Monga G, Ramponi A, Falzoni P U, Boldorini R
Dipartimento di Scienze Mediche, II Facoltà di Medicina e Chirurgia di Novara, Università di Torino, Italy.
Pathol Res Pract. 1994 Dec;190(12):1208-11; discussion 1212-3. doi: 10.1016/S0344-0338(11)80451-6.
A renal neoplasia, displaying the typical features of angiomyolipoma (an admixture of smooth muscle cells, mature adipocytes and tangles of thick-walled blood vessels) was found in a nine-year-old boy. In addition, cytoplasmic crystalloid structures and HMB 45 positivity were found in smooth muscle cells. Similar findings were also observed in a small nodule of the liver. According to literature data, angiomyolipoma is very rare in children and almost exclusively associated with tuberous sclerosis. Our case suggest that the diagnostic hypothesis of angiomyolipoma can not be disregarded when a renal mass is identified in a young patient even in a clinical setting other than tuberous sclerosis.
在一名9岁男孩中发现了一种肾脏肿瘤，其表现出血管平滑肌脂肪瘤的典型特征（平滑肌细胞、成熟脂肪细胞和厚壁血管束的混合）。此外，在平滑肌细胞中发现了细胞质晶体结构和HMB 45阳性。在肝脏的一个小结节中也观察到了类似的发现。根据文献资料，血管平滑肌脂肪瘤在儿童中非常罕见，几乎仅与结节性硬化症相关。我们的病例表明，即使在结节性硬化症以外的临床环境中，当在年轻患者中发现肾脏肿块时，血管平滑肌脂肪瘤的诊断假设也不能被忽视。
