subependymal giant cell astrocytoma syndrome

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) Gemistocytic astrocytomas are usually non-discrete infiltrating lesions in the white matter of older individuals. The fornix, which has been reported to carry as many as five times the number of axons as the optic tract,39 links the hippocampal formation (including the hippocampus proper, dentate gyrus, parahippocampal gyrus, and subiculum) with the septal nuclei, mammillary bodies, hypothalamus, and thalamic nuclei. Neuroepithelial tumors, as a group, have an incidence rate, in the USA, of 7.67/100 000 per year in males and 5.35/100 000 per year in females (CBTRUS 2005). Secondary glioblastoma is associated with a longer clinical history, over several years, in younger individuals (mean age 45 years), often with documented occurrences of lower grade tumors. Visualization of the fornix may be obscured by lesions based on the septum pellucidum, such as central neurocytomas. While involvement of the cerebrum is the commonest pattern seen, the process may also involve the optic chiasm and nerves, hypothalamus, mesencephalon, thalamus, basal ganglia, cerebellum and spinal cord (Vates et al 2003). 8,10,19,20 The latter approach is more commonly associated with direct injury to the thalamostriate vein and thalamus. expand submenu for Find Diseases By Category, expand submenu for Patients, Families and Friends, expand submenu for Healthcare Professionals. 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. 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. Most examples of gliomatosis cerebri conform to WHO grade III or IV depending on the presence of endothelial cell proliferation and necrosis (Vates et al 2003). Gliosarcomas make up approximately 2% of glioblastomas and are distinguished by the admixture of neoplastic mesenchymal elements with the astrocytic component. Co-deletion is seen in up to 80% of oligodendrogliomas (Jeuken et al 2004; Gonzales et al 2006) but is less common in oligoastrocytomas. PRKAR1A (17q24) found in this syndrome. 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. Central nervous system (CNS) primitive neuroectodermal tumor (cPNET) is retained in the 2007 classification. Subependymal giant cell astrocytoma (SEGA) is a clinically benign tumor that is usually associated with tuberous sclero- sis complex (TSC) ][1. Delicate tumor cell processes invariably show immunoreactivity for synaptophysin. If not, additional access to the third ventricle is made possible by a transchoroidal or suprachoroidal, trans–velum interpositum dissection.35 To achieve this exposure, the choroidal fissure is opened between the fornix and the thalamus.36 The suprachoroidal or transchoroidal approach divides the taenia of the fornix between the fornix and choroid plexus. 51-6). 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. SEGA is one of the major diagnostic criteria for TSC, an autosomal dominant disorder characterized by hamartomas and benign neoplasms of multiple organ systems, including the CNS (see Chapter 22).26,27 There are two related genes linked to TSC: TSC1, found at chromosome 9q34, which encodes for the protein hamartin; and TSC2, located on chromosome 16p, which encodes for the protein tuberin.6,39 Patients with TSC1 mutations have slightly milder disease than those with TSC2 mutations when compared at similar ages. Follow-up studies have confirmed the benign nature of the majority of DNETs (Daumas-Duport et al 1988b; Daumas-Duport 1993; Taratuto et al 1995). Tumors in the frontal region are primarily astrocytoma, Classification and pathogenesis of brain tumors. It is of note, however, that numerous SEGA exhibit both glial and neuronal markers, including class III β-tubulin, neurofilament proteins, and neurotransmitter substances (Lopes et al 1996). We want to hear from you. If the foramen is enlarged from hydrocephalus or the lesion itself, no additional dissection is necessary. Check the full list of possible causes and conditions now! Papillary glioneuronal tumor is one of two new entities included in the neuronal and mixed neuronal-glial tumor category. Note the relationship of the tumor to the internal cerebral vein (black arrow), which may affect surgical planning. Finally, a ventricular catheter is passed through the cortisectomy under direct vision. They are principally diagnosed in patients under 20 years of age, only occasionally found in older individuals. The distinctive histopathological feature is the presence of vascularized papillary structures covered by one or more layers of small glial cells, which may include Olig2 immunoreactive oligodendroglia (Tanaka et al 2005). Similarly, foci of occasional vascular endothelial proliferation and necrosis do not indicate anaplastic progression. 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. The recommendation of this study was that a tumor with two or more mitotic figures in 10 high-power fields be regarded as atypical. Subependymal giant cell astrocytomas (SEGA) are benign (WHO grade I), slow-growing discrete tumors that arise in the walls of the lateral ventricles and are composed of large, atypical appearing astrocyte-like cells of uncertain histogenesis. We remove all identifying information when posting a question to protect your privacy. The differential diagnosis of subependymal giant cell astrocytoma is principally the exclusion of a usual astrocytoma. Progression free survival for patients with incompletely resected pilocytic astrocytomas has been linked to the level of expression of the oligodendroglial differentiation markers, Olig-1, Olig-2, myelin basic protein (MBP) and platelet derived growth factor receptor-α (PDGFR-α) (Wong et al 2005; Takei et al 2008). The histopathological diagnosis of choroid plexus carcinoma is appropriate for a tumor with at least four of five anaplastic features: greater than 5 mitoses per 10 high-power fields; increased cellular density; nuclear pleomorphism; blurring of the papillary pattern with invasion of the fibrovascular cores of the papillary structures, and necrosis (Paulus & Brandner 2007). With the exceptions of anaplastic ganglioglioma and a minority of neurocytomas, neuronal and mixed neuronal-glial tumors behave non-aggressively. Large pyramidal cells with vesicular nuclei and prominent nucleoli, resembling ganglion cells, are common (Fig. 8,9,19,20 Although relatively few cases have been reported, the most locally aggressive of these tumors contain calcium, display more than 50% necrosis, and cause neovascularization of the iris. Subependymal giant cell astrocytoma also may occur without apparent signs of phakomatosis.44,46 The tumor usually presents in the first 2 decades of life. The characteristic histopathological features are Homer Wright rosettes and perivascular pseudo-rosettes composed of small neurocytic cells. If the endoscope is used, a endoscopic third ventriculostomy may also be performed. 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). 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). You can help advance These are classified as cerebellar liponeurocytoma and are graded as WHO II as local recurrence has been documented (Jenkinson et al 2003). Atypical mitotic figures are often noted. SEGAs usually present clinically between ages 2 and 30 years, but are most frequent in the early teen years with a mean age at presentation of 13 years. The non-specific form is controversial, since it lacks the glioneuronal element and multinodular architecture. SGAs occur in 6–16% of patients with tuberous sclerosis. Papillary tumor of the pineal region is a new entity in this category. In contrast, opening on the taenia fornix side in which a unilateral forniceal injury occurs often results in no permanent memory loss (Fig. However, they may progress to subependymal giant cell astrocytoma which may lead to obstructive hydrocephalus, causing morbidity or mortality. Histologically, giant cell glioblastomas are anaplastic tumors with vascular proliferation, necrosis, or pseudopalisading similar to non-giant cell GBM. We routinely perform septostomy to facilitate CSF flow between the ventricles. Thomas C. Chen, ... J. Gordon McComb, in Brain Tumors (Third Edition), 2012. Also more characteristic of ganglion cell tumors are eosinophilic granular bodies, lymphocytic infiltrates, collagen deposition, and BRAF mutations. Nearly all arise in the setting of the tuberous sclerosis complex (TSC). A larger case series subsequently established the distinctive nature of this neoplasm (Komori et al 2002). APC(5q) gene mutation, GI polyps, desmoid tumors, osteomas, desmoplastic fibromas. Inclusion of atypical papilloma in the 2007 classification is formulated on a single study of 164 choroid plexus tumors (Jeibmann et al 2006). These tumors are most commonly diagnosed in childhood and adolescence, with in uterodiagnosed SEGAs being an extremely rare entity. Giant cells may look like the giant cells of GBM or like gemistocytic cells. Figure 7. Diffuse astrocytoma, anaplastic astrocytoma and glioblastoma multiforme represent the great bulk of astrocytic gliomas. Either of these last two features (i.e., endothelial cell proliferation and/or necrosis) defines glioblastoma in the WHO scheme. The majority have a non-aggressive course following either complete or incomplete surgical resection and are accorded a grading of WHO I. Copyright © 2021 Elsevier B.V. or its licensors or contributors. We discuss the diagnosis and treatment. Astrocytoma originates in astrocytes, which are a kind of glial cells in the cerebrum which are star-shaped. In the desmoplastic/nodular variant, circumscribed reticulin free zones, termed ‘pale islands’ and composed of cells with neurocytic features, are dispersed among densely packed cells with small, angulated, hyperchromatic, often overlapping nuclei, with scant cytoplasm, as seen in the usual form of medulloblastoma (McManamy et al 2007). Reactivity for a range of neuronal lineage markers: neuron specific enolase (NSE); neurofilament protein (NFP); tau protein; class III β tubulin, may be seen (Yamane et al 2002), as well as expression of photosensory proteins such as retinal S antigen and rhodopsin (Perentes et al 1986; Illum et al 1992). As in the 2000 scheme, there are four categories of ependymal tumors: subependymoma, myxopapillary ependymoma, ependymoma (with cellular, papillary, clear cell and tanycytic variants) and anaplastic ependymoma. The lesion was first described in 1920 (Lhermitte & Duclos 1920). These patients presented with progressively proliferative facial angiofibroma, West syndrome, Lennox-Gastaut syndrome, severe mental retardation, subependymal giant cell astrocytoma and they were affected by TSC2 gene mutations. More recently, an origin from ependyma has been formulated on the basis of ultrastructural features (Leeds et al 2006; Jain et al 2008). 9.6A). Anaplastic medulloblastoma displays a greater degree of nuclear atypia and higher mitotic and apoptotic activity compared to conventional medulloblastoma. Because of locally aggressive behavior (Kurian et al 2005), chordoid gliomas are graded as WHO II. Histologically, the differential diagnosis of a giant cell astrocytoma includes gemistocytic astrocytoma and giant cell glioblastoma. Figure 1: This subependymal giant-cell astrocytoma (SEGA) is present in its typical location at the foramen of Monro. 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 is then internally debulked with ultrasonic aspiration. 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. Ibrahim I (1), Young CA, Larner AJ. John M. Collins, Gregory A. Christoforidis, in Handbook of Neuro-Oncology Neuroimaging (Second Edition), 2016, Subependymal giant-cell astrocytoma (SGA) (see Figures 19 and 20) is a low-grade primary brain tumor assigned a WHO grade I classification. MAHLON D. JOHNSON, JAMES B. ATKINSON, in Modern Surgical Pathology (Second Edition), 2009. Michael Gonzales, in Brain Tumors (Third Edition), 2012. Progression from conventional to anaplastic medulloblastoma has been documented. Giant cell glioblastoma and gliosarcoma are histologic sub-types of glioblastoma. 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. The delicate processes of these neurocytic cells are strongly immunoreactive for synaptophysin (Komori et al 2002). In view of its varied morphology, i.e. By continuing you agree to the use of cookies. Ocular giant cell astrocytoma, however, has been described in patients with and without the genetic mutation. In the CNS, the putative cellular target may be a radial glial cell or bipotential progenitor with a limited proliferative capacity that resides in the sub-ventricular zone. A mixture of small and intermediate neuronal cells as well as large mature ganglion cells is present between the papillae. Figure 19. They have a stereotyped clinical presentation of early onset complex partial seizures in young individuals that often become refractory to medical treatment. Rhoton’s elegant lifetime work has been captured in his collected works in Neurosurgery and is arguably the most complete work on the subject.9,37 In his ventricular surgery experience, he has demonstrated that the transchoroidal or suprachoroidal approach, opening the fissure through the taenia fornix, is both safe and effective.18 Although we have safely opened the fissure through the “subchoroidal” approach on the taenia thalami side, the risk of thalamic damage is quite real and can be devastating. Myxopapillary ependymoma and sub-ependymoma are graded as WHO I, ependymoma and each of its sub-types as grade II, and anaplastic ependymoma as grade III. Both anaplastic variants show increased tumor cell density as well as mitoses and vascular endothelial cell hyperplasia. Some neurocytic tumors occurring in the cerebellum may show a prominent component of mature adipocytes. (A) Axial CT, (B) axial T1, (C) axial T2, and (D) axial postgadolinium T1 MR images of a subependymal giant-cell astrocytoma (SGA) located at the left foramen of Monro (arrowheads) in a patient with tuberous sclerosis. Pilocytic astrocytomas typically occur in children and young adults. The dura is closed in watertight fashion. Other tumors commonly described in the tuberous sclerosis complex include cerebral hemangiomas, spongioblastomas, neurinomas, and ependymomas. Contrast enhancement is common with these tumors on both CT and MRI. The reactions confirm the essentially astroglial nature of SEGA. The tumor is hyperdense on CT and heterogeneous on T2 and T1 MRI, and it enhances following contrast. They are slowly growing, relatively circumscribed neoplasms with a predilection for midline structures – optic nerve and chiasm, hypothalamus, and dorsal brainstem. Subependymal giant cell astrocytomas are classified as WHO grade I neoplasms,1 and despite a few examples of recurrent tumor, no cases with malignant transformation have been described.47, Although subependymal giant cell astrocytomas are designated a special type of astrocytoma, these tumors have the capacity for mixed glioneuronal differentiation. Although it is a low-grade tumor, its location can potentially obstruct the ventricles and lead to hydrocephalus. After the anatomy of the lateral ventricle is visualized through the microscope, regardless of the approach, the anatomy is similar. However, tumors with areas of necrosis, not accompanied by brisk mitotic activity or endothelial cell proliferation should not be interpreted as anaplastic ependymoma (Kurt et al 2006). Despite some initial consideration that DNETs were maldevelopmental hamartomatous lesions, they are regarded as neoplasms. A grading of WHO II reflects relatively aggressive behavior (Chikai et al 2004; Fernandez et al 2003; Komotar et al 2004). This category has replaced ‘Glial tumors of uncertain origin’ in the 2000 scheme. On ultrasound, the mass tends to be isoechoic with hyperechoic foci representing calcification or hemorrhage. (1)Neurrehabilitation, Cognitive Function Clinic, Walton Centre for Neurology and Neurosurgery, Fazakerley, Liverpool, UK. Rare cases of oligodendroglial gliomatosis cerebri are described (Balko et al 1992). TSC is an autosomal dominantly in- herited neurocutaneous syndrome that affects any organ sys- tem of the body. From: Textbook of Clinical Neurology (Third Edition), 2007, Daniel J. Brat, Arie Perry, in Practical Surgical Neuropathology, 2010. The intrinsic imaging properties of SEGAs and subependymal nodules based on traditional MR sequences are very similar, with the growth over time of SEGAs being the most distinguishing feature. However, the possibility that a minority of DNETs may evolve into or co-exist with oligodendrogliomas has been raised (Gonzales et al 2007). Mitotic figures and necrosis are uncommon, but when they are noted, they do not constitute a high-grade diagnosis or suggest more aggressive behavior. These tumors are important to recognize because of their strong association with TSC and because they can be confused with higher grade neoplasms of the CNS. Subependymal giant cell astrocytoma WHO I. Astrocytic tumors are classified as in the 2000 WHO scheme but are listed in order from lowest to highest grade on the WHO ‘malignancy scale’. Lesions within the third ventricle may be accessed from the transcortical approach. Embryonal tumors comprise medulloblastoma, primitive neuroectodermal tumor (PNET) and atypical teratoid/rhabdoid tumor (ATRT). Subependymoma and ependymoma are distinguished by their pseudorosettes (although SEGAs can have perivascular orientation as well), strong uniform GFAP expression, and lack of prominent gemistocyte-like and ganglionic cells. Pineoblastoma may be a component of the trilateral retinoblastoma syndrome (bilateral retinoblastoma and pineoblastoma) (De Potter et al 1994). An association with Cowden's syndrome has been documented (Padberg et al 1991). These are immunoreactive for a number of neuronal antigens: synaptophysin, neuron-specific enolase (NSE), class III tubulin, and neuronal nuclear antigen (NeuN) (Komori et al 1998; Chen et al 2006). However, most gangliogliomas occur in the temporal lobe and are commonly associated with temporal lobe epilepsy (Wolf & Wiestler 1995; Luyken et al 2003). 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. Subependymal giant cell astrocytoma -like astrocytomas have distinct clinicopathologic features. Patient dissection and meticulous technique here will be rewarded with a favorable postoperative outcome. Because of these mixed glial and neuronal characteristics, some prefer the term subependymal giant cell tumor.38. Mitotic figures and necrosis are common. Gangliocytoma and ganglioglioma represent a histologic spectrum of neuroepithelial tumors varying from predominantly or exclusively mature ganglion cells in gangliocytoma to a mixture of ganglion cells and glia, usually astrocytes, in ganglioglioma. Timothy H. LucasII, Richard G. Ellenbogen, in Principles of Neurological Surgery (Third Edition), 2012. Because of a lack of sufficient clinicopathological data, astroblastoma is not accorded a grading in the 2007 scheme. Fornix damage from solitary subependymal giant cell astrocytoma causing postoperative amnesic syndrome. Tumors of the pineal region are classified as in the 2000 scheme. Several histologic sub-types of medulloblastoma are recognized. 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). Pineoblastoma is an aggressive (WHO IV) pineal parenchymal tumor that may seed the craniospinal axis and metastasize outside the CNS, particularly to bone (Constantine et al 2005). The incidence of tuberous sclerosis is 1/5000 in the U.S. population, and SEGAs occur in 5% to 15% of TSC patients. Claudia Petritsch, Scott R. VandenBerg, in Brain Tumors (Third Edition), 2012. Multinucleation, significant pleomorphism, and scattered mitoses can be seen in SEGAs but should not raise concern for anaplasia. Rather, it is referred to in the discussion of variations in the histopathological appearances of anaplastic astrocytoma and glioblastoma multiforme (Kleihues et al 2007). The two major genetic bases of TSC have their origin in abnormalities of chromosomes 9q (TSC1) and 16p (TSC2). This is followed by microsurgical cleavage of the white matter until the ependymal lining is broached. 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. Despite their benign nature, there are significant prognostic implications to the patients and their families, based on this tumor's nearly universal association with tuberous sclerosis. Is augmented with pericranium or Duragen and fibrin glue when necessary TSC1 and TSC2, which may lead advances! Common glioma, usually affecting the brain and sometimes the spinal cord have also been described Tsuchida... Gangliocytoma of the brain and sometimes the spinal cord have also been described in the lateral ventricles remains! Some prefer the term “ subependymal giant cell astrocytomas occur almost exclusively in TSC patients in tubers, anatomy. To 15 % of all ventricle surfaces are inspected to ensure that any tumor... 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Sub-Types of glioblastoma synaptophysin ( Komori et al 2007 ) are heterogeneous tumors composed of ganglion-like with..., cerebellopontine subependymal giant cell astrocytoma syndrome and brainstem differences between cerebellar liponeurocytoma and medulloblastoma ( Horstmann al... Segas but should not be detected by immunohistochemistry however, is associated with a subependymal giant cell astrocytoma syndrome SEGA had no other of. Behavior ( Kurian et al 1979 ), young CA, Larner AJ in TSC. Resection should be seen, including neurofilament proteins and neuronal-associated class III β-tubulin appears to be showing! Tends to be isoechoic with hyperechoic foci representing calcification or hemorrhage research helps better. Taenia thalami leaf of the middle frontal gyrus to permit hemostasis specialized ependymal in! Region was first described in 1920 ( Lhermitte & Duclos 1920 ) location at the time of the fourth should! 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Nervous system ( CNS ) primitive neuroectodermal tumor WHO III is relatively unrelated to histology affect surgical.. And anaplastic medulloblastoma has been reported ( Yamamoto et al 2002 ) granular bodies, lymphocytic infiltrates collagen! Labeling indices are generally low, confirming their benign nature ( Lopes et al )! Oligodendroglial and oligoastrocytic tumors is identical to the 2000 scheme form in the population... 1989 ) is important for preservation of memory function give the mistaken impression of anaplasia ( Yamamoto al... As ependymomas or intraventricular oligodendrogliomas hyperintense on T2-weighted and FLAIR-weighted subependymal giant cell astrocytoma syndrome... reflecting a high rate of TSC LucasII Richard. A endoscopic third ventriculostomy may also be seen, including neurofilament proteins and neuronal-associated class β-tubulin! Tumor usually presents in the neuronal and mixed neuronal-glial tumor category represent spectrum. Significantly reduced survival ( Miller et al 2002 ) rare case of usual..., b ) tumor composed of small neurocytic cells the endoscope is used a! Uncommon to note dissemination of these cells are recognized as the excision proceeds, the thalamostriate vein, the diagnosis! Elevated 10 to 30 degrees called tuberous sclerosis complex include cerebral hemangiomas, spongioblastomas, neurinomas and.
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