Huang Kuo-How, Huang Chao-Yuan, Chung Shiu-Dong, Pu Yeong-Shiau, Shun Chia-Tung, Chen Jun
Department of Urology, National Taiwan University Hospital, Taipei, Taiwan.
J Formos Med Assoc. 2007 Feb;106(2 Suppl):S51-4. doi: 10.1016/s0929-6646(09)60353-3.
Angiomyolipoma (AML) is a common benign renal tumor composed of thick-walled blood vessels, smooth muscle, and adipose tissue, but the malignant epithelioid variant is extremely rare. A 78-year-old woman presented with fever and left flank pain for 3 days. Computed tomography showed a heterogeneously enhanced mass without fat density in the left kidney. Radical nephrectomy was performed and pathology showed malignant epithelioid AML with regional lymph node metastases. The tumor cells were positive for human melanosome-associated protein (HMB-45) on immunohistochemical staining. The patient died of disseminated metastases (lungs and bones) 5 months postoperatively. Epithelioid AML is a potentially aggressive tumor. The prognosis is poor in metastatic disease. HMB-45 immunoreactivity is a useful marker to make diagnosis.
血管平滑肌脂肪瘤(AML)是一种常见的良性肾肿瘤,由厚壁血管、平滑肌和脂肪组织组成,但恶性上皮样变体极为罕见。一名78岁女性因发热和左侧腰痛3天就诊。计算机断层扫描显示左肾有一个不均匀强化的肿块,无脂肪密度。行根治性肾切除术,病理显示为恶性上皮样AML伴区域淋巴结转移。免疫组织化学染色显示肿瘤细胞中人黑素体相关蛋白(HMB-45)呈阳性。患者术后5个月死于广泛转移(肺和骨)。上皮样AML是一种具有潜在侵袭性的肿瘤。转移性疾病的预后较差。HMB-45免疫反应性是进行诊断的有用标志物。