Department of Urology, Stony Brook University Medical Center, Stony Brook, NY 11794, USA.
J Pediatr Surg. 2012 Jan;47(1):e23-6. doi: 10.1016/j.jpedsurg.2011.10.003.
A 9-year-old girl had hypertension (systolic blood pressure of 125 mm Hg) noted at an annual well child visit. An ultrasound study demonstrated a large right renal cystic mass. A partial nephrectomy was performed. The surgical specimen was 9.7 × 9.4 × 6.4 cm and weighed 413.2 g. The tumor stained diffusely positive for smooth muscle actin and focally positive for factor VIII. Final histologic diagnosis was primary intrarenal lymphatic malformation. The case is unusual because of the presentation, size of the mass, as well as the therapeutic approach of partial nephrectomy.
一位 9 岁女孩在年度健康儿童体检时发现高血压（收缩压为 125mmHg）。超声研究显示右侧巨大的肾囊肿性肿块。行部分肾切除术。手术标本大小为 9.7×9.4×6.4cm，重量为 413.2g。肿瘤弥漫性肌动蛋白阳性，VIII 因子局灶阳性。最终组织学诊断为原发性肾内淋巴管畸形。该病例不同寻常，因为其表现、肿块大小以及部分肾切除术的治疗方法。
