Zheng Shan, Bi Xin-gang, Song Qing-kun, Yuan Zheng, Guo Lei, Zhang Hongtu, Ma Jian-hui
Department of Pathology, Cancer Institute and Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 17 South Panjiayuan Lane, P.O. Box 2258, Beijing, 100021, China.
Department of Urology, Cancer Institute and Hospital, Chinese Academy of Medical Sciences and Peking Union Medical College, 17 South Panjiayuan Lane, P.O. Box 2258, Beijing, 100021, China.
Int Urol Nephrol. 2015 Oct;47(10):1643-51. doi: 10.1007/s11255-015-1079-9. Epub 2015 Aug 15.
Epithelioid angiomyolipoma (EAML) is a rare entity of the kidney. The guideline for grossing and reporting of renal EAML has not been established for Chinese patients. We planned this study to provide some preliminary indicators for draft guidelines of pathological diagnosis among Chinese people.
The histopathological characteristics of 11 EAML cases from Cancer Hospital, Chinese Academy of Medical Sciences, were reviewed, and a pooled analysis based on our cases and cases from published articles was performed on the histopathological characteristics and prognosis of 56 Chinese patients with EAML. All the cases met the criteria of the 2004 World Health Organization classification of renal tumors.
The ratio of female to male was 1.2:1 with the mean age of 43.4 in the 11 cases. All the 11 cases were sampled following the guideline of renal cell carcinoma. The mean tumor size was 6.5 cm. Four (36.4 %) cases showed necrosis. Six (54.5 %) cases showed invasive borders. Only one case showed metastases. In pooled analysis of the total 56 cases with EAML, 10 cases (17.9 %) showed adverse prognosis. Tumor size, necrosis and invasive edge showed significant difference between favorite and adverse prognostic groups (P < 0.05).
The majority of EAML is benign, and true malignant EAML is rare. The sample of EAML should follow the sample guidelines of renal cell carcinoma with some modifications, emphasizing the presence of necrosis and invading edge. The information of tumor size, necrosis and invasive edge should be included in the diagnostic report of each EAML case.
上皮样血管平滑肌脂肪瘤(EAML)是一种罕见的肾脏肿瘤。目前尚未针对中国患者制定肾脏EAML大体标本取材及报告的指南。我们开展本研究旨在为中国人病理诊断指南草案提供一些初步指标。
回顾了中国医学科学院肿瘤医院11例EAML病例的组织病理学特征,并对我们的病例以及已发表文章中的病例进行汇总分析,研究56例中国EAML患者的组织病理学特征及预后。所有病例均符合2004年世界卫生组织肾脏肿瘤分类标准。
11例患者中女性与男性比例为1.2:1,平均年龄43.4岁。所有11例病例均按照肾细胞癌的取材指南进行取材。肿瘤平均大小为6.5 cm。4例(36.4%)出现坏死。6例(54.5%)有浸润性边界。仅1例出现转移。在对56例EAML患者的汇总分析中,10例(17.9%)预后不良。肿瘤大小、坏死及浸润边缘在预后良好组和预后不良组之间存在显著差异(P<0.05)。
大多数EAML是良性的,真正恶性的EAML很少见。EAML的标本取材应遵循肾细胞癌的取材指南并作一些修改,着重关注坏死及浸润边缘的存在情况。每个EAML病例的诊断报告中应包含肿瘤大小、坏死及浸润边缘的信息。