CD30 expression in malignant vascular tumors and its diagnostic and clinical implications: a study of 146 cases.
CD30 expression in malignant vascular tumors and its diagnostic and clinical implications: a study of 146 cases.
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DOI：
10.1097/pai.0000000000000048
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发表时间：
2014-05
期刊：
影响因子：
--
通讯作者：
Miettinen M
中科院分区：
文献类型：
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作者：
Alimchandani M;Wang ZF;Miettinen M
血管肉瘤（AS）是一种罕见的恶性血管肿瘤，而上皮样血管内皮瘤（EHE）是一种低级别恶性血管肿瘤。CD30是肿瘤坏死因子受体超家族成员8 （TNFRSF8）的一员。虽然CD30的表达最常与淋巴细胞恶性肿瘤或生殖细胞肿瘤相关，但偶尔也有报道称血管肉瘤呈CD30阳性。然而，有有限的数据明确评价其在恶性血管肿瘤中的作用。在这项研究中，我们评估了91例血管肉瘤（AS）， 30例不同部位的上皮样血管内皮瘤（EHE）和25例卡波西肉瘤（KS）。总的来说，CD30在31/91例AS（34%）和7/30例EHE（30%）中表达，而在KS中没有表达。CD30呈膜状表达，肿瘤细胞从局灶性到弥漫性呈阳性表达。阳性血管肉瘤包括血管形成的高分化肿瘤，也包括实体的、未分化的淋巴瘤样病例，其中一例在免疫组织化学时代之前被归类为淋巴瘤。cd30在大多数血管肉瘤中50%的肿瘤细胞中表达，但仅在7%的EHEs中表达。研究的55例血管肉瘤中，TIA-1或颗粒酶B免疫组化均未呈阳性，这些抗原被用作间变性大细胞淋巴瘤的特异性标记。与血管肉瘤相比，正常毛细血管和肌肉血管内皮呈不同程度的阳性。在血管瘤中，海绵状和梭形细胞血管瘤最常见内皮细胞cd30阳性，而在大多数其他血管瘤中，cd30阳性较少。总之，CD30表达发生在血管肉瘤和EHE的重要亚群中，需要与其他CD30阳性恶性肿瘤进行鉴别诊断，以避免诊断陷阱。目前尚不清楚强烈CD30阳性血管肉瘤患者是否可以使用最近引入的CD30抗体药物偶联物进行靶向治疗。
Angiosarcoma (AS) is a rare malignant vascular tumor while epithelioid hemangioendothelioma (EHE) is a vascular tumor of low grade malignancy. CD30 is a member of the tumor necrosis factor receptor superfamily, member 8 (TNFRSF8). While expression of CD30 is most commonly associated with lymphoid malignancies or germ cell tumors, occasional angiosarcomas have been reported as CD30-positive. However, there is limited data to evaluate its role definitively in malignant vascular tumors. In this study, we evaluated 91 angiosarcomas (AS), 30 epithelioid hemangioendothelioma (EHE) from various sites, and 25 Kaposi sarcomas (KS). Overall, CD30 was expressed in 31/91 cases (34%) of AS, in 7/30 cases (30%) of EHE but in none of the KS. CD30 was expressed in a membranous staining pattern and positivity in tumor cells varied from focal to diffuse. The positive angiosarcomas included vasoformative more differentiated tumors and also solid, undifferentiated, lymphoma-like examples, one of which was classified as lymphoma prior to the era of immunohistochemistry. The CD30-expression was seen in >50% of tumor cells in a majority of angiosarcomas but only in 7% of EHEs. None of the 55 angiosarcomas studied were immunohistochemically positive for TIA-1 or granzyme B, antigens used as more specific markers for anaplastic large cell lymphoma. Compared with angiosarcoma, normal vascular endothelia of capillaries and muscular vessels showed variable positivity. Among hemangiomas, cavernous and spindle cell hemangiomas showed most frequent endothelial CD30-positivity, whereas in most other hemangiomas, CD30-positivity was scant. In conclusion, CD30 expression occurs in a significant subset of angiosarcomas and EHE and needs to be included in the differential diagnosis with other CD30-positive malignancies to avoid a diagnostic pitfall. It remains to be determined whether patients with strongly CD30-positive angiosarcomas could be candidates for targeted therapy using the recently introduced CD30 antibody drug conjugates.