Br J Ophthalmol. 2014 Jul;98(7):880-8. doi: 10.1136/bjophthalmol-2013-304460.
The purpose of this investigation is to describe the clinical, imaging, histologic and flow dynamic characteristics of orbital cavernous haemangioma.
In this clinicopathologic series, clinical features were obtained from patient records. All imaging studies were reviewed. All specimens were reviewed with haematoxylin and eosin, and 10 were subject to a staining protocol including: Movat Pantachrome, periodic acid Schiff, D2-40, CD31, GLUT-1, Ki-67, vascular endothelial growth factor receptor 1 (VEGF-r1) (flt-1), VEGF-r2 (Flk-1), VEGF, anti-smooth muscle actin (SMA), CD20, CD4, CD8 and CD68. Imaging and pathology were reviewed in a systematic fashion.
Clinically, lesions were more common in middle-aged females presenting with axial proptosis and pain. One-third demonstrated signs of optic nerve dysfunction. Dynamic imaging revealed focal early and diffuse late enhancement. Lesions demonstrated slow growth at 0.2 cm3/year. Histologically, all lesions demonstrated large vascular channels with mature-appearing endothelium and abundant stroma. Three salient features were noted and characterised: thrombosis, nests of perivascular hypercellularity and expanded stromal elements. Acute thrombosis was a feature of each specimen (<10% of channels). Fibrin clots were lined by a layer of CD31+ endothelium. Perivascular hypercellular areas stained uniformly with CD31 and less so with VEGFr2. Additionally, focal areas of Ki67+ and CD68+ cells were found in these regions. Expanded stroma contained CD31+ microcapillary networks and stained diffusely with anti-SMA.
Cavernous haemangioma demonstrate clinical features and growth characteristics of a benign mass. Dynamic imaging highlights their slow flow vascular nature. Histologically, the hypercellularity and stromal changes identified can be understood within the pathogenic context of thrombosis and recanalisation in an organised lesion.
本研究旨在描述眼眶海绵状血管瘤的临床、影像学、组织学和血流动力学特征。
在这个临床病理系列中，我们从患者病历中获得了临床特征。所有影像学研究均进行了回顾。所有标本均进行了苏木精-伊红染色，并对 10 例标本进行了染色方案，包括：Movat 泛染色、过碘酸雪夫染色、D2-40、CD31、GLUT-1、Ki-67、血管内皮生长因子受体 1（VEGF-r1）（flt-1）、VEGF-r2（Flk-1）、VEGF、抗平滑肌肌动蛋白（SMA）、CD20、CD4、CD8 和 CD68。对影像学和病理学进行了系统回顾。
临床上，病变多见于中年女性，表现为轴向突出和疼痛。三分之一的患者有视神经功能障碍的迹象。动态影像学显示局灶性早期和弥漫性晚期强化。病变以 0.2cm3/年的速度缓慢生长。组织学上，所有病变均显示大的血管腔，伴有成熟的内皮细胞和丰富的基质。注意到并描述了三个显著特征：血栓形成、血管周围细胞巢和扩张的基质成分。急性血栓形成是每个标本的特征（<10%的血管）。纤维蛋白血栓由一层 CD31+内皮细胞覆盖。血管周围细胞巢区域 CD31 和 VEGFr2 染色均匀，染色较少。此外，在这些区域还发现了局灶性 Ki67+和 CD68+细胞。扩张的基质含有 CD31+微毛细血管网络，并用抗 SMA 弥漫染色。
海绵状血管瘤具有良性肿块的临床和生长特征。动态影像学突出了其缓慢血流的血管性质。组织学上，在有组织的病变中，血栓形成和再通的发病机制下，可以理解这些区域的细胞增多和基质变化。
