Mekni A, Kourda J, Chelly I, Ferchichi L, Bellil K, Hammouda K B, Kchir N, Zitouna M, Khaldi M, Haouet S
Department of Pathology, La Rabta Hospital, 1007 Bab-Saadoun, 7, rue Moncef-Bey, Menzeh-7, 2091 Tunis, Tunisia.
Neurochirurgie. 2008 Feb;54(1):15-20. doi: 10.1016/j.neuchi.2007.12.003. Epub 2008 Mar 4.
Most hemangiopericytomas (HPC) are located in the musculoskeletal system and the skin, while the location in the central nervous system (CNS) is rare. The latter represents 2 to 4% in large series of meningeal tumors, thus accounting for less than 1% of all CNS tumors. In the central nervous system, tumors with a hemangiopericytomatous histolopathological pattern can be either hemangiopericytomas or solitary fibrous tumors. CNS-HPCs have a relentless tendency for local recurrence and metastases outside the CNS. Metastasis can also appear many years after adequate treatment of the primary tumor. We present a pathological study of eight patients with CNS-HPC and compare our results with corresponding published data. The CNS-HPC group consisted of three males and five females with a mean age of 36.75 years. The tumors were supratentorial in four cases, infratentorial in two cases, tentorial in one case and located in the spinal cord in the last one. Histologically, CNS-HPCs were similar to their soft tissue counterparts. One case demonstrated increased cellularity, marked nuclear hyperchromasia and marked cellular pleomorphism with infiltration of the cerebellum. All patients underwent surgery with gross-total resection in all cases. No patients received postoperative radiation therapy. Only four patients recurred locally after six, seven and eight months, and five years. Our study presents the pathological features of CNS-HPC as a distinct entity from both meningioma and solitary fibrous tumors. A comparative review of literature with our results is discussed.
大多数血管外皮细胞瘤(HPC)位于肌肉骨骼系统和皮肤,而位于中枢神经系统(CNS)的情况较为罕见。在大量脑膜瘤病例中,后者占2%至4%,因此在所有中枢神经系统肿瘤中占比不到1%。在中枢神经系统中,具有血管外皮瘤组织病理学模式的肿瘤可能是血管外皮细胞瘤或孤立性纤维瘤。中枢神经系统血管外皮细胞瘤有局部复发和中枢神经系统外转移的持续倾向。转移也可能在原发肿瘤得到充分治疗多年后出现。我们对8例中枢神经系统血管外皮细胞瘤患者进行了病理研究,并将我们的结果与相应的已发表数据进行比较。中枢神经系统血管外皮细胞瘤组包括3名男性和5名女性,平均年龄为36.75岁。肿瘤位于幕上4例,幕下2例,小脑幕1例,最后1例位于脊髓。组织学上,中枢神经系统血管外皮细胞瘤与其软组织对应物相似。1例显示细胞增多、明显的核深染和明显的细胞多形性,并侵犯小脑。所有患者均接受了手术,所有病例均实现了大体全切。没有患者接受术后放疗。只有4例患者分别在6个月、7个月、8个月和5年后局部复发。我们的研究展示了中枢神经系统血管外皮细胞瘤作为一种与脑膜瘤和孤立性纤维瘤不同的独特实体的病理特征。并讨论了与我们的结果相关的文献综述。