Departments of Pathology, IRCCS "Casa Sollievo della Sofferenza" Hospital, San Giovanni Rotondo (FG), Italy.
Adv Anat Pathol. 2011 Sep;18(5):356-92. doi: 10.1097/PAP.0b013e318229c004.
We reviewed the world literature on solitary fibrous tumors of the central nervous system from August 1996 to July 2011, focusing on both clinicopathological features and diagnostic findings. The anatomical distribution of the 220 cases reported so far reveals that most are intracranial and just over one-fifth are intraspinal. In decreasing frequency, intracranial tumors involve the supratentorial and infratentorial compartments, the pontocerebellar angle, the sellar and parasellar regions, and the cranial nerves. Intraspinal tumors are mainly located in the thoracic and cervical segments. Although most solitary fibrous tumors of the central nervous system are dural based, a small subset presents as subpial, intraparenchymal, intraventricular, or as tumors involving the nerve rootlets with no dural connection. Preoperative imaging and intraoperative findings suggest meningioma, schwannoma or neurofibroma, hemangiopericytoma, or pituitary tumors. Immunohistochemistry is critical to establish a definitive histopathological diagnosis. Vimentin, CD34, BCL2, and CD99 are the most consistently positive markers. The usual histologic type generally behaves in a benign manner if complete removal is achieved. Recurrence is anticipated when resection is subtotal or when the tumor exhibits atypical histology. The proliferative index as assessed by MIB1 labeling is of prognostic significance. Occasionally, tumors featuring conventional morphology may recur, perhaps because of minimal residual disease left behind during surgical extirpation. Rare extracranial metastases and tumor-related deaths are on record. Surgery is the treatment of choice. Stereotactic and external beam radiation therapy may be indicated for postsurgical tumor remnants and for unresectable recurrences. Long-term active surveillance of the patients is mandatory.
我们回顾了 1996 年 8 月至 2011 年 7 月间中枢神经系统孤立性纤维肿瘤的世界文献，重点关注临床病理特征和诊断发现。迄今为止报告的 220 例解剖分布表明，大多数为颅内，超过五分之一为脊髓。颅内肿瘤发病率依次递减，累及幕上和幕下腔、脑桥小脑角、鞍区和鞍旁区以及颅神经。脊髓肿瘤主要位于胸段和颈段。尽管大多数中枢神经系统孤立性纤维肿瘤起源于硬脑膜，但一小部分为软膜下、脑实质内、脑室内或神经根内肿瘤，与硬脑膜无连接。术前影像学和术中发现提示脑膜瘤、神经鞘瘤或神经纤维瘤、血管外皮细胞瘤或无硬脑膜连接的垂体瘤。免疫组织化学对于建立明确的组织病理学诊断至关重要。波形蛋白、CD34、BCL2 和 CD99 是最常阳性的标志物。如果完全切除，通常组织学类型表现为良性行为。如果切除不完全或肿瘤表现为非典型组织学，则预计会复发。增殖指数（通过 MIB1 标记评估）具有预后意义。偶尔，具有常规形态的肿瘤可能会复发，可能是由于手术切除时遗留了少量残留疾病。有记录罕见的颅外转移和肿瘤相关死亡。手术是首选治疗方法。立体定向和外照射放疗可能适用于手术后的肿瘤残余和不可切除的复发病灶。对患者进行长期的积极监测是必要的。
