(jié)�；颊哂�2017年3月6日在氣管插管全麻下行腹腔鏡根治性右腎切除術(shù)。檢索中國知網(wǎng)和Pubmed等數(shù)據(jù)庫中關(guān)于腎EAML的文獻復(fù)習并討論。結(jié)果:患者于2017年3月6日行腹腔鏡下右腎根治性切除術(shù)。術(shù)后病理(病理號:S1702549):(右)腎惡性上皮樣血管平滑肌脂肪瘤(11x9cm),輸尿管切線及腎門血管未查見腫瘤。免疫組化染色黑色素瘤相關(guān)抗原(HMB-45)強陽性、MelanA陽性�；颊咝g(shù)后7天后恢復(fù)良好,順利出院,未行放、化療等輔助治療,隨訪至今暫未無復(fù)發(fā)及轉(zhuǎn)移。結(jié)論及意義:腎血管平滑肌脂肪瘤(AML)是一個良性的病變過程。腎上皮樣血管肌脂肪瘤(EAML)是腎臟血管肌脂肪瘤一種罕見的變異類型。2004年世界衛(wèi)生組織男性和泌尿生殖系統(tǒng)腫瘤將它定義為"一種以上皮細胞為主的具有擴散的特征的潛在的惡性間質(zhì)腫瘤"。EAML主要由類上皮細胞、梭形細胞和巨細胞組成,不包含或只有少量的脂肪瘤的組織,其影像學檢查極易誤診為腎細胞癌或肉瘤。其治療主要依靠手術(shù)切除,因病例罕見,研究較少,化療效果尚無肯定結(jié)論。腎EAML的確診主要依靠術(shù)后病理,尤其是免疫組織化學檢查,特征表現(xiàn)為腫瘤細胞顯示HMB-45、MelanA強陽性。約有1/3發(fā)展為惡性,惡性EAML以局部浸潤以及淋巴結(jié)轉(zhuǎn)移為主,預(yù)后總體較差。其治療手段主要為手術(shù)切除,對于化療藥物及靶向治療的研究很多,表柔比星、mTOR抑制劑依維莫司、吉西他濱等藥物短期可能有效,但長期效果不明顯。隨著研究的不斷深入,可以發(fā)現(xiàn)腎EAML的可能的活躍靶點,進而為腎EAML輔助治療找到新方向。
[Abstract]:Objective: to investigate the clinical features, pathological features, diagnosis, treatment and prognosis of renal epithelioid angiomyolipoma. To improve the understanding and diagnosis and treatment of renal epithelioid angiomyolipoma (EAML). Materials and methods: we retrospectively analyzed the clinical data of a case of renal epithelioid angiomyolipoma treated in Urology Department of Shandong Provincial Hospital on March 3 2017. The patient was 37 years old. She was admitted to hospital because of "intermittent hematuria for more than one month." B-ultrasound examination in local hospital showed that the right renal solid space occupying place was Cajiao. Ct examination showed that the tumor volume was 7.3cmx9.4 cmx10.5cm, the shape of the tumor was irregular, and the dynamic contrast enhanced scan was performed, and the tumor size was 7.3cmx9.4 cmx10.5cm. The enhancement of tumor in arterial phase was not obvious, while in venous phase, renal parenchyma phase was obviously enhanced, but the enhancement degree was lower than that in normal stage, and the lesion was slightly enhanced compared with normal kidney. The lesion broke through the perirenal fat sac, broke through the renal pelvis and squeezed the inferior vena cava, and the boundary was unclear. No enlarged lymph nodes were seen behind the peritoneum. The patient underwent laparoscopic radical right nephrectomy under tracheal intubation and general anesthesia on March 6, 2017. To search for literature review and discussion on renal EAML in the databases of Chinese knowledge Network and Pubmed and so on. Results: laparoscopic radical nephrectomy was performed on March 6, 2017. Postoperative pathology (pathological number: S1702549% (right) malignant renal epithelioid angiomyolipoma (11 x 9 cm), ureteral tangent line and renal hilar vessel were not found. The melanoma associated antigen (HMB-45) was strongly positive and MelanA was positive. The patient recovered well 7 days after operation and was discharged smoothly, without radiotherapy, chemotherapy and other adjuvant treatment. There was no recurrence or metastasis during the follow-up. Conclusion and significance: renal angiomyolipoma (AMLA) is a benign process. Renal epithelioid angiomyolipoma (EAMLL) is a rare variant of renal angiomyolipoma. In 2004, WHO defined it as "a proliferative characteristic of epithelial cells in men and urogenital tumors." The underlying malignant interstitial neoplasms ". EAML mainly consists of epithelioid cells, A tissue consisting of spindle cells and giant cells that does not contain or contain only a small number of lipomas, and whose imaging findings are easily misdiagnosed as renal cell carcinoma or sarcoma. Its treatment mainly depends on surgical resection, because the case is rare, the research is less, the chemotherapeutic effect does not have the definite conclusion. The diagnosis of renal EAML mainly depends on postoperative pathology, especially immunohistochemical examination, which is characterized by tumor cells showing strong positive for HMB-45 and Melan A. About one third of the patients developed malignancy, and local invasion and lymph node metastasis were dominant in malignant EAML, and the prognosis was generally poor. Surgical resection is the main treatment method. There are many studies on chemotherapeutic drugs and targeted therapy. Evimoxus and gemcitabine inhibitors of epirubicin may be effective in the short term, but the long-term effect is not obvious. With the development of research, the possible active target of renal EAML can be found, and a new direction for renal EAML adjuvant therapy can be found.
【學位授予單位】：山東大學
【學位級別】：碩士
【學位授予年份】：2017
【分類號】：R737.11

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