Sharma Mehar Chand, Ralte Angela Mercy, Gaekwad Shailesh, Santosh Vani, Shankar S K, Sarkar Chitra
Department of Pathology, AIIMS, New Delhi, India.
Pathol Oncol Res. 2004;10(4):219-24. doi: 10.1007/BF03033764. Epub 2004 Dec 27.
Subependymal giant cell astrocytomas (SEGAs) are relatively rare tumors but occur commonly in the setting of the familial syndrome of tuberous sclerosis complex (TSC). In view of its varied morphology, i.e. resemblance to astrocytic and ganglion cells, its histogenesis remains controversial. We studied 23 cases of SEGA, 19 from our own institute and 4 from NIMHANS, Bangalore. These 19 cases of SEGAs were collected over a period of 23 years (1979 to 2001), and accounted for 0.16% of intracranial tumors and 0.51% of all gliomas reported at our center. The majority of patients presented with visual disturbances (19/23, 82.6%) in the form of decreased vision (60.8%) and blindness (21.7%), generalized tonic clonic seizures (43.4%) and focal motor seizures (4.37%). Age ranged from 4 to 37 years (mean 13.2 years) with male predominance (M:F 2.2:1), and the duration of symptoms varied from 1 month to 96 months (mean 17.2 months). Lateral ventricular involvement was the most common site (91.3%), followed by the third ventricle (8.6%). Nine patients (39.1%) had stigmata of tuberous sclerosis (6 at the time of diagnosis and 3 in the follow-up period). Two patients died due to surgical complications, while the rest were alive and well in the follow-up period ranging from 3 to 264 months (mean 37.1 months). Two patients experienced recurrences, one two years and another 22 years after surgery. Microscopic examination showed varied histology consisting of sweeping bundles of spindle cells, gemistocyte and ganglion-like cells with interspersed inflammatory cell component. The inflammatory cell component on special staining turned out to be an admixture of mast cells and T lymphocytes. Six cases showed areas of necrosis and/or mitosis, but were not indicative of aggressive nature of this tumor. Immunoreactivity for GFAP, NF, S-100, NSE and synaptophysin indicates that this is a hybrid tumor with glial and neuronal differentiation. None of the tumors was immunopositive for HMB-45. The significance of the presence of T lymphocytes and mast cells is not clear. It could be related to tumor immunology and may indicate a favorable prognosis.
室管膜下巨细胞星形细胞瘤（SEGA）是相对罕见的肿瘤，但常见于结节性硬化症复合体（TSC）的家族综合征背景下。鉴于其形态多样，即类似于星形细胞和神经节细胞，其组织发生仍存在争议。我们研究了23例SEGA，其中19例来自我们自己的研究所，4例来自班加罗尔的国家精神卫生和神经科学研究所（NIMHANS）。这19例SEGA是在23年（1979年至2001年）期间收集的，占我们中心报告的颅内肿瘤的0.16%和所有胶质瘤的0.51%。大多数患者以视力下降（60.8%）和失明（21.7%）形式出现视觉障碍（19/23，82.6%）、全身性强直阵挛性发作（43.4%）和局灶性运动性发作（4.37%）。年龄范围为4至37岁（平均13.2岁），男性占优势（男:女为2.2:1），症状持续时间从1个月至96个月不等（平均17.2个月）。侧脑室受累是最常见的部位（91.3%），其次是第三脑室（8.6%）。9例患者（39.1%）有结节性硬化症的体征（诊断时6例，随访期间3例）。2例患者死于手术并发症，其余患者在3至264个月（平均37.1个月）的随访期内存活且情况良好。2例患者复发，分别在手术后2年和22年。显微镜检查显示组织学多样，由梭形细胞束、肥胖型星形细胞和神经节样细胞组成，伴有散在的炎症细胞成分。特殊染色显示炎症细胞成分是肥大细胞和T淋巴细胞的混合物。6例显示有坏死和/或有丝分裂区域，但并不表明该肿瘤具有侵袭性。GFAP、NF、S-100、NSE和突触素的免疫反应性表明这是一种具有神经胶质和神经元分化的混合性肿瘤。没有肿瘤对HMB-45呈免疫阳性。T淋巴细胞和肥大细胞存在的意义尚不清楚。这可能与肿瘤免疫学有关，可能预示着良好的预后。
