Division of Dermatology, Centre Hospitalier Universitaire Sainte-Justine, University of Montreal, Montreal, Quebec, Canada.
J Am Acad Dermatol. 2010 Dec;63(6):1052-7. doi: 10.1016/j.jaad.2009.12.017.
Vascular tumors and malformations can be challenging to diagnose. Although they may initially appear very similar, they have distinct clinical courses and management. Wilms tumor 1 (WT1) gene expression has been reported in many different tumors including hematologic malignancies and some solid tumors.
We sought to evaluate the expression of WT1 in 126 vascular lesions (64 vascular tumors, one Masson tumor, and 61 vascular malformations).
Based on the International Society for the Study of Vascular Anomalies classification of vascular anomalies, we studied the expression of WT1 in vascular tumors composed of infantile hemangioma, congenital hemangiomas (non-involuting, rapidly involuting, and not otherwise specified), pyogenic granuloma, tufted angioma, cherry angioma, Kaposi sarcoma, and angiosarcoma. We also studied WT1 expression in vascular malformations composed of angiokeratoma/verrucous hemangioma, combined vascular malformations, venous malformations, glomuvenous malformations, lymphatic malformations/lymphangioma, telangiectasia, and targetoid hemosiderotic hemangioma.
All vascular tumors and proliferations had positive WT1 cytoplasmic endothelial immunostaining whereas only 3 vascular malformations were WT1 positive. Moreover the positivity of WT1 in these vascular malformations was focal and involved only re-endothelialized neovessels within thrombi.
The low number of malignant vascular tumors is a limitation.
Immunohistochemical detection of WT1 could be a useful tool to routine evaluation of vascular anomalies allowing the distinction of vascular tumors and proliferations from vascular malformations. Staining for WT1 may guide the clinician in difficult cases, as positive results would suggest a proliferative vascular lesion whereas negative results might point to a vascular malformation.
血管肿瘤和畸形的诊断具有挑战性。虽然它们最初可能看起来非常相似，但它们具有不同的临床过程和管理方法。Wilms 肿瘤 1（WT1）基因表达已在许多不同的肿瘤中报道，包括血液恶性肿瘤和一些实体瘤。
我们试图评估 WT1 在 126 个血管病变（64 个血管肿瘤、1 个 Masson 瘤和 61 个血管畸形）中的表达。
根据国际血管异常研究学会对血管异常的分类，我们研究了 WT1 在由婴儿血管瘤、先天性血管瘤（非消退型、快速消退型和未特指型）、化脓性肉芽肿、丛状血管瘤、樱桃状血管瘤、卡波西肉瘤和血管肉瘤组成的血管肿瘤中的表达。我们还研究了 WT1 在由血管角皮瘤/疣状血管瘤、混合性血管畸形、静脉畸形、静脉动静脉畸形、淋巴管畸形/淋巴管瘤、毛细血管扩张和靶样含铁血黄素性血管瘤组成的血管畸形中的表达。
所有血管肿瘤和增生均有 WT1 细胞质内皮免疫染色阳性，而只有 3 个血管畸形 WT1 阳性。此外，这些血管畸形中的 WT1 阳性是局灶性的，仅涉及血栓内再内皮化的新生血管。
恶性血管肿瘤数量少是一个局限性。
WT1 的免疫组织化学检测可能是常规评估血管异常的有用工具，可区分血管肿瘤和增生与血管畸形。WT1 染色可在困难病例中为临床医生提供指导，阳性结果提示增殖性血管病变，而阴性结果可能提示血管畸形。
