Subependymal giant-cell astrocytoma. Here, they may block the flow of fluid between the brain … They invariably occur in the setting of tuberous sclerosis and affect the region near the foramen of Monro, potentially obstructing the flow of CSF and causing hydrocephalus. However, recurrence and progression of pilocytic astrocytomas in adults has been reported (Stüer et al 2007). Central nervous system (CNS) primitive neuroectodermal tumor (cPNET) is retained in the 2007 classification. Have a question? MAHLON D. JOHNSON, JAMES B. ATKINSON, in Modern Surgical Pathology (Second Edition), 2009. Circumscribed astrocytic tumors (pleomorphic xanthoastrocytoma, subependymal giant cell astrocytoma, pilocytic astrocytoma) have well-defined margins, are benign, and are typically managed with surgery alone. They are a common cause of mortality and morbidity in both the young and old. resemblance to astrocytic and ganglion cells, its histogenesis remains controversial. Fornix damage from solitary subependymal giant cell astrocytoma causing postoperative amnesic syndrome. The latter is an aggressive neoplasm in which discrete islands of neuropil-like material, staining strongly for synaptophysin are present within an otherwise typical anaplastic astrocytoma or glioblastoma (Teo et al 1999). Mitotic figures and necrosis are uncommon, but when they are noted, they do not constitute a high-grade diagnosis or suggest more aggressive behavior. National Library of Medicine … In addition to identifying this tumor in its typical location near the foramen of Monro, the identification of stigmata related to tuberous sclerosis in the same patient confirms the diagnosis of SGA (see Figure 20).83–88 SGA is one of the few brain tumors that may be identified at birth and should therefore be included in the differential diagnosis of neonatal brain tumors when appropriate. Extension into the third ventricle is uncommonly seen. Moreover, it is uncommon to note dissemination of these tumors into the CSF space. Daumas-Duport and colleagues proposed a two-tier grading scheme based on histopathological and imaging features: grade A with no endothelial cell hyperplasia and no contrast enhancement; grade B with either endothelial cell hyperplasia or contrast enhancement (Daumas-Duport et al 1997). The histopathological diagnosis of anaplastic ependymoma is appropriate where there are appreciable numbers of mitotic figures, vascular endothelial cell hyperplasia and/or necrosis. Both gangliocytoma and ganglioglioma are graded as WHO I. Anaplasia in anaplastic ganglioglioma (WHO III), refers to features in the glial component that are commonly seen in anaplastic astrocytoma and glioblastoma multiforme, i.e., increased mitoses, vascular endothelial proliferation, necrosis and elevated proliferation indices. Co-deletion is seen in up to 80% of oligodendrogliomas (Jeuken et al 2004; Gonzales et al 2006) but is less common in oligoastrocytomas. Subependymal giant cell astrocytoma. Necrosis in anaplastic oligoastrocytoma however, is associated with a significantly reduced survival (Miller et al 2006). The flap is based on the coronal suture, with the medial border off the midline and the anterior border at least 2 cm anterior to the coronal suture and the posterior border about 2 cm behind. The majority have a non-aggressive course following either complete or incomplete surgical resection and are accorded a grading of WHO I. Pleomorphic xanthoastrocytoma may rarely form the glial component of a ganglioglioma (Kordek et al 1995) and co-existence of PXA and ganglioglioma as separate composite tumors has also been reported (Perry et al 1997a). Occasional tumor cells are atypical and binucleate and these unusual features can give the mistaken impression of anaplasia. In one series of 47 pathologically proven lateral ventricular neoplasms, Jelinek et al (1990) found that the clinical characteristics most consistent with SGCA include presentation in the first three decades of life, location at the foramen of Monro, and tumor enhancement with contrast on CT scan. Surgical resection is associated with complications, however, and does not always result in symptomatic relief.43,44 Everolimus, an agent that targets the upregulated mTOR pathway that drives SEGA growth, has been used successfully to treat SEGAs.45 A recent study demonstrated that more than 60% of patients had significantly reduced tumor volumes relative to baseline after 60 months of treatment.46, S. Ramkissoon, in Pathobiology of Human Disease, 2014. rare disease research! Lesions within the third ventricle may be accessed from the transcortical approach. The tumor cells exhibit a wide spectrum of cytoarchitectures: small elongated cells in a variably fibrillated matrix, intermediate size polygonal cells, and variable numbers of giant, ganglion-like cells. In general, cortical tubers are more readily apparent on MRI (see Figure 20), whereas calcified subependymal nodules are more readily identified on CT (see Figure 19).83,84,87 The extent of brain involvement with cortical tubers has been shown to correlate with the severity of disease in these patients.83,86,87 Patients with tuberous sclerosis probably benefit from annual surveillance for these tumors during childhood. The patient is placed in the supine position with the head elevated 10 to 30 degrees. The subependymal giant cell astrocytomas (SEGA), almost always arise during the first two decades in association with the tuberous sclerosis complex (Lopes et al 2007). The tumor arises most commonly in the lateral ventricle near the foramen of Monro. Subependymal giant cell astrocytoma (SEGA) is a type of brain tumor that can develop in patients with tuberous sclerosis complex (TSC). Subependymal giant cell astrocytoma (SEGA) is a World Health Organization grade I tumor of glioneuronal origin, which is most commonly located at the caudothalamic groove adjacent to the foramen of Monro. Astrocytoma originates in astrocytes, which are a kind of glial cells in the cerebrum which are star-shaped. The prevalence rate of TSC in patients with SEGA ranges from 5% to 20%. PRKAR1A (17q24) found in this syndrome. When first reported (Kepes et al 1979), PXAs appeared to behave in a non-aggressive manner. Patients with SEGAs usually present clinically between ages 2 and 30 years, but the tumors occur most frequent in the early teen years, with a mean age at presentation of 13 years. The clinical setting, age of onset, and site of the tumor, together with the characteristic giant cells expressing GFAP and neurofilament proteins, can make this important distinction. Papillary glioneuronal tumor is one of two new entities included in the neuronal and mixed neuronal-glial tumor category. Immunohistochemically, their primary astrocytic nature is confirmed by moderate GFAP and S-100 protein immunoreactivity. The differential diagnosis for tumors of the lateral ventricle in addition to SGCA includes ependymoma, subependymoma, primary cerebral neuroblastoma, astrocytoma, oligodendroglioma, meningioma, CN, and choroid plexus papilloma. TSC is an autosomal dominantly in- herited neurocutaneous syndrome that affects any organ sys- tem of the body. The two-tier WHO scheme for grading oligodendroglial tumors contrasts with previous schemes proposing four grades (Smith et al 1983; Mörk et al 1986). The latter is composed of large epithelioid cells with prominent nucleoli and exhibits abundant mitotic figures and apoptotic debris as well as areas of necrosis (Giangaspero et al 1992; Verma et al 2008). 7.7C).31,32 Most characteristic is the compact arrangement of large, astrocyte-like cells with abundant glassy cytoplasm, combined with large round-to-ovoid vesicular nuclei and prominent nucleoli similar to those of ganglion cells (Fig. Co-deletion is predictive of responsiveness to alkylating chemotherapeutic agents (Cairncross et al 1998) as well as prolonged recurrence free survival (Cairncross et al 1998; Ino et al 2000, 2001). Subependymal hamartomas are mostly asymptomatic. These tumors predominate in young children with few cPNETs having been described in adults (Ohba et al 2008). The most common extracranial manifestations include facial cutaneous angiofibromas, renal angiomyolipoma, pulmonary lymphangioleiomyomatosis, subungual fibroma, cardiac rhabdomyoma, intestinal polyps, and visceral cysts.26, By neuroimaging, these tumors are solitary and circumscribed within the lateral ventricles, ranging in size from under 1 cm to over 6 cm (Fig. Subependymal astrocytoma Subependymal giant cell astrocytoma (+) Supependymoma (+) Teratoid medulloepithelioma Teratoma, benign (O) Teratoma (+) Transitional meningioma (O) Tumor cells, benign (O) Tumor cells, malignant Venous hemangioma (O) Rosette-forming glioneuronal tumor of the fourth ventricle is the second new entity in the neuronal and mixed neuronal-glial tumor category with a grading of WHO I. 7.7F), and present within the ventricle rather than the parenchyma. These tumors occur almost exclusively in children under 3 years of age. Tumor cells have morphological features similar to pinocytes and are arranged in rosettes as well as diffuse sheets. Mutations of the TP53 gene, occurring early in tumor evolution, are the hallmark of secondary glioblastoma, while amplification and re-arrangement of the epidermal growth factor receptor (EGFR) gene are characteristic of primary glioblastoma (Ohgaki et al 2004; Ohgaki & Kleihues 2007). Where there is predominant or exclusive neuronal differentiation without formation of mature ganglion cells, the term CNS neuroblastoma is appropriate, whereas CNS ganglioneuroblastoma contains mature ganglion cells in addition to features of neuroblastoma. In view of its varied morphology, i.e. When first reported, this neoplasm was regarded as a dysembryoplastic neuroepithelial tumor involving the cerebellum (Kuchelmeister et al 1995). It characteristically grows inside the ventricles, which are fluid-filled spaces deep into the brain, and can often block the normal outflow of this fluid, thus causing hydrocephalus. Check the full list of possible causes and conditions now! Once the lesion has been removed, the surrounding ventricle surfaces are inspected to ensure that any adherent tumor is resected. Several histologic sub-types of medulloblastoma are recognized. Two commonly affected genes underlying TSC and therefore SEGAs are TSC1 and TSC2, which encode for the proteins hamartin and tuberin, respectively. Note that subependymal nodules (white arrows) are more conspicuous on the CT examination than on the MRI. Sub-ependymal giant cell astrocytomas occur almost exclusively in patients with tuberous sclerosis complex (Ahlsén et al 1994; Ess et al 2005). Anaplastic astrocytoma (WHO grade III) and glioblastoma multiforme (WHO grade IV) are distinguished from diffuse astrocytoma by their denser hypercellularity, greater nuclear and cellular pleomorphism, greater numbers of mitotic figures, endothelial cell proliferation, and necrosis. However, molecular-genetic studies have indicated clear differences between cerebellar liponeurocytoma and medulloblastoma (Horstmann et al 2004). Intrinsic CT features include the presence of calcifications and a hyperdense appearance relative to cortex (see Figure 19). The amnesic syndrome is characterized by difficulty learning and recalling information, either previously encoded (retro-grade) or … Ultrastructurally, individual cells of SEGA show a combination of astrocytic and neuronal features, the latter including microtubules, occasional dense core granules and/or secretory vesicles, and even synapses. Tumors of the frontal horn can become very large and cause obstruction of the foramen of Monro with ventricular dilation. Among pulse sequences, FLAIR MRI displays cortical tubers (arrowheads) most effectively. The three entities in the choroid plexus tumor category represent a spectrum from benign to malignant. gene region (16p13) has been described in a few cases of, M. Beatriz S. Lopes, Bernd W. Scheithauer, in, John M. Collins, Gregory A. Christoforidis, in, Handbook of Neuro-Oncology Neuroimaging (Second Edition), Daniel J. Brat MD, PhD, Arie Perry MD, in, Practical Surgical Neuropathology: A Diagnostic Approach (Second Edition), Surgical Pathology of Neoplasms of the Central Nervous System, Stem cells and progenitor cell lineages as targets for neoplastic transformation in the central nervous system, Claudia Petritsch, Scott R. VandenBerg, in, Microsurgical Approaches to the Ventricular System, Principles of Neurological Surgery (Third Edition). Despite documentation of anaplastic features (mitoses, vascular endothelial cell hyperplasia, necrosis), their behavior is universally benign (Cuccia et al 2003; Kim et al 2001) and they are graded as WHO I. Pleomorphic xanthoastrocytoma (PXA) occurs predominantly in children and young adults often located superficially, with occasional extension into overlying meninges. In their original series, Kepes and colleagues proposed an origin from sub-ependymal astrocytes, based on ultrastructural similarities between these and PXA tumor cells. In addition to TSC1 and TSC2 mutations, activating BRAF V600E mutations have been identified in SEGAs, with one study demonstrating mutations in 6 of 14 cases.13,40, SEGAs are benign tumors (WHO grade I) and likely represent hamartomatous rather than neoplastic proliferations.41 Malignant transformation is not a part of the natural history of this tumor type. Histologically, SEGAs are composed of heterogeneous cells exhibiting a broad range of astroglial phenotypes (Fig. The small cells were initially described as having oligodendroglial features but their processes are immunoreactive for synaptophysin and neuron-specific enolase (Leung et al 1994), suggesting a neuronal lineage. These features are similar to those seen in tubers, the hamartomatous cortical lesions of tuberous sclerosis. This is a complex group of embryonal tumors, occurring in the supra-tentorial compartment and composed of cells resembling primitive neuroectoderm of the developing nervous system. Recent studies have shown that SEGAs express thyroid transcription factor 1 (TTF-1), a feature shared by other tumors arising from ventral forebrain structures, such as pituicytoma and chordoid glioma of the third ventricle.34–36 While SEGAs have traditionally been categorized as astrocytomas, their histogenesis is not completely defined and there are often tumor cells present that more closely resemble neurons or have intermediate features with astrocytoma-like cytoplasm and neuronal-like nuclei; these may show staining for 68-kD neurofilament protein, synaptophysin (Fig. If you do not want your question posted, please let us know. SEGAs may fill the lateral ventricle and distort the adjacent brain due to mass effect, but do not usually show invasive properties. Mitotic figures and necrosis are common. A grading of WHO II reflects relatively aggressive behavior (Chikai et al 2004; Fernandez et al 2003; Komotar et al 2004). The two major genetic bases of TSC have their origin in abnormalities of chromosomes 9q (TSC1) and 16p (TSC2). Obstruction of CSF flow can result in hydrocephalus and transependymal edema. TSC is an autosomal dominantly inherited neurocutaneous syndrome that affects any organ system of the body. Angiocentric glioma is a low-grade (WHO I), non-aggressive tumor of probable but uncertain glial histogenesis, which occurs most frequently in the cerebral hemispheres. The term ‘desmoplastic infantile astrocytoma/ganglioma’, used in the 2000 and 2007 classifications, evolves from the recognition that these tumors display a histologic spectrum from predominantly astrocytic to mixed astrocytic/ganglion cell. Therefore, surveillance is offered to patients with tuberous sclerosis. These features, although typical of SGA, are also features found in most lateral ventricular tumors. Subependymal giant cell astrocytoma (SEGA) is a clinically benign tumor that is usually associated with tuberous sclero- sis complex (TSC) ][1. They are slowly growing, relatively circumscribed neoplasms with a predilection for midline structures – optic nerve and chiasm, hypothalamus, and dorsal brainstem. Pineocytomas are low-grade (WHO I), slowly growing tumors that do not extend beyond the pineal and do not seed the craniospinal axis (Fauchon et al 2000). 7.7D). Follow-up of 79 patients (59 grade A, 20 grade B) showed median survival times of 11 years in grade A and 3.5 years in grade B (Daumas-Duport et al 1997). The majority of tumor cells demonstrate variable immunoreactivity for GFAP and S-100 protein in addition to neuronal-associated epitopes such as class β-III tubulin, NF-H/M (Figs 2.36, 2.37) and neurotransmitters with variable ultrastructural features suggestive of neuronal differentiation, including microtubules, occasional dense-core granules, and rare synapse formation (Lopes & VandenBerg 2007). OBJECTIVES Intraventricular astrocytomas (subependymal giant cell astrocytomas) of tuberous sclerosis have a poor prognosis due to the obstruction of CSF flow. Multinucleation, significant pleomorphism, and scattered mitoses can be seen in SEGAs but should not raise concern for anaplasia. Grossly, SEGAs are typically circumscribed, solid nodules sharply demarcated from underlying parenchyma. Subependymal giant cell astrocytomas are nodular, solid tumors arising from the wall of the lateral ventricle, often overlying the basal ganglia.1 Less frequently, they arise in the third ventricle. Histopathologically, they form an often overlapping morphological and behavioral continuum in contrast to the clear separation between pilocytic, subependymal giant cell and pleomorphic xanthoastrocytomas. These demonstrate GFAP immunoreactivity in their cytoplasm and have been termed, minigemistocytes and gliofibrillary oligodendrocytes (Kros et al 1996; Matyja et al 2001). SEGAs are uncommon tumors and account for less than 1% of all intracranial masses. Also more characteristic of ganglion cell tumors are eosinophilic granular bodies, lymphocytic infiltrates, collagen deposition, and BRAF mutations. The majority of central neurocytomas behave non-aggressively and are graded as WHO II. Everolimus therapy was associated with a clinically meaningful reduction in volume of the primary subependymal giant-cell astrocytoma, as assessed on independent central review (P<0.001 for baseline vs. 6 months), with a reduction of at least 30% in 21 patients (75%) and at least 50% in 9 patients (32%). Once inside the ventricle, surgical landmarks provide orientation. 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