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Extensively Myxoid and Hyalinized Sinonasal Capillary Hemangiomas: A Clinicopathologic Study of 16 Cases of a Distinctive and Potentially Confusing Hemangioma Variant.

广泛黏液样和透明变的鼻窦毛细血管瘤,一种独特的、可能引起混淆的血管瘤亚型:16例临床病理研究

Abstract

Capillary hemangiomas, the most common vascular tumors of the sinonasal region, are benign endothelial neoplasms, typically growing in an easily recognized lobular pattern. Some sinonasal capillary hemangiomas may show atypical features, such as high cellularity or mitotic activity, and represent more challenging diagnoses. Over the past several years we have seen in consultation a number of examples of sinonasal capillary hemangiomas displaying very striking stromal myxoid change and hyalinization, features that have received scant attention in the past. Available slides from 16 sinonasal capillary hemangiomas previously coded as showing such changes were retrieved from our archives. Submitting diagnoses included "query angiofibroma, rule out malignancy" (N=4), "vascular polyp, rule out malignancy" (N=3), "query malignant vascular tumor" (N=4), "sinonasal hemangiopericytoma" (N=1), and "benign vascular tumor" (N=1). Available radiographic studies often showed worrisome features. Grossly, the tumors ranged from 1.1 to 6.0 cm and appeared as ulcerated, vascular-appearing polyps. Microscopically, the tumors showed striking stromal myxoid change and/or hyalinization, which largely obscured the underlying lobular capillary arrangement. Within this myxohyaline matrix, a florid capillary proliferation was present, frequently with nonatypical mitotic activity. In some instances a branching, "hemangiopericytoma-like" vascular pattern was present in areas. The overall cellularity was low to moderate, and endothelial atypia or hyperchromatism was absent. Ulceration and thrombosis were frequently present. Immunostains to CD31, CD34, and SMA highlighted areas of lobular growth pattern inapparent on the routinely stained slides. Four tested cases were negative for androgen receptors and β-catenin. Follow-up from 12 patients revealed no local recurrences or metastases. Awareness of that sinonasal capillary hemangioma may show these unusual stromal changes, and the use of ancillary immunohistochemistry to highlight its lobular growth pattern should allow its confident distinction from more aggressive endothelial tumors (eg, angiosarcoma) and from nonendothelial tumors, including nasopharyngeal angiofibroma, solitary fibrous tumor, and sinonasal hemangiopericytoma-like tumor.

摘要

毛细血管瘤是鼻窦部最常见的血管肿瘤,是良性的血管内皮肿瘤,通常呈容易识别的分叶状生长模式。部分鼻窦毛细血管瘤可表现出非典型特征,如高细胞密度或高核分裂活性,对诊断造成更大的挑战。过去几年中我们会诊了许多例鼻窦毛细血管瘤,间质显示显著的黏液变和透明变性,这些特征过去并不被重视。从档案资料中检索到16例显示黏液样变和透明变性的鼻窦毛细血管瘤病理切片。临床诊断包括“怀疑血管纤维瘤,需除外恶性(4例)”、“血管性息肉,需除外恶性肿瘤(3例)”、“怀疑恶性血管肿瘤(4例)”、“鼻窦血管外皮瘤(1例)”和“良性血管肿瘤(1例)”。影像学检查常表现为令人担忧的特征。大体检查,肿瘤大小1.1—6.0厘米,表现为溃疡形成的血管性息肉。镜下,肿瘤显示间质显著黏液样变和/或透明变性,很大程度上掩盖了分叶状排列的毛细血管成分。黏液玻变基质中,见毛细血管旺炽性增生,通常无非典型核分裂象。有时部分区域见分支状、血管外皮瘤样血管结构。总体上细胞密度为低到中等,缺乏血管内皮细胞的非典型性或核深染现象。常见溃疡和血栓形成。CD31、CD34和SMA的免疫组化染色能突出显示常规HE染色切片中不明显的毛细血管分叶状生长模式。有4例检测了AR和β-catenin,均阴性。随访了12例,无局部复发或转移。意识到鼻窦毛细血管瘤可以发生这些不寻常的间质改变,并辅助以免疫组化检测,突出显示毛细血管的分叶状生长模式,可以让我们有把握地区别侵袭性血管内皮肿瘤(如 血管肉瘤)和非血管内皮肿瘤,后者包括鼻咽血管纤维瘤、孤立性纤维性肿瘤和鼻窦血管外皮瘤样肿瘤。

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