Takei Hidehiro, Adesina Adekunle M, Powell Suzanne Z
Department of Pathology, The Methodist Hospital, Houston, Texas 77030, USA.
Neuropathology. 2009 Apr;29(2):181-6. doi: 10.1111/j.1440-1789.2008.00941.x. Epub 2008 Jul 30.
Subependymal giant cell astrocytoma (SEGA) is a benign, slowly growing tumor typically occurring in the setting of tuberous sclerosis complex (TSC). However there are several reported cases in which patients with a solitary SEGA had no other stigmata of TSC. We describe a case of SEGA in a 75-year-old woman representing the oldest patient reported to-date. The patient had a history of radical vulvectomy for malignant melanoma (MM), and died of autopsy-confirmed widespread systemic metastasis. Postmortem examination of the brain revealed a single 2.1 x 1.0 x 0.8 cm intraventricular nodule in the lateral ventricle. Histologically, it was composed of interlacing bundles of spindle-shaped tumor cells with thin delicate processes admixed with relatively large pleomorphic cells with abundant glassy eosinophilic cytoplasm, as seen in a SEGA. Immunohistochemically, GFAP, S-100 protein, and neuron specific enolase were positive, and synaptophysin labeled a few tumor cells. Also noted were rare isolated MM cells within the tumor (i.e., tumor-to-tumor metastasis). Autopsy showed no manifestations of TSC systemically or intracranially. The histopathological differential diagnosis was limited and included giant cell ependymoma and, much less likely, giant cell glioblastoma and pleomorphic xanthoastrocytoma. This case illustrates that SEGA can be found incidentally in an elderly individual with no associated symptoms and also indicates that SEGA can occur outside the setting of TSC. Tumor metastasis to an occult SEGA is extremely rare.
室管膜下巨细胞星形细胞瘤(SEGA)是一种良性、生长缓慢的肿瘤,通常发生在结节性硬化症(TSC)的背景下。然而,有几例报告称,孤立性SEGA患者没有TSC的其他体征。我们描述了一例75岁女性的SEGA病例,该患者是迄今为止报告的年龄最大的患者。该患者有恶性黑色素瘤(MM)根治性外阴切除术史,死于尸检证实的广泛全身转移。死后脑部检查发现侧脑室内有一个2.1×1.0×0.8 cm的单个脑室内结节。组织学上,它由交错的梭形肿瘤细胞束组成,这些细胞具有纤细的突起,并与相对较大的多形性细胞混合,这些细胞具有丰富的玻璃样嗜酸性细胞质,这在SEGA中可见。免疫组化显示,GFAP、S-100蛋白和神经元特异性烯醇化酶呈阳性,突触素标记了少数肿瘤细胞。还注意到肿瘤内有罕见的孤立MM细胞(即肿瘤至肿瘤转移)。尸检显示全身或颅内均无TSC表现。组织病理学鉴别诊断有限,包括巨细胞室管膜瘤,极少见的巨细胞胶质母细胞瘤和多形性黄色星形细胞瘤。该病例说明SEGA可在无相关症状的老年个体中偶然发现,也表明SEGA可在TSC背景之外发生。肿瘤转移至隐匿性SEGA极为罕见。