Deresse Tilahun, Bogale Mandante, Alemayehu Dawit, Dessalegn Megbar, Seid Marta
Department of Surgery, School of Medicine, Debre Berhan University, 445, Ethiopia.
Department of Surgery, School of Medicine, Debre Markos University, 269, Ethiopia.
Int J Surg Case Rep. 2023 Aug;109:108538. doi: 10.1016/j.ijscr.2023.108538. Epub 2023 Jul 20.
Renal angiomyolipoma (AML), which is a rare solid kidney tumor with benign characteristics, also known as a renal hamartoma, can exhibit various clinical symptoms and severe consequences may arise if the lesion becomes large.
A 58-year-old woman was admitted to a hospital, with general fatigue, abdominal swelling, and epigastric fullness. Upon examination, a large mass was palpated, which occupied almost the entire right abdomen. The abdominal computed tomography scan revealed a large right renal mass measuring 22 × 18 × 8 cm, which was exophytic and heterogeneous with a large fat component and an enhancing solid part. The tumor was successfully excised through a generous right subcostal incision with left-side extension. The total weight of the resected specimen was 2500 g, which appears to be the largest angiomyolipoma ever resected in Ethiopia.
Renal AML, a benign tumor derived from mesenchymal components, is sometimes referred to as a "hamartoma" due to its variable makeup. The most common complaints of patients with renal AML are lower back pain, hematuria, and physical finding of hypotension (shock), though patients with giant AML, as in this case, may also experience gastrointestinal symptoms due to the mass' compression.
Although treatment options requiring contemporary medical technologies and skilled manpower are difficult to offer in set ups of resource-limited countries, such as the one we reported from, giant renal angiomyolipoma can be treated safely with open nephrectomy.
肾血管平滑肌脂肪瘤（AML）是一种罕见的具有良性特征的实性肾肿瘤，也被称为肾错构瘤，可表现出多种临床症状，如果病变变大可能会产生严重后果。
一名58岁女性因全身乏力、腹胀和上腹部饱胀入院。检查时可触及一个巨大肿块，几乎占据了整个右腹。腹部计算机断层扫描显示右肾有一个巨大肿块，大小为22×18×8厘米，呈外生性且不均匀，有大量脂肪成分和强化的实性部分。通过一个宽大的右侧肋下切口并向左延伸，成功切除了肿瘤。切除标本的总重量为2500克，这似乎是埃塞俄比亚有史以来切除的最大的血管平滑肌脂肪瘤。
肾AML是一种源自间充质成分的良性肿瘤，由于其组成成分多样，有时被称为“错构瘤”。肾AML患者最常见的主诉是腰痛、血尿和低血压（休克）的体格检查发现，不过像本病例中的巨大AML患者，由于肿块压迫也可能出现胃肠道症状。
尽管在资源有限的国家，如我们报道的这种情况下，难以提供需要当代医疗技术和熟练人力的治疗选择，但巨大肾血管平滑肌脂肪瘤可以通过开放性肾切除术安全治疗。
