- Virchows Archiv 多中心研究（21 机构）：51 例肾脏嗜酸性空泡状肿瘤（EVT）对照 149 例鉴别诊断相关嗜酸性肾肿瘤，评估 CD56 免疫组化。
- CD56 在 49/51（98.0%）EVT 阳性，3+（>50% 细胞）最常见（85.7%）且特异性最高（89.1%）；但嗜酸细胞腺瘤 33.3%、FLCN 突变型 25.0%、ESC-RCC 5.0% 也可 3+。
- 低级别嗜酸细胞肿瘤、上皮样 AML、浸润性尿路上皮癌、FH 缺失型 RCC 均阴性；EVT 普遍 KIT/CD10 阳性，常伴 TSC1/TSC2/MTOR 变异。
- 实践提示：考虑 EVT 时 CD56 可作辅助标志，但需结合形态与其他 IHC/分子，不能单靠 3+ 定诊。原文付费墙，本站仅获摘要。
收录范围：PubMed 摘要中英对照。原文为订阅/付费墙内容，本站未获全文与图表。
摘要
嗜酸性空泡状肿瘤（eosinophilic vacuolated tumor，EVT）是一种新兴的肾脏肿瘤实体，因其与其他嗜酸性肾肿瘤在形态、免疫组化及分子层面存在重叠而诊断困难。本研究评估 CD56 免疫组化在 EVT 诊断中的价值。自 21 家机构汇总 51 例 EVT，并纳入鉴别诊断相关肿瘤（n = 149）。复核临床病理特征及诊断时已做的免疫组化/分子检测。对每例肿瘤一张代表性切片行 CD56 全片免疫染色，评分：0（≤ 2% 肿瘤细胞）、1+（2–10%）、2+（10–50%）、3+（> 50%）。EVT 队列（男∶女 = 1∶1.4，中位年龄 = 50 岁）含活检 7 例、肾部分切除 31 例、根治性肾切除 13 例。49/51（98.0%）EVT 表达 CD56，其中 3+ 最常见（n = 42/49；85.7%）且特异性最高（89.1%）。但嗜酸细胞腺瘤 12/36（33.3%）、FLCN 突变型肾肿瘤 2/8（25.0%）及嗜酸性实性囊性肾细胞癌（ESC-RCC）1/20（5.0%）亦可呈 CD56 3+。偶见 1+/2+：嗜酸性嫌色细胞肾细胞癌 4/28（14.3%）、嗜酸性透明细胞肾细胞癌 1/9（11.1%）、SDH 缺失型肾细胞癌 1/10（10.0%）。低级别嗜酸细胞肿瘤（n = 23）、上皮样血管平滑肌脂肪瘤（n = 7）、浸润性尿路上皮癌（n = 5）及 FH 缺失型肾细胞癌（n = 3）均 CD56 阴性。EVT 中 KIT、CD10、cathepsin K、GPNMB 阳性率分别为 51/51、41/41、36/48（75.0%）、6/6。17/17 例 EVT 检出体细胞 TSC1/TSC2/MTOR 变异（含 1 例 GPNMB 阳性）；5 例未做 DNA 测序的 GPNMB 阳性 EVT 中有 2 例伴已知胚系 TSC1 突变。当鉴别诊断考虑 EVT 时，CD56 是评估嗜酸性肾肿瘤有用的辅助免疫组化标志物。
- Virchows Archiv multi-institutional study (21 centres): 51 renal eosinophilic vacuolated tumours (EVT) vs 149 differential eosinophilic renal tumours, testing CD56 IHC.
- CD56 positive in 49/51 (98.0%) EVTs; 3+ (>50% cells) most frequent (85.7%) and most specific (89.1%), but also seen in 33.3% oncocytomas, 25.0% FLCN-mutated tumours and 5.0% ESC-RCC.
- LOT, epithelioid AML, invasive urothelial carcinoma and FH-deficient RCC were all CD56-negative; EVTs were uniformly KIT/CD10 positive and harboured TSC1/TSC2/MTOR variants when sequenced.
- Practice point: CD56 helps when EVT is in the differential, but 3+ alone is not diagnostic. Subscription article; abstract only on this site.
Scope: the PubMed abstract. The article is subscription/paywalled, so the full text and figures were not obtained.
Abstract
Eosinophilic vacuolated tumor (EVT) is an emerging kidney entity that can pose diagnostic challenges because of the morphologic, immunohistochemical and molecular overlap with other eosinophilic renal tumors. Herein, we investigated the diagnostic utility of CD56 immunohistochemistry in EVTs. A total of 51 EVTs were collated from 21 institutions along with differential diagnostic tumors (n = 149). Clinicopathologic features were reviewed, including immunohistochemistry/molecular testing performed at diagnosis. Whole-slide immunostaining for CD56 (one representative section per tumor) was performed and scored as follows: 0 (≤ 2% tumor cells), 1+ (2-10%), 2+ (10-50%), 3+ (> 50%). The EVT cohort (M:F = 1:1.4, median age = 50 years) comprised 7 biopsies, 31 partial nephrectomies, and 13 radical nephrectomies. CD56 was expressed in 49/51 (98.0%) EVTs, with the 3 + pattern as the most frequent (n = 42/49; 85.7%) and most specific (89.1%). However, CD56 3 + expression was also found in 12/36 (33.3%) oncocytomas, 2/8 (25.0%) FLCN-mutated renal tumors, and 1/20 (5.0%) eosinophilic solid and cystic renal cell carcinoma (RCC). CD56 is occasionally expressed, with a 1+/2 + pattern, in eosinophilic chromophobe RCC (n = 4/28, 14.3%), eosinophilic clear cell RCC (n = 1/9, 11.1%), and SDH-deficient RCC (n = 1/10, 10.0%). Low grade oncocytic tumor (n = 23), epithelioid angiomyolipoma (n = 7), invasive urothelial carcinoma (n = 5), and FH-deficient RCC (n = 3) were negative for CD56. KIT, CD10, cathepsin K, and GPNMB were positive in 51/51, 41/41, 36/48 (75.0%), and 6/6 EVTs, respectively. Somatic TSC1/TSC2/MTOR variant(s) were detected in 17/17 EVTs, including one GPNMB-positive tumor. Two of five GPNMB-positive EVTs that have not undergone DNA sequencing were associated with known germline TSC1 mutation. CD56 is a useful adjunct immunohistochemical marker in the assessment of eosinophilic renal tumors when EVT is a diagnostic consideration.