Solitary subependymal giant cell astrocytoma incidentally found at autopsy in an elderly woman without tuberous sclerosis complex
Department of Pathology, The Methodist Hospital, Houston, Texas 77030, USA.Neuropathology (Impact Factor: 1.65). 08/2008; 29(2):181-6. DOI: 10.1111/j.1440-1789.2008.00941.x
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.
Chapter: Pathogenesis of TSC in the Brain[Show abstract] [Hide abstract]
ABSTRACT: IntroductionTubersSENs and SEGAsCell LineagemTOR Activation and Biallelic TSC Gene InactivationAlternative Signaling Cascades in TSC Brain LesionsStructural Alterations in Nontuber Brain AreasConclusions and Future DirectionsReferences
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ABSTRACT: The authors report their experience of using Gamma Knife surgery (GKS) in patients with subependymal giant cell astrocytoma (SEGA). Over a 20-year period, the authors identified 6 patients with SEGAs who were eligible for GKS. The median patient age was 16.5 years (range 7-55 years). In 4 patients, GKS was used as a primary management therapy. One patient underwent radiosurgery for recurrent tumors after prior resection, and in 1 patient GKS was used as an adjunct after subtotal resection. The median tumor volume at GKS was 2.75 cm(3) (range 0.7-5.9 cm(3)). A median radiation dose of 14 Gy (range 11-20 Gy) was delivered to the tumor margin. The median follow-up duration was 73 months (range 42-90 months). Overall local tumor control was achieved in 4 tumors (67%) with progression-free periods of 24, 42, 57, and 66 months. Three tumors regressed and one remained unchanged. In 2 patients the tumors progressed, and in 1 of these patients the lesion was managed by repeated GKS with subsequent tumor regression. The other relatively large tumor (5.9 cm(3)) was excised 9 months after GKS. The progression-free period for all GKS-managed tumors varied from 9 to 66 months. There were no cases of hydrocephalus or GKS-related morbidity. Gamma Knife surgery may be an additional minimally invasive management option for SEGA in a patient who harbors a small but progressively enlarging tumor when complete resection is not safely achievable. It may also benefit patients with a residual or recurrent tumor that has progressed after surgery.
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ABSTRACT: Subependymal giant cell astrocytoma (SEGA) is a rare, benign brain tumour developing in patients with tuberous sclerosis complex (TSC). Typical histopathological findings of this neoplasm are solid sheets and perivascular pseudorosettes of large, gemistocytic, polygonal and occasionally ganglion-like cells within a fibrillated background, accompanied by spindle-shaped cells creating broad fascicles. Rich vascular stroma, and numerous calcifications are common. Mitoses, focal necrosis, and endothelial proliferation are rarely encountered. In this study we report 3 cases of SEGA out of 29 TSC patients, operated on in the Department of Neurosurgery, Children's Memorial Health Institute, from 1990 to 2011 and retrospectively reviewed. These 3 cases exhibited distinct anaplastic features that might mimic malignant glioma. Histologically, the tumours were composed of pleomorphic, gemistocytic, polygonal, ganglion-like or multinucleated cells arranged in sheets or forming perivascular pseudorosettes. Numerous foci of necrosis, microvascular proliferation and high mitotic activity with atypical mito-tic figures were documented. The Ki67 labelling index was about 15-20%. These tumours might be confused with high-grade gliomas and such a misleading diagnosis might result in aggressive radio- or chemotherapy. Despite the common statement that morphological features of anaplasia, i.e. pleomorphism, necrosis, microvascular proliferation and increased mitotic activity, are not of prognostic value, the tumour behaviour in our cases seems to be more aggressive and requires longer follow-up studies. We suggest recognizing such cases of subependymal giant cell astrocytoma as atypical SEGAs.
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