Maulana Azad Medical College, New Delhi, India.
Ann R Coll Surg Engl. 2021 Jun;103(6):e184-e188. doi: 10.1308/rcsann.2020.7036. Epub 2021 May 6.
Angiomyolipoma is a benign solid renal neoplasm. A giant angiomyolipoma is more than 10cm by size, but it can grow to huge proportions. Our case appears to be the third largest angiomyolipoma and the largest among bilateral giant renal angiomyolipoma in the indexed literature. A 26-year-old man presented with large right abdominal swelling for the past three years, which was occupying his right flank and iliac region, extending beyond the midline. Computed tomography of the abdomen revealed a large well-defined mass in the right side of the abdomen, crossing the midline and measuring 35 × 20 × 12cm. The left kidney showed a similar fatty lesion of 14 × 6cm. The findings were consistent with angiomyolipoma. Further evaluation for tuberous sclerosis by magnetic resonance imaging the brain demonstrated multiple subependymal nodules. Giant renal angiomyolipoma is an uncommon tumour with bilateral giant angiomyolipoma being a rare entity. Preoperative embolisation helps in reducing size of the tumour. In case of giant and bilateral angiomyolipoma, evaluation for tuberous sclerosis should always be done.
血管平滑肌脂肪瘤是一种良性的肾脏实体肿瘤。巨血管平滑肌脂肪瘤的大小超过 10cm,但它可以长到巨大的比例。我们的病例似乎是第三大血管平滑肌脂肪瘤,也是索引文献中双侧巨大肾血管平滑肌脂肪瘤中最大的一个。一名 26 岁男性因过去三年右侧腹部肿胀就诊,肿胀占据了他的右侧腰部和髂区,超出了中线。腹部 CT 显示右侧腹部有一个大而清晰的肿块,穿过中线,大小为 35×20×12cm。左肾有一个类似的脂肪病变,大小为 14×6cm。这些发现与血管平滑肌脂肪瘤一致。对脑部磁共振成像进行结节性硬化症的进一步评估显示多个室管膜下结节。巨大的肾血管平滑肌脂肪瘤是一种不常见的肿瘤,双侧巨大的肾血管平滑肌脂肪瘤则是一种罕见的实体瘤。术前栓塞有助于缩小肿瘤的大小。对于巨大和双侧的血管平滑肌脂肪瘤,应始终进行结节性硬化症的评估。