目的:眼眶原发性恶性孤立性纤维性肿瘤(SFT)是一种罕见的梭形细胞肿瘤,需要切除活检进行组织病理学诊断。我们提出了一个使用当代免疫组织化学染色的临床病例,世界卫生组织最新分类报告,并提供文献综述。
方法:报告一例65岁男性右侧突出,有限的内收,上睑下垂,地球横向位移,脸颊上的花彩。神经成像显示2.2厘米,弥漫性增强的外异质质量。基于核异型性的整体肿瘤切除证实了SFT恶性肿瘤,细胞过多,和增加的有丝分裂活性(13个有丝分裂图/10个高功率场)。Ki-67在良性肿瘤中显示2%的核染色,在恶性对应物中显示10-15%的染色。免疫组织化学分析显示弥漫性Stat6阳性,CD34阳性,恶性部分部分缺乏染色,恶性部分的S-100阳性,而CAM5.2、Desmin、肌动蛋白,CD31和CD117。
结论:免疫分析有助于诊断眼眶恶性孤立性纤维性肿瘤。完全切除肿瘤仍然是首选治疗方法。胸外SFT的行为是不可预测的,所有地方的SFT患者都需要小心,长期随访。
OBJECTIVE: Primary malignant solitary fibrous tumor (SFT) of the orbit is a rare spindle cell neoplasm that requires excisional biopsy for histopathological diagnosis. We present a clinical case using contemporary immunohistochemical stains, report on the latest World Health Organization classification, and provide a review of the literature.
METHODS: Report of a single case of a 65 year old male who presented with right-sided proptosis, limited adduction, ptosis, lateral globe displacement, and cheek festooning. Neuroimaging revealed a 2.2 cm, extraconal heterogeneous mass that diffusely enhanced.En-bloc tumor resection confirmed SFT malignancy based upon nuclear atypia, hypercellularity, and increased mitotic activity (13 mitotic figures/10 high powered fields). Ki-67 showed 2% nuclear staining in the benign tumor and 10-15% staining in the malignant counterpart. Immunohistochemical analysis revealed diffuse Stat6 positivity, CD 34 positivity with partial lack of staining within the malignant portion, S-100 positivity in the malignant portion, and overall negativity for CAM 5.2, desmin, actin, CD 31, and CD 117.
CONCLUSIONS: Immunoprofiling is helpful to making the diagnosis of malignant solitary fibrous tumor of the orbit. Complete tumor resection continues to be the preferred treatment. The behavior of extrathoracic SFT is unpredictable, and patients with SFT in all locations require careful, long-term follow-up.