目的：探讨原发于颅内的孤立性纤维性肿瘤（Intracranial solitary fibrous tumor，ISFT）的影像学特征及病理学对照分析。方法：回顾性分析2005年1月—2015年12月我院收治的8例ISFT患者，所有病灶均经术后病理证实。行头CT平扫6例，其中4例行增强检查，全部8例均行头MR平扫及增强检查。结果：临床主要症状为头痛（5/8）。所有病例均为单发病灶，大部分位于幕上（6/8），且与脑膜关系密切。肿瘤长径3～7 cm不等（平均4.5 cm），分叶状、类圆形或不规则形。CT示肿瘤以囊实混杂为主（4/6），不均匀强化者可见迂曲血管影。MR示肿瘤边界清晰，多数瘤周无或轻度水肿（5/8）。T1WI以等信号为主，T2WI信号混杂，部分病灶（5/8）高低信号分界清晰。囊实混杂者明显不均匀强化，部分病灶（4/8）T2WI低信号区可见强化。病理示瘤细胞以梭形为主，排列方式多变，细胞密集区与疏松区交替出现，间质富含血管，可见胶原、黏液样变。免疫组化示Vimentin弥漫阳性（8/8），CD34阳性率87.5%（7/8），Bcl-2阳性率75%（6/8），Ki-67 5%～20%不等。病理诊断良性4例，低度恶性2例，交界性2例。结论：ISFT通常为单发肿块，边界清晰，幕上多见，与脑膜关系密切，多以囊实混杂为主。T2WI可特征性表现为“阴阳征”，增强扫描明显不均匀强化，T2WI低信号区可见强化，仅依靠影像学诊断准确率不高，需结合病理及免疫组化。此外，部分良性病灶可恶变、复发，故应定期随访。
Abstract
Objective: To investigate the imaging and pathological characteristics of primary intracranial solitary fibrous tumor(ISFT). Methods: Eight patients with primary ISFT received operation in our hospital from January 2005 to December 2015. All of the tumors were confirmed by pathology. Six cases received CT scan(4 with enhancement). All the cases underwent gadolinium-enhanced MR. Results: The main clinical symptom was headache(5/8). All the 8 tumors were single, and most origined from supratentorial(6/8), close to the meninges. Diameter of the tumors ranged from 3～7 cm(4.5 cm on average). And the tumors are lobulated, round or irregular in shape. CT scan showed the tumors were mainly mixed cystic and solid(4/6). Varicose veins were visible within tumors with heterogeneous enhancement. MR scan showed clear margin, with or without mild peritumoral edema in most cases(5/8). T1WI showed the signal was mainly isointense to the white matter. T2WI showed the signal of tumors was heterogeneous, with clear boundary between high and low signal(5/8). Contrast-enhanced T1WI showed marked heterogeneous enhancement, and some of the foci of low T2WI signal intensities showed enhancement(4/8). Pathological results showed the tumor cells were mainly spindle shaped, with varying arrangement, and the area with densely or loosely arranged cells appeared alternately. Blood vessels were rich in the stroma, and collagen and myxoid were visible. Immunohistochemical results showed Vimentin was diffusely positive(8/8). CD34 was positive in 7 tumors, Bcl-2 in 6 and Ki-67 ranged 5%～20%. Pathology showed 4 were benign, 2 were low-grade malignant and 2 were borderline. Conclusion: ISFT is solitary, clearly marginal, supratentorial origined, and closely related to the meninges. Most of them are mixed cystic and solid, with significantly heterogeneous enhancement. The “yin and yang” sign and enhancement of low signal area in T2WI are characteristic. The diagnosis of ISFT should not only depend on the imaging. The combination of imaging, pathology and immunohistochemistry is necessary. In addition, some benign tumors may relapse, so it should be followed up regularly.
关键词
/
神经纤维瘤 /
病理学 /
体层摄影术 /
X线计算机 /
磁共振成像
Key words
Neurofibroma /
Pathology /
Tomography, X-ray computed /
Magnetic resonance imaging
中图分类号：
Ｒ739.41
Ｒ730.264
Ｒ814.42
Ｒ445.2
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