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Epithelioid angiomyolipoma of the kidney: pathological features and clinical outcome in a series of consecutively resected tumors

机译:肾脏上皮样血管平滑肌脂肪瘤:一系列连续切除的肿瘤的病理特征和临床结果

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The 2004 World Health Organization classification of tumors defines epithelioid angiomyolipoma of kidney as a potentially malignant mesenchymal neoplasm with reported metastasis in approximately one-third of the cases. However, this conclusion was based primarily on individual case reports and small retrospective series. More recently reported larger series have shown varying results. We reviewed 437 consecutive renal angiomyolipomas with primary resection at three tertiary-care institutions with high nephrectomy volumes. Only tumors showing >80% epithelioid histology were included in this study. Tumors resected elsewhere and reviewed in consultation were not included. Twenty of these 437 (4.6%) were classified as epithelioid angiomyolipoma. The female to male ratio was 11:9, mean age 49.7 (range, 30–80) years, and mean tumor size 8.7 (range, 1–25)?cm. Microscopic tumor necrosis was present in 10 (50%) tumors and mitotic activity (range, <1–5/10 high power fields) in 8 (40%); atypical mitoses were seen in only 1 (5%) tumor. Pleomorphic ganglion-like or multinucleated giant cells were seen in 18 (90%) tumors. With a mean follow-up of 82.5 (range, 1–356) months, seventeen patients were alive with no-evidence-of-disease at the time of last follow-up; two patients died of unrelated causes with no-evidence-of-disease, and one patient (5%) developed distant metastases. Our data, based on consecutively resected angiomyolipomas with long clinical follow-up, suggests that epithelioid angiomyolipomas constitute a small proportion of all angiomyolipomas, and the rate of aggressive behavior among epithelioid angiomyolipomas, even when showing morphologic features previously reported to portend aggressive clinical behavior, is very low.
机译:2004年世界卫生组织的肿瘤分类将肾脏的上皮样血管平滑肌脂肪瘤定义为潜在的恶性间质肿瘤,据报道约有三分之一的病例发生了转移。但是,该结论主要基于个案报告和小型回顾性系列。最近报道的较大系列已显示出不同的结果。我们回顾了在高肾脏切除术量的三个三级医疗机构进行的437例连续性肾脏血管平滑肌脂肪瘤的初步切除。本研究仅包括显示> 80%的上皮样组织学的肿瘤。不包括在其他地方切除并经会诊复查的肿瘤。在这437个样本中,有20个(4.6%)被分类为上皮样血管平滑肌脂肪瘤。男女之比为11:9,平均年龄49.7(范围30–80)岁,平均肿瘤大小8.7(范围1–25)?cm。微观肿瘤坏死存在于10个(50%)肿瘤中,有丝分裂活性(范围,<1-5 / 10高倍视野)存在于8个(40%)中。仅1个(5%)肿瘤中可见非典型的有丝分裂。在18(90%)肿瘤中发现了多形神经节样或多核巨细胞。平均随访82.5个月(范围1–356个月),有17例患者在最后一次随访时还没有疾病证据。 2例患者死于无关病因而无疾病证据,1例患者(5%)发生远处转移。我们基于连续切除的血管平滑肌脂肪瘤并经过长期临床随访的数据表明,上皮样血管平滑肌脂肪瘤占所有血管平滑肌脂肪瘤的比例很小,并且在上皮样血管平滑肌脂肪瘤之间的侵袭行为发生率也很高,即使先前显示出可预示侵袭性临床行为的形态特征,非常低。

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