Seyam Raouf M, Alkhudair Waleed K, Kattan Said A, Alotaibi Mohamed F, Alzahrani Hassan M, Altaweel Waleed M
Department of Urology, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
J Kidney Cancer VHL. 2017 Oct 16;4(4):13-25. doi: 10.15586/jkcvhl.2017.97. eCollection 2017.
Renal angiomyolipoma (RAML), though a rare benign tumor, may impose a significant morbidity or even mortality due to its unique characteristics and the complications subsequent to its treatment. The classic tumor variant is composed of smooth muscular, vascular, and fatty components. The most straightforward diagnosis is when the fat component is abundant and gives a characteristic appearance on different imaging studies. In fat-poor lesions, however, the diagnosis is difficult and presumed a renal cell carcinoma. Yet, some variants of RAML, though rare, express an aggressive behavior leading to metastasis and mortality. The challenge lies in the early detection of benign variants and identifying aggressive lesions for proper management. Another challenge is when the vascular tissue component predominates and poses a risk of hemorrhage that may extend to the retroperitoneum in a massive life-threatening condition. The predicament here is to identify the characteristics of tumors at risk of bleeding and provide a prophylactic treatment. According to the clinical presentation, different treatment modalities, prophylactic or therapeutic, are available that span the spectrum of observation, embolization, or surgery. Renal impairment may result from extensive tumor burden or as a complication of the management itself. Improvement of diagnostic techniques, super-selective embolization, nephron-sparing surgery, and late treatment with the mammalian target of rapamycin inhibitors have provided more effective and safe management strategies. In this review, we examine the evidence pertaining to the risks imposed by RAML to the patients and identify merits and hazards associated with different treatment modalities.
肾血管平滑肌脂肪瘤（RAML）虽是一种罕见的良性肿瘤，但因其独特的特征及治疗后的并发症，可能导致严重的发病甚至死亡。典型的肿瘤变体由平滑肌、血管和脂肪成分组成。最直接的诊断是当脂肪成分丰富且在不同影像学检查中呈现出特征性表现时。然而，在脂肪含量少的病变中，诊断困难，常被误诊为肾细胞癌。不过，一些RAML变体虽罕见，但具有侵袭性，可导致转移和死亡。挑战在于早期发现良性变体并识别侵袭性病变以进行恰当管理。另一个挑战是当血管组织成分占主导且存在出血风险时，出血可能会在危及生命的严重情况下蔓延至腹膜后。这里的困境是识别有出血风险的肿瘤特征并提供预防性治疗。根据临床表现，有不同的治疗方式可供选择，包括预防性或治疗性的，涵盖观察、栓塞或手术等。肾功能损害可能源于广泛的肿瘤负荷或作为治疗本身的并发症。诊断技术的改进、超选择性栓塞、保留肾单位手术以及使用雷帕霉素哺乳动物靶点抑制剂进行后期治疗，提供了更有效和安全的管理策略。在本综述中，我们研究了与RAML给患者带来的风险相关的证据，并确定了不同治疗方式的优缺点。
