For its rarity, we must consider this diagnosis when faced with a mass near the foramen of Monro in the pediatric popu … 2020 Feb 25;11:26. doi: 10.25259/SNI_10_2019. 2017 May;45(5):397-404. doi: 10.11477/mf.1436203521. Subependymal nodules are collections of abnormal, swollen glial cells and giant cells which cannot be differentiated as normal neural tissue. Subependymal giant cell astrocytoma is a rare tumor of the central nervous system whose diagnosis is based on clinical, radiological, histological and immunohistochemical arguments. HHS Precontrast CT, Giant Cell Astrocytoma Postcontrast CT, Giant Cell Astrocytoma TSC is an autosomal dominantly in-herited neurocutaneous syndrome that affects any organ sys-tem of the body. Author information: (1)1 Department of Radiology, Children's Hospital of Philadelphia, Philadelphia, PA, USA. Adachi M, Nakamura M, Shinozaki N, Miyazaki T. No Shinkei Geka. The diagnosis is based on tissue, e.g. 1 As SEGAs are distinct from astrocytomas, several authors have suggested using the term “subependymal giant cell tumor” instead. Surgery is often curative. Background: 29, No. -. [Subependymal nodules-sudependymal giant cell astrocytoma complex in children with tuberous sclerosis]. Around 5-15% of patients with tuberous sclerosis develop these tumors. Clin Neuropathol 34 (3): 128-31. doi : 10.5414/NP300818 . They have a tendency to calcify and can progress into subependymal giant cell astrocytomas, which are histologically indistinguishable from SENs but distinguishable based on their larger size, higher growth rate, and potential for mass effect compared … Unable to process the form. 2. Although they are almost exclusively encountered in the setting of tuberous sclerosis, case repor… Subependymal giant-cell astrocytoma associated with tuberous sclerosis. Neurology. Although this tumor is considered specif-ic for TSC, there are reports of patients with SEGA who have none of the other stigmata of TSC. 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 tuberous sclerosis are available 9. Subependymal Giant Cell Astrocytoma Treatment. Subependymal Giant Cell Astrocytoma. eCollection 2020. We describe the CT and MR findings in a patient diagnosed with tuberous sclerosis after presenting with a hemorrhagic subependymal giant cell astrocytoma (SEGCA). White matter glial abnormalities may also be present.  |  (2003) ISBN:0443071098. 1 There was no history Ichikawa T, Wakisaka A, Daido S, Takao S, Tamiya T, Date I, Koizumi S, Niida Y. 2012;49:439–44. 2013;33 (1): 21-43. (1994) ISBN:0824788265. SEGA is an intraventricular glioneuronal tumor arising from the ventricular wall near the foramen of Monro. From the Radiologic Pathology Archives: Intraventricular Neoplasms: Radiologic-Pathologic Correlation. This site needs JavaScript to work properly. Except for SGCAs, these abnormalities can be seen in almost all patients with TS. Conclusions: Subependymal giant cell astrocytoma (SEGA, SGCA, or SGCT) is a low-grade astrocytic brain tumor (astrocytoma) that arises within the ventricles of the brain. Comparison of primary subependymal giant cell astrocytoma (SEGA) response in individual patients by independent central radiology review at 6 months and 60 months, and the best response at any time point. 2008;190 (5): W304-9. J Neurosurg Pediatr, 16(2):134-137, 15 May 2015 Cited by: 6 articles | PMID: 25978531 Radiographics. 2009;69(1 Pt 1):8-14. 3. After fixation in 10 % neutral-buffered formalin, embedding in paraffin and staining with hematoxylin, eosin and safran, the definitive diagnosis was subependymal giant cell astrocytoma. Subependymal giant cell astrocytoma (SEGA) is a slow-growing benign tumor most often seen in patients with tuberous sclerosis complex (TSC) with an incidence of nearly 15% in this patient population. Subependymal giant cell astrocytomas are believed to arise from a subependymal nodule present in the ventricular wall in a patient with tuberous sclerosis, although this has yet to be categorically established 4,8. These tumours are multilobulated well-circumscribed tumours arising from the wall of the lateral ventricles near the foramen of Monro. Recommendations from the International Tuberous Sclerosis Complex Consensus Conference. -, Watanabe Y, Oki S, Migita K, Isobe N, Okazaki T, Nabika S. A case of subependymal giant cell astrocytoma not associated with tuberous sclerosis. Subependymal giant cell astrocytoma (SEGA) is a clinically benign tumor that is usually associated with tuberous sclero-sis complex (TSC) ][1 . 2007;114 (2): 97-109. Objective: To analyze the cumulative efficacy and safety of everolimus in treating subependymal giant cell astrocytomas (SEGA) associated with tuberous sclerosis complex (TSC) from an open-label phase II study (NCT00411619).Updated data became available from the conclusion of the extension phase and are presented in this ≥5-year analysis doi: 10.1016/j.pediatrneurol.2013.08.017. 8. 2020 Jan 1;17(1-3):10-13. Subependymal giant cell tumors in tuberous sclerosis complex. (2005) The Journal of molecular diagnostics : JMD. Neurosurg Rev. While these tumors are not uncommon in tuberous sclerosis, hemorrhage into them is extremely rare. "Consistent nuclear expression of thyroid transcription factor 1 in subependymal giant cell astrocytomas suggests lineage-restricted histogenesis.". From baseline to 6 months, there was a clinically meaningful reduction in volume of the primary subependymal giant-cell astrocytoma, as determined by independent central review (P < .001). It is the most common cerebral neoplasm in tuberous sclerosis. Fujiwara S, Takaki T, Hikita T, Nishio S. Childs Nerv Syst. ADVERTISEMENT: Supporters see fewer/no ads, Please Note: You can also scroll through stacks with your mouse wheel or the keyboard arrow keys. Subependymal giant cell astrocytoma: diagnosis, screening, and treatment. 1. typically appears as an intraventricular mass near the foramen of Monro 2. they are usually larger than 1 cm 3. lesions are iso- or slightly hypoattenuating to grey matter 4. calcification is common and haemorrhage is possible 5. accompanying hydrocephalus may be present 6. often shows marked contrast enhancement (subepend… Roth J, Roach ES, Bartels U, Jóźwiak S, Koenig MK, Weiner HL, Franz DN, Wang HZ. The dotted lines denote clinically relevant cutoffs of ≥30% and ≥50% reductions from baseline in primary SEGA volume. Glioblastoma (giant cell glioblastoma, gliosarcoma). 22 (6): 1473-505. Get the latest public health information from CDC: https://www.coronavirus.gov, Get the latest research information from NIH: https://www.nih.gov/coronavirus, Find NCBI SARS-CoV-2 literature, sequence, and clinical content: https://www.ncbi.nlm.nih.gov/sars-cov-2/. They have a tendency to calcify and can progress into subependymal giant cell astrocytomas, which are histologically indistinguishable from SENs but distinguishable based on their larger size, higher growth rate, and potential for mass effect compared … 7. Would you like email updates of new search results? UPMC's neurosurgical team may recommend a combination of surgical and non-surgical approaches to treat subependymal giant cell astrocytomas. Do subependymal nodules grow? See this image and copyright information in PMC. Pathophysiology. 2 Ring-shaped lateral ventricular nodules: an incidental finding on brain magnetic resonance imaging Surgery is the standard treatment for subependymal giant cell astrocytoma. AJR Am J Roentgenol. 5. Hill BJ(1), Gadde JA(2), Palasis S(3). No Shinkei Geka. Innov Clin Neurosci. The 2007 WHO classification of tumours of the central nervous system. Louis DN, Ohgaki H, Wiestler OD et-al. NIH They are principally diagnosed in patients under 20 years of age, only occasionally found in older individuals. The tumour in the left hemisphere fulfilled the neuroradiological diagnostic criteria for a subependymal giant cell astrocytoma (SEGA). Serial imaging is most helpful here, as growth implies the latter. Immunohistochemical examination of these tumours demonstrates the following reactivity 8: The foramen of Monro is the classic location, and the tumour arises when a subependymal nodule transforms into subependymal giant cell astrocytomas over a period of time. Recommendations from the International Tuberous Sclerosis Complex Consensus Conference 2012. {"url":"/signup-modal-props.json?lang=gb\u0026email="}, {"containerId":"expandableQuestionsContainer","displayRelatedArticles":true,"displayNextQuestion":true,"displaySkipQuestion":true,"articleId":5888,"mcqUrl":"https://radiopaedia.org/articles/subependymal-giant-cell-astrocytoma/questions/738?lang=gb"}. Objective: To analyze the cumulative efficacy and safety of everolimus in treating subependymal giant cell astrocytomas (SEGA) associated with tuberous sclerosis complex (TSC) from an open-label phase II study (NCT00411619).Updated data became available from the conclusion of the extension phase and are presented in this ≥5-year analysis For its rarity, we must consider this diagnosis when faced with a mass near the foramen of Monro in the pediatric population even if there are no other features of tuberous sclerosis complex. The foramen of Monro is the classic location, and the tumour arises when a subependymal noduletransforms into subependymal giant cell astrocytomas over a period of time. and subependymal giant cell astrocytoma on brain imaging. 1. Clarke MJ, Foy AB, Wetjen N, Raffel C (2006) Imaging characteristics and growth of subependymal giant cell astrocytomas. They can be either asymptomatic or symptomatic due obstructive hydrocephalus, surgery treatment is often curative. Background: Subependymal giant cell astrocytomas (SEGA) are slow-growing tumors, which can cause obstructive hydrocephalus in patients with tuberous sclerosis complex (TSC). The prevalence rate of … Although it is a low-grade tumor, its location can potentially obstruct the ventricles and lead to hydrocephalus. PDF | Subependymal giant cell astrocytomas (SEGAs) are the most common intracranial tumors in Tuberous Sclerosis Complex (TSC). Beaumont TL, Godzik J, Dahiya S, Smyth MD. Genetic testing of the neonate showed the mutation for TS. Gliomatosis cerebri. We report a case of subependymal giant cell astrocytoma in a 10-year-old white girl, who had no clinical symptoms of tuberous sclerosis.  |  For its rarity, we must consider this diagnosis when faced with a mass near the foramen of Monro in the pediatric population even if there are no other features of tuberous sclerosis complex. Subependymal giant cell astrocytoma (SEGA) is a tumor that typically occurs in the lateral ventricles near the foramina of Monro. Subependymal giant cell astrocytoma: diagnosis, screening, and treatment. National Center for Biotechnology Information, Unable to load your collection due to an error, Unable to load your delegates due to an error, Computed tomography scan revealing a voluminous mass in the perilateral ventricle with similar attenuation to that of cortical gray matter (, Tumor composed of spindle cells and globular large cells, producing an aspect of ganglion cells: (. -, Louis DN, Ohgaki H, Wiestler OD, Cavenee WK, editors. a biopsy. (2001) ISBN:0443064261. 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. Based on the radiological abnormalities of the brain and heart, tuberous sclerosis (TS) was strongly suspected. Subependymal giant cell astrocytoma (SEGA) is a clinically benign tumor that is usually associated with tuberous sclerosis complex (TSC) . Fetal subependymal giant cell astrocytoma: A case report and review of the literature. Two commonly affected genes underlying TSC and therefore SEGAs are TSC1 and TSC2, which encode for the proteins hamartin and tuberin, respectively. Watanabe Y, Oki S, Migita K, Isobe N, Okazaki T, Nabika S. Barnes A, Wang MM, Feltes J, Ko J, Guzman MA. subependymal giant cell astrocytoma: report of 20 cases Guang-Hai Mei1,2†, Xiao-Xia Liu1,2†, Ping Zhou1* and Ming Shen1* Abstract Background: Subependymal giant cell astrocytoma (SEGA) is a clinically benign brain tumor associated with tuberous sclerosis complex (TSC). Subependymal giant cell astrocytoma is a rare tumor that occurs in the wall of the lateral ventricle and foramen of Monro and, rarely, in the third ventricle. Pediatr Neurosurg. Surgery. Comparison of primary subependymal giant cell astrocytoma (SEGA) response in individual patients by independent central radiology review at 6 months and 60 months, and the best response at any time point. A smaller number of ganglionic appearing giant pyramidal-like cells 8. A subset of subependymal giant cell astrocytoma-like astrocytomas are alternative lenghtening of telomere-positive and occur in the absence of ATRX alterations, thereby suggesting mutations in other DNA repair/maintenance genes may also facilitate alternative lenghtening of telomeres. Anaplastic astrocytoma. 9. Subependymal giant cell tumors are a well-known manifestation of tuberous sclerosis, affecting 5-15% of patients with the condition 8. There are still controversies on early diagnosis of the tumor. Karagianni A, Karydakis P, Giakoumettis D, Nikas I, Sfakianos G, Themistocleous M. Surg Neurol Int. The ependymal lining over subependymal giant cell astrocytomas remains intact making CSF seeding highly unlikely 7. It is most commonly associated with tuberous sclerosis complex (TSC). https://librepathology.org/wiki/Subependymal_giant_cell_astrocytoma Subependymal giant cell astrocytoma in the absence of tuberous sclerosis complex: case report. Subependymal Giant Cell Astrocytoma Size Measurement in Tuberous Sclerosis Complex: Noncontrast vs Contrast-Enhanced 3-Dimensional T1-Weighted Magnetic Resonance Imaging (MRI). It was described for the first time in 1908 by Vogt as part of the typical triad of tuberous sclerosis complex. Subependymal nodule Subependymal giant cell astrocytoma Cardiac rhabdomyoma, single or multiple Lymphangiomatosis Renal angiomyolipoma Minor features. Astrocytic Tumors Many primary tumors of the central nervous system (CNS) arise from glial cells. Neurosurg Focus 20:E5 PubMed CrossRef Google Scholar. TSC is an autosomal dominantly inherited neurocutaneous syndrome that affects any organ system of the body. WHO Classification of tumours of the central nervous system. Clipboard, Search History, and several other advanced features are temporarily unavailable. Diffuse astrocytoma, anaplastic astrocytoma, and glioblastoma have the same or similar molecular genetics and the basic property of diffusely infiltrating the surrounding CNS tissue. e cortical tubers were the likely etiology of her seizure and the patient was placed on Keppra but more concerning was the astrocytoma mass in the body of the right lateral ventricle. Current consensus guidelines recommend treatment of asymptomatic SEGAs with an mechanistic target of rapamycin (mTOR) inhibitor because … [Hemorrhagic Onset of Subependymal Giant Cell Astrocytoma Associated with Tuberous Sclerosis:A Case Report and Review of Literature]. On imaging, they classically appear as an intraventricular mass near the foramen of Monro, larger than 1 cm, showing calcifications, heterogeneous MRI signal, and marked contrast enhancement. Subependymal giant cell tumours are a well-known manifestation of tuberous sclerosis, affecting 5-15% of patients with the condition 8. Medicina (B Aires). Epub 2013 Oct 15. 2003;31:543–8. doi: 10.1159/000120796. Brain tumors, an encyclopedic approach. COVID-19 is an emerging, rapidly evolving situation. IARC Press: Lyon; 2007. Subependymal giant cell astrocytoma (SGCA). The glial cells, in turn, are comprised of astrocytes, oligodendrocytes, and ependymal cells. 1989 Feb;5(1):43-4. doi: 10.1007/BF00706748. 2013 Dec;49(6):439-44. doi: 10.1016/j.pediatrneurol.2013.08.017. Kumar R, Singh V. Subependymal giant cell astrocytoma: a report of five cases. The estimated prevalence of cortical tuber and/or subependymal nodule is 95%–100% and that of white matter At the 2012 Washington Consensus Conference, it was decided by the invited expert panel to document the definition of subependymal giant cell astrocytoma as a lesion at the caudothalamic groove with either a size of more than 1 cm in any direction or a subependymal lesion at any location that has shown serial growth on consecutive imaging regardless of size. abnormalities are cortical tubers, subependymal nodules, subependymal giant cell astrocytomas (SGCAs), and white matter abnormalities. Most subependymal giant cell astrocytomas will show avid enhancement after contrast administration; however, a growing subependymal lesion even in the absence of enhancement should be considered a subependymal giant cell astrocytoma. Young children who have tuberous sclerosis may be offered to screen because of the increased risk of developing subependymal giant cell astrocytomas. Multiple randomly distributed pits in dental enamel Hamartomatous rectal polyps Bone cysts Cerebral white matter migration lines Gingival fibromas Nonrenal hamartomas Retinal achromic patch Diagnosis. 1 There was no history or evidence of TS on clinical examination of family members. differential for intraventricular tumours, glioma treatment response assessment in clinical trials, World Health Organisation (WHO) oncology response criteria, Response Evaluation Criteria in Solid Tumours (RECIST), ATRX (alpha-thalassaemia/intellectual disability syndrome X-linked), additional variable and focal reactivity: class III beta-tubulin, NeuN, SOX2, typically appears as an intraventricular mass near the foramen of Monro, lesions are iso- or slightly hypoattenuating to grey matter, calcification is common and haemorrhage is possible, accompanying hydrocephalus may be present, often shows marked contrast enhancement (subependymal nodules also enhance). Textbook of Radiology and Imaging. Subependymal giant cell astrocytomas are almost exclusively diagnosed in patients with tuberous sclerosis under the age of 20 and are rarely found in adulthood. The incidence of SEGA in tuberous sclerosis complex (TSC) varies from 5% to 14%1-3. Please enable it to take advantage of the complete set of features! 1994;20:233–9. Case presentation: Goh S, Butler W, Thiele EA.  |  Subependymal giant cell astrocytoma (SEGA) is a slow-growing benign tumor most often seen in patients with tuberous sclerosis complex (TSC) with an incidence of nearly 15% in this patient population. 7 (4): 544-9. Astrocytes give rise to a number of tumors which the World Health Organization (WHO) has classified into the following entities: Pilocytic astrocytoma. They are principally diagnosed in patients under 20 years of age, only occasionally found in older individuals. Subependymal nodules are collections of abnormal, swollen glial cells and giant cells which cannot be differentiated as normal neural tissue. Methods: Sixty-four subependymal giant cell astrocytoma surgeries in 57 tuberous sclerosis complex patients with at least a 12-month follow-up were included in the study. Informa HealthCare. The best way to distinguish it from a subependymal giant cell astrocytoma is the size. Surgery. These lesions tend to calcify. Kaye AH, Laws ER. The tumor is characterized by slow growth and a benign biological behavior (WHO grade I). -, Sinson G, Sutton LN, Yachnis AT, Duhaime AC, Shut L. Subependymal giant cell astrocytomas in children. Diffuse astrocytoma (fibrillary, protoplasmic, and gemistocytic). Churchill Livingstone. A computed tomography scan revealed a voluminous mass in her perilateral ventricle. The prevalence rate of TSC in patients with SEGA ranges from 5% to 20%. A case of solitary subependymal giant cell astrocytoma: two somatic hits of TSC2 in the tumor, without evidence of somatic mosaicism. -, Roth J, Roach ES. Radiographics. A subset of subependymal giant cell astrocytoma-like astrocytomas are alternative lenghtening of telomere-positive and occur in the absence of ATRX alterations, thereby suggesting mutations in other DNA repair/maintenance genes may also facilitate alternative lenghtening of telomeres. Current evidence suggests that they are of a mixed neuronal and glial lineage, although they continue to be classified as astrocytomas 5. Subependymal Giant-cell Astrocytoma Masquerading as Restrictive Eating Disorder and Depression in an Adolescent. SEGA vs. SGTC: SEGA is most commonly known as the brain neoplasm associated with TSC (18, 24, 28).Historically, these tumors were considered to be astrocytomas. The main treatment is surgery, which is indicated if a tumour is symptomatic, or growth is demonstrated on MRI. Smith A, Smirniotopoulos J, Horkanyne-Szakaly I. Histologically subependymomas consistent of microcystic spaces and bland appearing cells without appreciable nuclear atypia or mitoses. Acta Neuropathol. e proximity to the right foramen of Monroe, its incomplete calci cation, enhancement on MRI, and large Subependymal giant cell astrocytomas are considered WHO grade I lesions in the current (2016) WHO classification of CNS tumours 8. Oral sirolimus has also been trialled 3. Subependymal giant cell astrocytomas are located at or near the foramen of Monroe and enlarge over time while remaining histologically benign and seen at 15 % of tuberous sclerosis cases.Anatomically,these tumors differ from the subependymal hamartomas by … USA.gov. The cells that appear astrocytic, usually resemble gemistocytes; large polygonal cells with prominent eosinophilic cytoplasm. Computed tomography scan revealing a voluminous mass in the perilateral ventricle with similar…, Tumor composed of spindle cells and globular large cells, producing an aspect of…, NLM In the clinical context of known tuberous sclerosis, the appearance is virtually pathognomonic, and the main differential is between a subependymal nodule and a subependymal giant cell astrocytomas. Gulf Professional Publishing. Radiology 183:227–238 PubMed Google Scholar. CONCLUSIONS: Subependymal giant cell astrocytoma is a rare tumor of the central nervous system whose diagnosis is based on clinical, radiological, histological and immunohistochemical arguments. Kalantari BN, Salamon N. Neuroimaging of tuberous sclerosis: spectrum of pathologic findings and frontiers in imaging. Histologically, subependymal nodules and subependymal giant cell tumours are essentially indistinguishable, and the distinction lies in the potential for growth and mass effect 5. 6. Subependymal giant cell astrocytoma (SEGA) is a clinically benign tumor that is usually associated with tuberous sclero-sis complex (TSC) ][1 . The prevalence rate of TSC in patients with SEGA ranges from 5% to 20%. Brain tumors, a comprehensive text. The tumour in the left hemisphere fulfilled the neuroradiological diagnostic criteria for a subependymal giant cell astrocytoma (SEGA). 2004;27:274–80. Surgery is the standard treatment for subependymal giant cell astrocytoma. The tumor size, age of the patients, mutation in the TSC1 or TSC2 gene, indication for the surgery, … 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.1 As SEGAs are distinct from astrocytomas, several authors have suggested using the term “subependymal giant cell tumor” instead.1, 3 SEGAs can present with increased intracranial … Subependymal giant cell astrocytomas (SGCAs or alternatively SEGAs) are benign tumours (WHO grade I), seen almost exclusively in young patients with tuberous sclerosis. When symptoms occur, they are usually a result of obstructive hydrocephalus because of mass effect around the ventricular system at the level of the interventricular foramen (of Monro). They frequently contain cysts and calcification 8. ADVERTISEMENT: Radiopaedia is free thanks to our supporters and advertisers. 2004;63 (8): 1457-61. Koeller KK, Sandberg GD. Solitary subependymal giant cell astrocytoma incidentally found at autopsy in an elderly woman without tuberous sclerosis complex Neuropathology, Vol. The dotted lines denote clinically relevant cutoffs of ≥30% and ≥50% reductions from baseline in primary SEGA volume. These tumors require routine surveillance with magnetic resonance imaging. Morantz RA, Walsh JW. An extemporaneous examination was in favor of a benign ganglioglioma tumor. Pediatr Neurol. From the archives of the AFIP. [A case of subependymal giant cell astrocytoma not associated with tuberous sclerosis]. Pediatr Neurol. Subependymal giant cell astrocytoma is a rare tumor of the central nervous system whose diagnosis is based on clinical, radiological, histological and immunohistochemical arguments. UPMC's neurosurgical team may recommend a combination of surgical and non-surgical approaches to treat subependymal giant cell astrocytomas. Subependymal Giant Cell Astrocytoma Treatment. Cerebral intraventricular neoplasms: radiologic-pathologic correlation. TSC is an autosomal dominantly in-herited neurocutaneous syndrome that affects any organ sys-tem of the body. The most common imaging finding are tubers, which are hamartomas along the subependymal surface and cortex. It is one of the intracranial lesions found in tuberous sclerosis complex (which include subependymal nodules, cortical tubers, retinal astrocytoma and subependymal giant cell astrocytoma), but cases without such lesions have also been reported in the literature. Subependymal giant cell tumours are often asymptomatic. 4. Check for errors and try again. Can not be differentiated as normal neural tissue by Vogt as part of typical. 2 ), Gadde JA ( 2 ), Palasis S ( 3 ) subependymomas consistent of spaces... J, Dahiya S, Tamiya T, Hikita T, Date,.:43-4. doi: 10.1007/BF00706748 SEGA volume, Takao S, Koenig MK, Weiner HL Franz... Astrocytoma ( SEGA ), Nishio S. 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