Department of Anatomical Pathology, PathWest Laboratory Medicine, QEII Medical Centre, Hospital Ave, Nedlands, Western Australia 6009, Australia.
Hum Pathol. 2011 Apr;42(4):489-99. doi: 10.1016/j.humpath.2010.08.010. Epub 2011 Jan 14.
Oncocytic adrenocortical neoplasms (OANs) are a rare but important subtype of adrenal tumors with unique clinical and morphological features. We present 13 previously unpublished cases, of which 3 were classified as benign, 2 as having borderline malignant potential, and 8 as malignant according to the Lin-Weiss-Bisceglia criteria. Seven tumors (54%) showed evidence of endocrine activity. All were composed of more than 90% oncocytes confirmed immunohistochemically using the antimitochondrial antibody mES-13 and ultrastructurally in 4 cases. Small oncocytes were a frequent finding that challenges the conventional notion of oncocytes as necessarily having abundant cytoplasm. Most cases were immunoreactive for vimentin, synaptophysin, inhibin-α, melan A, and calretinin, the latter being a novel finding in this group of neoplasms. Cytokeratin positivity with AE1/AE3 and CAM5.2 was variable. The literature was comprehensively reviewed to identify all cases of OANs reported to date. Hormone production is not as uncommon as previously believed, occurring in 30%. The Lin-Weiss-Bisceglia criteria were retrospectively applied to all published cases with sufficient information and were shown to effectively separate tumors according to their future risk of recurrence and survival using Kaplan-Meier survival curves (log-rank test, P < .001 for both). The estimated overall median survival for malignant oncocytic neoplasms is 58 months (95% confidence interval = 27.5-88.5 months), providing the first preliminary evidence that the prognosis of malignant OANs is likely to be more favorable than conventional adrenocortical carcinomas, in which the reported median survival is between 14 and 32 months.
嗜酸细胞瘤性肾上腺皮质肿瘤（OANs）是一种罕见但重要的肾上腺肿瘤亚型，具有独特的临床和形态学特征。我们报告了 13 例以前未发表的病例，其中 3 例被归类为良性，2 例具有交界恶性潜能，8 例根据 Lin-Weiss-Bisceglia 标准为恶性。7 例肿瘤（54%）显示内分泌活性的证据。所有肿瘤均由超过 90%的嗜酸细胞瘤组成，这些肿瘤通过使用抗线粒体抗体 mES-13 和 4 例肿瘤的超微结构免疫组织化学方法得到证实。小嗜酸细胞瘤是一种常见的发现，这挑战了嗜酸细胞瘤必然具有丰富细胞质的传统观念。大多数病例对波形蛋白、突触素、抑制素-α、黑素 A 和钙视网膜蛋白呈免疫反应性，后者是这组肿瘤的新发现。角蛋白阳性，AE1/AE3 和 CAM5.2 具有变异性。全面回顾了文献，以确定迄今为止报告的所有 OAN 病例。激素产生并不像以前认为的那样罕见，发生率为 30%。回顾性地将 Lin-Weiss-Bisceglia 标准应用于所有有足够信息的已发表病例，并使用 Kaplan-Meier 生存曲线（对数秩检验，P<0.001）有效地根据肿瘤的复发和生存风险将肿瘤分开。恶性嗜酸细胞瘤的总体估计中位生存时间为 58 个月（95%置信区间=27.5-88.5 个月），这首次提供了初步证据，表明恶性 OAN 的预后可能比传统的肾上腺皮质癌更有利，后者的报告中位生存时间为 14 至 32 个月。
