Department of Pathology, Cleveland Clinic, Ohio 44195, USA.
Arch Pathol Lab Med. 2011 Aug;135(8):1037-41. doi: 10.5858/2010-0668-OAR.
Angiocentric glioma is a rare, epilepsy-associated, low-grade neoplasm with a characteristic perivascular growth pattern.
To describe the clinicopathologic features of 5 angiocentric gliomas and to evaluate for coexistent malformation of cortical development/cortical dysplasia.
Retrospective review of the clinicopathologic features of 5 angiocentric gliomas (3 males and 2 females; median age at surgery, 10 years; range, 3-19 years).
Seizures were the most common presenting symptom (n = 4); 1 patient presented with headaches. Four of the tumors were located in the parieto-occipital, parietal, or temporal cortex and 1 case arose in the thalamus. All tumors consisted of an angiocentric growth pattern of bipolar spindle cells with mild pleomorphism. Three tumors also demonstrated a focal solid growth pattern. Evidence of adjacent malformation of cortical development/focal cortical dysplasia was observed in 4 of 4 cases with sufficient tissue for evaluation; all were Palmini et al type I lesions (type IA, n = 1; type IB, n = 3). All patients were alive at last known follow-up (17-131 months).
The thalamic location of 1 tumor represents an undescribed location for this typically superficial cortical tumor. A subset of angiocentric gliomas, similar to other low-grade chronic epilepsy-related tumors of childhood, are associated with coexistent malformation of cortical development, suggesting a developmental basis to their origin.
血管中心性胶质瘤是一种罕见的、与癫痫相关的低度恶性肿瘤，具有特征性的血管周围生长模式。
描述 5 例血管中心性胶质瘤的临床病理特征，并评估是否存在皮质发育不良/皮质发育不良的共存畸形。
回顾性分析 5 例血管中心性胶质瘤的临床病理特征（3 例男性，2 例女性；手术时的中位年龄为 10 岁；范围为 3-19 岁）。
癫痫是最常见的首发症状（n=4）；1 例患者表现为头痛。4 例肿瘤位于顶枕叶、顶叶或颞叶，1 例发生在丘脑。所有肿瘤均由双极梭形细胞呈血管中心性生长模式，轻度多形性。3 例肿瘤还表现出局灶性实性生长模式。在 4 例有足够组织进行评估的病例中，均观察到邻近皮质发育不良/局灶性皮质发育不良的证据；均为 Palmini 等 I 型病变（IA 型，n=1；IB 型，n=3）。所有患者在最后一次已知随访时均存活（17-131 个月）。
1 例肿瘤位于丘脑，代表了这种通常位于皮质浅层的肿瘤的一个未描述部位。一组血管中心性胶质瘤与其他儿童慢性癫痫相关的低度恶性肿瘤相似，与皮质发育不良并存，提示其起源具有发育基础。
