眶部孤立性纤维瘤：两例临床病理研究并文献复习。
Solitary Fibrous Tumor of the Orbit: A Clinicopathologic Study of Two Cases With Review of the Literature.
机构信息
Department of Ophthalmology, Faculty of Medicine and Graduate School of Medicine, Hokkaido University, Sapporo, Japan.
Department of Ophthalmology, Orbital Disease & Neuro-Ophthalmology Center, Teine Keijinkai Hospital, Sapporo, Japan.
出版信息
In Vivo. 2020 Nov-Dec;34(6):3649-3654. doi: 10.21873/invivo.12211.
BACKGROUND/AIM: Orbital solitary fibrous tumor (SFT) is a rare lesion among orbital tumors, which can be misdiagnosed as another mesenchymal tumor. In this study we report two cases of orbital SFT, focusing on the imaging and pathological findings of the vascular structure inside the tumor.
CASE REPORT
A 26-year-old woman and 43-year-old man presented with orbital SFT. The pathological findings revealed a patternless growth pattern of the tumor cells and hemangiopericytoma-like vascularity as well as thickened, dilated blood vessels within the tumor tissue. Tumor cells revealed a diffuse strong positivity for cluster of differentiation 34 (CD34) and signal transducer and activator of transcription 6 (STAT6) in both cases, while B-cell lymphoma 2 (bcl-2) and CD99 were positive in one case. Characteristic findings within the tumor were the arterial components, where a variety of STAT6, CD99 and bcl-2-positive smooth muscle cells were intermingled.
CONCLUSION
Histologically, the tumor tissues might be characterized by not only conventional hemangiopericytoma-like vasculature but also dilated arterial vessels, which were shown to be part of the tumor components.
背景/目的：眼眶孤立性纤维肿瘤（SFT）是眼眶肿瘤中一种罕见的病变，易误诊为另一种间叶性肿瘤。本研究报告了两例眼眶 SFT，重点关注肿瘤内血管结构的影像学和病理学表现。
病例报告
一名 26 岁女性和一名 43 岁男性均表现为眼眶 SFT。病理检查显示肿瘤细胞呈无模式生长模式，伴有血管外皮细胞瘤样血管生成以及肿瘤组织内增厚、扩张的血管。两例均可见肿瘤细胞弥漫强表达分化簇 34（CD34）和信号转导子和转录激活子 6（STAT6），而 bcl-2 和 CD99 在一例中阳性。肿瘤内的特征性表现为动脉成分，其中多种 STAT6、CD99 和 bcl-2 阳性的平滑肌细胞相互交织。
结论
组织学上，肿瘤组织的特点不仅是常规的血管外皮细胞瘤样血管，还有扩张的动脉血管，这些血管被认为是肿瘤成分的一部分。
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