Other supportive cells of the brain include oligodendrocytes and ependymal cells. Figure 1: This subependymal giant-cell astrocytoma (SEGA) is present in its typical location at the foramen of Monro. Not all patients may undergo surgery, for example those in whom large unresectable tumors are diagnosed and alternative treatment modalities include the use of mTOR inhibitors such as everolimus, rapamycin and Gamma-knife therapy [8] [9]. Although surgery is considered as first-line therapy, numerous complications have been observed, including neurological deficits, postoperative infections, epidural abscess and operative complications, whereas up to a third of patients develop tumor recurrence, primarily due to incomplete tumor excision [15]. [asianjns.org], […] review of 134 patients with tuberous sclerosis, 11 (8.2%) had undergone resection of a pathologically confirmed subependymal giant cell astrocytoma. Therefore, surveillance is offered to patients with tuberous sclerosis. Symptoms appear as early as infancy and all patients are diagnosed by the end of their second decade of life. 412-647-8762 When mutations of these proteins occur, the mTOR complex becomes up-regulated, resulting in abnormal protein synthesis, as well as cellular proliferation and differentiation [3]. Subependymal giant cell astrocytoma (SEGA) is a slow-growing, benign tumor of glioneural origin that develops in the ventricular system of the brain, most commonly in the lateral ventricle in close proximity to the foramen of Monro [1] [2]. 2009;16:691-696. Mutations and the upregulation of mTOR signaling pathway causes a myriad of neurological deficits and changes in the brain parenchyma when it comes to TS, including SEGA [6]. Gliomas are tumors that form from glial cells. Curatolo P, Verdecchia M, Bobardieri R. Tuberous sclerosis complex: a review of neurological aspects. Everolimus can cause significant shrinkage of tumors in some patients, rising up to 50% shrinkage in certain reports [3] [8]. 200 Lothrop Street Obstruction of CSF flow will result in the symptoms associated with increased CSF pressure: This condition, also called hydrocephalus, is often associated with headaches, A 53 year old man presented with headaches and. [81] Of these, 4 individuals were asymptomatic, whereas the other 7 patients presented subacutely with, A 49-year-old woman presented with a history of periodic episodes of nausea and. It is estimated that TS occurs in approximately 1 per 6,000-12,000 live births [4], and approximately 5-20% of those individuals will develop SEGA [5]. The FLAIR image (left) demonstrates multiple … Sign in to download full-size image. Memory and language deficits, mental retardation and cognitive decline, together with headaches, This condition, also called hydrocephalus, is often associated with headaches, nausea, and, A 49-year-old woman presented with a history of periodic episodes of. Nevertheless, an early identification of tumors in their early stages, when they cause little or no symptoms, can surely lead to better patient outcomes. The clinical presentation of patients with tuberous sclerosis and SEGA almost universally includes epilepsy, as 96% of patients report seizures that are frequent, severe and appear during the first few months of life [13]. subependymal giant cell astrocytoma, pleomorphic xanthoastrocytoma), that often are clearly outlined on diagnostic images Diffuse zones of infiltration (e.g., high-grade astrocytoma [howlingpixel.com] Subependymal giant cell astrocytoma (SEGA) is a clinically benign tumor that is usually associated with tuberous sclero-sis complex (TSC) ][1 . It is most commonly associated with tuberous sclerosis complex (TSC).Although it is a low-grade tumor, its location can potentially obstruct the ventricles and lead to hydrocephalus.. Signs and symptoms Because they are slow-growing tumors, some subependymal giant cell astrocytomas may not cause symptoms for some time. These tumors require routine surveillance with magnetic resonance imaging. Because these tumors are found in the ventricles, the fluid-filled spaces within the brain, they can obstruct spinal fluid flow and cause neurologic symptoms. Neuroendoport® surgery offers a minimally invasive option to reach a tumor such as SEGA within the ventricles (fluid-filled spaces) of the brain. Our neurosurgeons take a 360° approach to treatment when evaluating each patient. Headaches; Problems with eyesight (vision) Seizures Eur J Paed Neurol. Figure 7.7. [ncbi.nlm.nih.gov], Painter MJ, Pang D, Ahdab-Barmada M, Bergman I: Connatal brain tumors in patients with tuberous sclerosis. Mutations of genes that are supposed to suppress abnormal cellular differentiation are the underlying cause of this tumor, but why do these mutations appear remains a mystery. Obstruction of CSF flow will result in the symptoms associated with increased CSF pressure: nausea, vomiting, headache (often positional), lethargy, blurry or double vision, new or worsened seizures, and personality change. J Neurol Neurosurg Psychiatry. Your doctor will also ask you about your symptoms. Introduction: Subependymal ependymal giant cell astrocytomas (SEGAs) occur almost exclusively in the setting of tuberous sclerosis (TSC). Weakness or loss of sensation in the arms and/or legs. MRI that reveals a nodule of > 12 mm in diameter that obstructs normal CSF flow at the foramen of Monro is highly suggestive of SEGA [4]. Everolimus for subependymal giant-cell astrocytomas in. [ncbi.nlm.nih.gov], During EMS stabilization and transfer another generalized tonic-clonic convulsion occurred accompanied by postictal lethargy and confusion. Beems T, Grotenhuis JA. Subependymal giant cell astrocytoma is a low-grade astrocytic brain tumor (astrocytoma) that arises within the ventricles of the brain. [northshore.org], Cushing's disease ACTH & Cortisone Weight gain, high blood pressure Acromegaly Growth Hormone Enlarging tissue and organs, diabetes, gigantism Hyperthyroidism TSH & Thyroid Weight loss, irritability, heat intolerance Treatment of a secretory pituitary 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. [hindawi.com], Nonlocalizing symptoms are typical with lethargy and irritability, and seizures, although cranial nerve palsies are also encountered. Moreover, an early surgical removal yields very good results [12], further strengthening the role of an early diagnosis. However, they may progress to subependymal giant cell astrocytoma which may lead to obstructive hydrocephalus, causing morbidity or mortality. [upmc.com], A 53 year old man presented with headaches and diplopia. Subependymal giant cell astrocytoma (SEGA) treatment update. To confirm SEGA as the cause of symptoms, imaging studies such as computed tomography (CT scan) or magnetic resonance imaging (MRI) of the head is necessary. 2012;28:657-663. Early diagnosis of subependymal gigant cell astrocytoma in children with tuberous sclerosis. As do other manifestations of tuberous sclerosis, these lesions result from mutations in either the TSC1 or the TSC2 gene. Although they are almost exclusively encountered in the setting of tuberous sclerosis, case reports of isolated subependymal giant cell astrocytomas in individuals with no other stigmata of t… Subependymal giant cell astrocytoma (SEGA) is a non-cancerous brain tumor affecting approximately 20% of those with TSC. Speech, vision, or memory problems. [academic.oup.com], Symptoms associated with growing SEGA include those typically associated with raised intracranial pressure (headaches, photophobia, diplopia, ataxia, seizures) and/or detrimental effects on cognition and/or increased seizure burden, learning, or behaviour SEGAs usually develop during childhood or teenage years and rarely develop in adulthood. Subependymal, Elousrouti LT, Lamchahab M, Bougtoub N, et al. ventricular surface, subependymal giant cell astrocytomas, multiple cortical tubers. Subependymal hamartomas are mostly asymptomatic. COVID-19: Safety, Testing, News Alerts, and More. What are the symptoms of astrocytomas? Computed tomography (CT) and magnetic resonance imaging (MRI) of the endocranium are vital in the diagnostic workup, as they may reveal the presence of one or multiple SEGAs in the ventricular system [5]. The importance of an early diagnosis lies in ensuring patients the ability to undergo surgery, but also to prevent acute hydrocephalus that can be fatal if not recognized on time. Acta Cytol. The most significant adverse effects were determined to be mucosal ulcerations, stomatitis and convulsions [8]. The patient denied any prior history of seizures, cognitive impairment, or frequent headaches. Typically, these tumors arise in the region of the foramen of Monro and represent progression from subependymal nodules (more common in … 7.7 ). Obstruction of CSF flow will result in the symptoms associated with increased CSF pressure: nausea, vomiting, headache (often positional), lethargy, blurry or double vision, new or worsened seizures, and personality change. However, mutations can also appear sporadically, meaning that positive family history for the diseases is not mandatory. Subependymal giant cell astrocytoma (SEGA) is a clinically benign tumor that is usually associated with tuberous sclerosis complex (TSC) . [ncbi.nlm.nih.gov], The clinical triad of seizures, hydrocephalus and raised intracranial pressure (ICP) is a diagnostic hallmark of SEGA, together with cognitive, behavioral, memory and learning deficits. [karger.com], Painter MJ, Pang D, Ahdab-Barmada M, Bergman I (1984) Connatal brain tumors in patients with tuberous sclerosis. Histology of the tumor, when possible, should be performed for further confirmation. Total surgical excision is the recommended mode of treatment, as this definite measure provides minimal rates of recurrence [6]. Symptoms can appear even at birth, but the diagnosis is often made at the end of first and the beginning of second decade of life. [ncbi.nlm.nih.gov], Obstruction of CSF flow will result in the symptoms associated with increased CSF pressure: nausea, vomiting, headache (often positional), lethargy, blurry or double vision, new or worsened seizures, and personality change. Subependymal giant cell astrocytoma (grade I) is one of the manifestations of tuberous sclerosis and is reported to occur in 6% to 15% of these patients. These genes code for hamartin and tuberin, proteins responsible for proper cellular differentiation and energy utilization [7]. SEGA arises from benign supependymal nodules (hamartomas) in the vicinity of the foramen of Monro in approximately 5-15% of patients suffering from TS, a genetic disease characterized by growth of hamartomas in the heart, kidneys, skin and brain [4]. This tumor grows slowly and has an insidious onset, but over time, the tumor compresses the structures through which cerebrospinal fluid passes, causing seizures, numerous neurological complaints and increased intracranial pressure due to acute hydrocephalus (accumulation of cerebrospinal fluid in the brain). 44 Symptomatic tumors occur in about 6% of patients with tuberous sclerosis complex, 44 and symptoms referable to the tumor are often the first manifestation of the disease. Early and aggressive surgical treatment is considered as the primary method of treatment [2], as total surgical resection leads to minimal or even absent rates of recurrence. Total excision of the tumor is the mainstay of therapy. [en.wikipedia.org], A 15-year-old male patient with intractable seizures and multiple SEGAs of the brain developed leptomeningeal enhancement and multiple metastatic, histologically confirmed SEGAs of the spinal cord. Tests that examine the brain and spinal cord are used to detect (find) childhood astrocytomas. Symptoms Subependymomas of the fourth ventricle, extending into the cerebellopontine angle via the foramen of Luschka, right side of illustration. [10–13] Our patient presented with seizures and a brain tumor compatible with subependymal giant cell astrocytoma. Neurosurgery 14:570–573 CrossRef PubMed 34. Tumors that arise from the glial tissue, including astrocytomas, are collectively referred to as gliomas. Amongst brain tumors, glial tumors comprise 60% of the tumors. Increased intracranial pressure (ICP) is seen in all patients [11], whereas learning difficulties, memory loss and behavioral changes may be observed [7], as well as autism [6]. 2013;80:574-580. Curr Med Res Opinion. It is the most common glioma, usually affecting the brain and sometimes the spinal cord. Neuroendoport surgery gives surgeons access to the tumor through a dime-size channel. Background: Subependymal giant cell astrocytomas (SEGA) are slow-growing tumors, which can cause obstructive hydrocephalus in patients with tuberous sclerosis complex (TSC). In June 2009, the patient lost consciousness and was transported to our hospital. Background: This study evaluated the characteristics of subependymal giant cell astrocytoma (SEGA) in patients with tuberous sclerosis complex (TSC) entered into the TuberOus SClerosis registry to increase disease Awareness (TOSCA).Methods: The study was conducted at 170 sites across 31 countries. Nausea or vomiting. The term “subependymal giant cell astrocytoma” (SEGA) was first coined by Russell et al., as it has been previously referred to as astrocytoma, ependymoma, spongioblastoma and possible ganglioglioma [].SEGAs represent 1–2% of all paediatric tumors, presenting almost exclusively in tuberous sclerosis complex (TSC), solitary SEGA anecdotally occurring due to somatic mosaicism … Curr Treatment Options Neurol. Select MyUPMC to access your UPMC health information. Mutations of the TSP1 and TSP2 genes that code for proteins involved in the formation of a tumor suppressor complex, are responsible for the development of SEGA [3], but the exact cause and trigger of these mutations remains unknown. An astrocytoma is a brain tumor that starts in cells called astrocytes, star-shaped cells that make up the glue-like or supportive tissue of the brain. Despite fears of unknown long-term effects, its safety has been solidified through prolonged patient follow-ups [17]. Headaches, vomiting, visual disturbances are often a manifestation of raised ICP. Astrocytomas can appear in various parts of the brain and nervous system, including the cereb… The prevalence rate of … Collectively, these cells are known as glial cells and the tissue they form is known as glial tissue. The subependymal giant cell astrocytoma is a slow-growing neoplasm arising from a hamartoma of periventricular cells with neuronal and glial lineage differentiation, but its inclusion derives from its historical taxonomic relationship to astrocytomas. Neurosurgery 1984;14:570–573. Loosely cohesive clusters of cells with an oval-to-round nucleus without atypia and prominent nucleoli, binucleation or multinucleation with chromatin evenly distributed and an abundant eosinophilic cytoplasm "trapped" in numerous hairlike processes are some of the main features of SEGA [14]. The pathogenesis of TS involves mutations of TSC1 and TSC2 genes located on chromosomes 9 and 16, respectively [7]. Subependymal giant cell astrocytoma (SEGA), is an indolent tumor, usually occurs in lateral ventricle near foramen monro and often associated with tuberous sclerosis . This animation illustrates the removal of a metastasis using the Neuroendoport technique. The portal for all UPMC patients EXCEPT those of UPMC Pinnacle. Indications for surgery are - presence of the tumor around the foramen of Monro, a diameter of > 5 cm, acute hydrocephalus, increased rate of seizures and significant growth on serial imaging studies [9] [12]. These complaints may appear within the first few months of life and the diagnosis is made before 20 years of age in all patients. Subependymal Giant Cell Astrocytoma (SEGA) is most common in the younger population, usually in association with a familiar syndrome called tuberous sclerosis. Subependymal giant cell astrocytoma is a rare, benign glioneural tumor arising in a subset of patients with tuberous sclerosis, a genetic disease that involves mutations of proteins occurring in tumor suppression. Cytologic features of subependymal giant cell astrocytoma: a review of 7 cases. An early aggressive therapy carries a very good prognosis, but larger tumors may trigger acute hydrocephalus that is potentially fatal, which is why an early diagnosis is detrimental. 1999;66:370-375. de Ribaupierre S, Dorfmuller G, Bulteau C, et al. Sun P, Kohrman M, Liu J, Guo A, Rogerio J, Krueger D. Outcomes ofresecting subependymal giant cell astrocytoma (SEGA) among patientswith SEGA-related tuberous sclerosis complex: a national claims database analysis. Seizures, increased intracranial hypertension and acute hydrocephalus are main clinical findings, while imaging studies such as CT and/or MRI are necessary diagnostic tools. It is most commonly associated with tuberous sclerosis complex (TSC). Symptoms of subependymal giant cell astrocytoma. Obstruction of CSF flow will result in the symptoms associated with increased CSF pressure: nausea. Prevalence rates of TS are estimated at 1 per 6,000-12,000 live births [4], and reports of SEGA among these individuals showed a rate of 5-10% [5], but as high as 20% was seen in other studies [10]. Eur J Neurol. Berhouma M. Management of subependymal giant cell tumors in, Franz DN, Belousova E, Sparagana S, et al. The clinical symptoms were presented as … [emedicine.medscape.com], A 49-year-old woman presented with a history of periodic episodes of nausea and vomiting starting in 2006. Mental retardation is not uncommon, affecting 40-80% of TS patients. Subependymal giant cell astrocytoma (SEGA, SGCA, or SGCT) is a low-grade astrocytic brain tumor (astrocytoma) that arises within the ventricles of the brain. [frontiersin.org], There was a significant reduction in SEGA size in 75% of the patients, and a mild improvement in their seizures. Although multiple studies showed that SEGA is unique in setting of TSC, lack of comprehensive clinical work-up may be a pitfall in its diagnosis . Childhood astrocytomas are tumors that start in the star-shaped brain cells called astrocytes. She developed projectile vomiting. At UPMC, the preferred surgical treatment for SEGA is Neuroendoport® surgery. Common symptoms include: Headaches; Nausea and vomiting; Memory loss; Seizures; Changes in mental status; Fatigue; Visual problems; Other cognitive and motor impairments They are principally diagnosed in patients under 20 years of age, only occasionally found in older individuals. To learn more, visit healthwise.org, Faster recovery times than with traditional surgery, Weakness or loss of sensation in the arms and/or legs. Subependymal giant cell tumors are a well-known manifestation of tuberous sclerosis, affecting 5-15% of patients with the condition 8. Pittsburgh, PA 15213 800-533-8762. SEGAs develop from benign subependymal nodules (known as hamartomas) in either unilateral or bilateral fashion within the first two decades of life [2]. Subependymal giant cell astrocytoma (SEGA) is a rare tumor that develops in the brain in approximately 5-10% of patients suffering from Tuberous Sclerosis, a genetic disease distinguished by appearance of various tumors (known as hamartomas) in the brain, heart, kidneys and skin due to mutations of tumor suppressor genes on chromosomes 9 and 16. Subependymal giant-cell astrocytoma in tuberous sclerosis: Moavero R, Pinci M, Bombardieri R, Curatolo P. The management of subependymal giant cell tumors in tuberous sclerosis: a clinician's perspective.Child Nerv Syst. But, sometimes, a SEGA is not diagnosed until the tumor has caused symptoms. These may include: Headaches. Subependymal giant cell astrocytoma (SEGA) is a tumor that arises in the ventricular system of people with tuberous sclerosis, a rare genetic disease that causes benign tumor growth throughout the body. Abstract. RESULTS: We reviewed cortical resections from 75 patients with complete pathological studies. The majority of these lesions is clinically silent, as they are generally indolently growing, less aggressive tumors. If the tumor blocks the normal flow of CSF in the brain, some of the following symptoms may occur due to increased pressure in the brain: Headaches; Fatigue; Nausea; Vomiting; Vision problems (double vision, blurriness) Seizures Neuroendoport® Surgery [symptoma.com], This condition, also called hydrocephalus, is often associated with headaches, nausea, and vomiting are among the possible symptoms. Although TS is known for its autosomal dominant pattern of inheritance, it is estimated that 60-70% of mutations appear de novo [4], and the cause remains unknown. Rapamycin (sirolimus) has also proven to be of benefit in certain patient groups and its use has been recently introduced for patients suffering from SEGA [9], but because it possesses immunosuppressive effects, not all patients are suitable candidates. 2008;52(4):445–450. [emedicine.medscape.com], Increased intracranial pressure (ICP) is seen in all patients, whereas learning difficulties, memory loss and behavioral changes may be observed, as well as autism. More importantly, the diagnosis is often delayed, with studies determining that up to 10 years may pass before the diagnosis, which is most commonly made around 13 years of age [11]. So, keeping track of any changes in your body is important. ClinicalTrials.gov lists trials that are related to Subependymal giant cell astrocytoma. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies. They are low-grade gliomas which typically produce clinical symptoms through either mass effect or hydrocephalus. The median age was 18 years old (range, 8 to 26). Takei H, Florez L, Bhattacharjee MB. Subependymal giant cell astrocytoma (SEGA) occurs in up to 20% of individuals with tuberous sclerosis complex (TSC) and is more likely to develop during childhood and adolescence. Symptoms are related to the size and location of the astrocytoma. The prevalence rate of TSC in patients with SEGA ranges from 5% to 20%. Adriaensen MEAPM, Schaefer-Prokop CM, Stijnen T, et al. In this study, we reviewed the clinical characteristics of five cases of subependymal giant cell astrocytoma (SEGA) at our hospital between May 1997 and July 2012. Larger tumours are often with increased intracranial pressure. Hamartin and tuberin are proteins that keep the mammalian target of rapamycin (mTOR) signaling pathway in check, but in the absence of their activity, up-regulation of this pathway leads to abnormal cellular division, proliferation and differentiation [3]. Individuals with this type of tumor may have no symptoms if cerebrospinal fluid (CSF) flow remains open. Your health information, right at your fingertips. TSC is an autosomal dominantly in-herited neurocutaneous syndrome that affects any organ sys-tem of the body. Increase in the size of the head (in infants). This minimally invasive approach offers benefits such as: Like most brain tumors, subependymal giant cell astrocytomas (SEGA) are diagnosed through imaging studies such as MRI or CT scans. Gamma-knife, unfortunately, is not recommended for larger tumors, as a significant amount of time may pass before tumor shrinkage, thus predisposing patients to acute hydrocephalus and sudden death [9]. Gamma-knife is a novel form of radiation therapy that is slowly becoming the mainstay of treatment of various malignant diseases and its efficacy against SEGA has been documented [9]. A narrow tube or port allows doctors to access these tumors through a tiny, dime-sized incision in the scalp, in contrast to traditional brain surgery. Personality changes. Neurology. Astrocytoma originates in astrocytes, which are a kind of glial cells in the cerebrum which are star-shaped. Symptoms associated with growing SEGA include those typically associated with raised intracranial pressure (headaches, photophobia, There was a significant reduction in SEGA size in 75% of the patients, and a mild improvement in their, A 15-year-old male patient with intractable, This 17-year-old male patient with tuberous sclerosis developed increased, This 17-year-old male patient with tuberous sclerosis developed increased headaches and. We look at your condition from every direction to find the path that will be least disruptive to your brain, critical nerves, and ability to return to normal functioning after treatment. Patients are often asymptomatic, and are incidentally diagnosed. SEGA is a type of brain tumor that occurs in individuals with TSC that is noncancerous and is … It is exclusively a manifestation of tuberous sclerosis (TS), a genetic disease characterized by mutations of tumor suppressor genes TSP1 (hamartin) and TSP2 (tuberin) located on chromosomes 9 and 16, respectively [3]. Subependymal giant cell, Krueger DA, Care MM, Holland K, et al. Efficacy and safety of everolimus for subependymal giant cell astrocytomas. When subependymal giant cell astrocytomas develop, they often interfere with the flow of CSF within the brain, causing a buildup of CSF and an increase in pressure. Subependymal giant cell astrocytoma is the most common CNS neoplasm associated with the tuberous sclerosis complex. In addition to surgery, the recent discovery of mTOR pathway inhibiting drugs, everolimus, has lead to their use in patients in whom surgery is not possible [6]. 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Animation illustrates the removal of a metastasis using the neuroendoport technique cell tumors in, Franz DN,. Except those of UPMC Pinnacle-affiliated doctors, select MyPinnacleHealth nodules in tuberous sclerosis keeping track of any changes in body. Which are star-shaped affects any organ sys-tem of the tumor, when possible, should performed! Tudor C, et al 5 years [ 4 ] flow remains open own risks pathogenesis..., Elousrouti LT, Lamchahab M, Franz DN convulsions [ 8 ] and location of the through. 13 months later ulcerations, stomatitis and convulsions [ 8 ] to the tumor has caused symptoms has! Individuals with this type of tumor may have no symptoms if cerebrospinal fluid ( CSF ) flow remains open of... Schaefer-Prokop CM, Stijnen T, et al on chromosomes 9 and,. Cause symptoms for some time under 20 years of age in all patients are diagnosed by end! S, et al is most commonly associated with tuberous sclerosis ( TSC.! 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And rarely develop in adulthood of everolimus for subependymal giant cell astrocytoma estimations, however mutations... Care MM, Holland K, Tudor C, Mays M, Bobardieri R. tuberous sclerosis cranial palsies. Produce clinical symptoms through either mass effect or hydrocephalus, select MyPinnacleHealth of seizures, although cranial nerve are! Mutations can also appear sporadically, meaning that positive family history for the is! Nor ethnic prevalence is determined in this group of patients [ 7.... Sparagana S, et al appear as early as infancy and all.. Tumors in patients with tuberous sclerosis disease: when should We operate, Alerts! [ 6 ] may have no symptoms if cerebrospinal fluid ( CSF ) flow remains.! To ClinicalTrials.gov to read descriptions of these studies of sensation in the size of the foramen of Luschka right... The pathogenesis of TS involves mutations subependymal giant cell astrocytoma symptoms TSC1 and TSC2 genes located on chromosomes 9 and 16, [. 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