Lavrut Pierre-Marie, Paparel Philippe, Decaussin-Petrucci Myriam
Service d'anatomie et cytologie pathologiques, hospices civils de Lyon, CHU Lyon Sud, université Lyon 1, chemin du Grand-Revoyet, 69495 Pierre-Bénite cedex, France.
Service d'urologie, hospices civils de Lyon, CHU Lyon Sud, université Lyon 1, chemin du Grand-Revoyet, 69495 Pierre-Bénite cedex, France.
Ann Pathol. 2017 Apr;37(2):182-187. doi: 10.1016/j.annpat.2017.01.005. Epub 2017 Mar 17.
Renal epithelioid angiomyolipoma (E-AML) is a rare mesenchymal tumor of the kidney included in the family of tumor with perivascular epithelioid cell differentiation (PEComas) and is frequently associated with tuberous sclerosis complex. Since its clinical and radiological features are not specific, the diagnosis remained mostly pathological. Microscopically, E-AML demonstrate proliferation of more than 80% of epithelioid cells with atypia, often associated with necrosis, hemorrhage, mitotic activity and vascular invasion. Tumor's cells are immunohistochemically positive for melanocytic and smooth muscle markers. The pathologic prognostic predictors are not well known and the malignancy remained based on the identification of distant metastasis. We herein report a case of E-AML diagnosed in a 58-year-old woman and review analysis in the literature to discuss the prognostic indicators of malignancy useful for risk stratification of E-AML and to manage the patients.
肾上皮样血管平滑肌脂肪瘤(E-AML)是一种罕见的肾脏间叶性肿瘤,属于伴有血管周上皮样细胞分化的肿瘤(PEComas)家族,常与结节性硬化症相关。由于其临床和放射学特征不具有特异性,诊断大多仍依赖病理检查。显微镜下,E-AML显示超过80%的上皮样细胞增生且具有异型性,常伴有坏死、出血、有丝分裂活性和血管侵犯。肿瘤细胞免疫组化显示黑素细胞和平滑肌标志物呈阳性。病理预后预测指标尚不明确,恶性程度仍基于远处转移的判定。我们在此报告一例58岁女性诊断为E-AML的病例,并回顾文献分析,以探讨有助于E-AML风险分层和患者管理的恶性预后指标。