Materiały źródłowe

• #1 Embryonal tumours | The Brain Tumour Charity

  https://www.thebraintumourcharity.org/brain-tumour-diagnosis-treatment/types-brain-tumour-children/embryonal-tumours/

  Embryonal tumours are more common in children and young adults. About 20-25% of childhood brain tumours are embryonal tumours. […] Embryonal tumours occur most frequently in younger children and incidence decreases with age with more than half being diagnosed in children less than 10 years old. […] Nearly three-quarters (73%) of these tumours are medulloblastomas.

• #2

  https://braintumourresearch.org/pages/types-of-brain-tumours-embryonal-tumours?srsltid=AfmBOop8FQuL2sjPK42i3HUyvGZHT0YtnEjwcRSxRcd2WnXIiEazaWYW

  Embryonal tumours represent 20% to 25% of primary paediatric central nervous system (CNS) tumours, with medulloblastoma accounting for the majority of these tumours. […] Embryonal tumours are up to ten times more likely to be diagnosed in a child under 9 years old than they are to be diagnosed in an adult. […] The prognosis for each type of embryonal tumour is included in the relevant section above, but overall survival rates for embryonal tumours (not including medulloblastoma) are generally poor, ranging from less than 5% to 50% of patients surviving more than 5 years from diagnosis.

• #3 Medulloblastoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1181219-overview

  Epidemiology data is changing ever since we have a better understanding of molecular and genetic behaviors of these tumors and especially after the new World Health Organization (WHO) classification from 2016. […] Incidence of medulloblastoma is 1.5-2 cases per 100,000 population, with 350 new cases in the United States each year. […] Medulloblastoma accounts for 64.3% of all embryonal tumors in pediatric patients (0-19 years old), according to the Central Brain Tumor Registry of the United States (CBTRUS). […] Males displayed higher incidence rate relative to females (males: 0.16 vs. females: 0.12), except in patients 1 year-old. […] Overall ratio tend to be 1.5:1 for males. […] Among all age group, the reports from CBTRUS citing the embryonal tumor group together, with total incidence rate of 0.25 per 100,000 per year with slight male predominance (0.29 vs. 0.2).

• #4 Childhood Medulloblastoma and Other CNS Embryonal Tumors (PDQ®) – NCI

  https://www.cancer.gov/types/brain/hp/child-cns-embryonal-treatment-pdq

  Embryonal tumors account for approximately 20% of primary CNS tumors (malignant CNS neoplasms and pilocytic astrocytomas) arising in children. These tumors occur along the pediatric age spectrum but tend to cluster early in life. The incidence of embryonal tumors in children aged 1 to 9 years is fivefold to tenfold higher than in adults. […] Medulloblastomas comprise the vast majority of pediatric embryonal tumors. By definition, they arise in the posterior fossa, where they constitute approximately 40% of all posterior fossa tumors. Other forms of embryonal tumors each make up 2% or less of all childhood brain tumors. […] Imaging studies and CSF analysis are included in the diagnostic and staging evaluation. […] After surgery, lumbar CSF analysis is performed, if deemed safe. Neuroimaging and CSF evaluation are considered complementary because as many as 10% of patients have evidence of free-floating tumor cells in the CSF without clear evidence of leptomeningeal disease on MRI scan.

• #5 Childhood Medulloblastoma and Other Central Nervous System Embryonal Tumors Treatment (PDQ®): Treatment – Health Professional Information [NCI] – Health Information Library | PeaceHealth

  https://www.peacehealth.org/medical-topics/id/ncicdr0000548358

  Embryonal tumors account for 20% to 25% of primary CNS tumors (malignant brain tumors and pilocytic astrocytomas) arising in children. These tumors occur along the pediatric age spectrum but tend to cluster early in life. The incidence of embryonal tumors in children aged 1 to 9 years is fivefold to tenfold higher than in adults. […] Medulloblastomas comprise the vast majority of pediatric embryonal tumors. By definition, they arise in the posterior fossa, where they constitute approximately 40% of all posterior fossa tumors. Other forms of embryonal tumors each make up 2% or less of all childhood brain tumors. […] Given that therapies for pineoblastomas are quite similar to those for embryonal tumors, pineoblastomas are discussed in this summary. […] The pathological diagnosis of embryonal tumors is based primarily on histological and immunohistological microscopic features. However, molecular genetic studies are employed increasingly to subclassify embryonal tumors. These molecular genetic findings are also being used for risk stratification and treatment planning.

• #6 Medulloblastoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1181219-overview

  Epidemiology data is changing ever since we have a better understanding of molecular and genetic behaviors of these tumors and especially after the new World Health Organization (WHO) classification from 2016. […] Incidence of medulloblastoma is 1.5-2 cases per 100,000 population, with 350 new cases in the United States each year. […] Medulloblastoma accounts for 64.3% of all embryonal tumors in pediatric patients (0-19 years old), according to the Central Brain Tumor Registry of the United States (CBTRUS). […] Males displayed higher incidence rate relative to females (males: 0.16 vs. females: 0.12), except in patients 1 year-old. […] Overall ratio tend to be 1.5:1 for males. […] Among all age group, the reports from CBTRUS citing the embryonal tumor group together, with total incidence rate of 0.25 per 100,000 per year with slight male predominance (0.29 vs. 0.2).

• #7 Incidence, Trends, and Survival of Children With Embryonal Tumors – PubMed

  https://pubmed.ncbi.nlm.nih.gov/26304823/

  Central nervous system (CNS) and non-CNS embryonal tumors occur principally in children and are rarely seen in adults. The incidence rates for rare entities such as atypical teratoid/rhabdoid tumors (AT/RT) or primitive neuroectodermal tumors in the CNS are rarely published. Incidence rates for certain subgroups, such as hepatoblastomas, have been increasing in some countries. […] Data of 8337 embryonal tumors, registered in children (0-14 years) between 1991 and 2012 (for AT/RT 2000-2012) in the population-based German Childhood Cancer Registry with complete national coverage were analyzed for incidence rates, time trends, and survival. […] For most entities, the incidence rates were the highest for children 1 year. An important exception was medulloblastomas, which occurred mainly in 1- to 9-year-olds. Neuroblastomas and ganglioneuroblastomas as well as Wilms tumors (nephroblastomas) had the highest age standardized incidence rates (13.7 and 9.4 per million, respectively). A statistically significant increasing trend for hepatoblastomas (annual average percent change 4.6%) was detected. […] This study presents a comprehensive overview of pediatric embryonal tumors from a well-established, complete nationwide cancer registry. Significant increasing trend for hepatoblastomas was detected for the first time in Europe.

• #8 Medulloblastoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1962091-overview

  Epidemiology data is changing ever since we have a better understanding of molecular and genetic behaviors of these tumors and especially after the new World Health Organization (WHO) classification from 2016. […] Incidence of medulloblastoma is 1.5-2 cases per 100,000 population, with 350 new cases in the United States each year. […] Medulloblastoma accounts for 64.3% of all embryonal tumors in pediatric patients (0-19 years old), according to the Central Brain Tumor Registry of the United States (CBTRUS). […] Males displayed higher incidence rate relative to females (males: 0.16 vs. females: 0.12), except in patients 1 year-old. […] Overall ratio tend to be 1.5:1 for males. […] Among all age group, the reports from CBTRUS citing the embryonal tumor group together, with total incidence rate of 0.25 per 100,000 per year with slight male predominance (0.29 vs. 0.2).

• #9 Embryonal Tumor: What It Is, Symptoms, Treatment & Prognosis

  https://my.clevelandclinic.org/health/diseases/embryonal-tumors

  Embryonal tumors make up an estimated 12% of all brain tumors in children from birth to age 14 in the United States. Among these, the most common, medulloblastoma, accounts for over 68% of all embryonal tumors. Atypical teratoid/rhabdoid tumors account for 17%. […] These tumors are most common among babies and children. They can affect adults but its rare. For example, atypical teratoid/rhabdoid tumors affect more male children younger than 3 years old. Embryonal tumors with multilayered rosettes are more common among female children younger than 4 years old. […] Treatment for embryonal tumors varies based on the type, size and location of the tumor. Common treatment options may include: Removal surgery, Radiation therapy, Chemotherapy, Targeted therapy (medications). […] Your child’s prognosis varies depending on the following: Tumor type, Tumor location, Whether or not the cancer spreads, Age, Results of surgery or previous treatment, General health.

• #10 Rare brain embryonal tumors in infancy and early childhood | MedLink Neurology

  https://www.medlink.com/articles/rare-brain-embryonal-tumors-in-infancy-and-early-childhood

  Due to varied diagnostic practices and scarce clinical data, disease features and determinants of outcomes for these tumors are poorly defined. Moreover, the development of much-needed innovative therapies is warranted for rare pediatric embryonal tumors. […] ETMRs may likely represent a relevant proportion of the previous category CNS-PNET; however, there is a lack of data on the true incidence and prevalence of this rare entity due in part to the historical misdiagnoses for these tumors and the lack of molecular tools for its diagnostic confirmation until recent years. Based on the prospective German P-HIT Trial, Juhnke and colleagues estimated the incidence of ETMR to be 1.35 per 1 million children aged 1 to 4 years (41).

• #11 Embryonal tumour with multilayered rosettes – Wikipedia

  https://en.wikipedia.org/wiki/Embryonal_tumour_with_multilayered_rosettes

  Embryonal tumor with multilayered rosettes (ETMR) is an embryonal central nervous system tumor. […] It is a rare disease occurring in less than 1 in 700,000 children under the age of 4. […] The rate of ETMR is not correlated with sex, occurring equally in children of all sexes. […] It occurs more often in children under the age of three, and very rarely in children older than ten. […] Due to the rarity of the disease, there is no standardized treatment. […] Despite aggressive treatment, the prognosis is poor with 5-year overall survival rates less than 30%.

• #12 Embryonal tumors – Symptoms and causes – Mayo Clinic

  https://www.mayoclinic.org/diseases-conditions/embryonal-tumor/symptoms-causes/syc-20579617

  Embryonal tumors most often happen in babies and young children. But they can happen at any age. […] Risk factors for embryonal tumors include: […] Young age. This cancer can happen at any age. But it happens most often in children. […] Hereditary syndromes. Some conditions that run in families can raise the risk of embryonal tumor. Examples include Fanconi anemia, Li-Fraumeni syndrome, nevoid basal cell carcinoma syndrome, Rubinstein-Taybi syndrome and Turcot syndrome.

• #13 Embryonal tumors | UM Health-Sparrow

  https://www.uofmhealthsparrow.org/departments-conditions/conditions/embryonal-tumors

  Embryonal tumors most often happen in babies and young children. But they can happen at any age. […] Risk factors for embryonal tumors include: Young age. This cancer can happen at any age. But it happens most often in children. […] Your healthcare team reviews your child’s medical history and symptoms. Tests and procedures used to diagnose embryonal tumors include: […] Treatment for embryonal tumors usually involves surgery. Other treatments might be used after surgery to reduce the risk that the tumor may come back. Which treatments are best for your child depends on your child’s age. Your child’s healthcare team also considers the type of embryonal tumor and its location.

• #14 Embryonal tumours | The Brain Tumour Charity

  https://www.thebraintumourcharity.org/brain-tumour-diagnosis-treatment/types-brain-tumour-children/embryonal-tumours/

  Embryonal tumours are more common in children and young adults. About 20-25% of childhood brain tumours are embryonal tumours. […] Embryonal tumours occur most frequently in younger children and incidence decreases with age with more than half being diagnosed in children less than 10 years old. […] Nearly three-quarters (73%) of these tumours are medulloblastomas.

• #15 Rare brain embryonal tumors in infancy and early childhood | MedLink Neurology

  https://www.medlink.com/articles/rare-brain-embryonal-tumors-in-infancy-and-early-childhood

  Embryonal brain tumors account for approximately 13% of primary brain tumors of childhood, following gliomas as the second most common CNS tumor type in children up to 14 years of age (67; 72). […] The most recent Central Brain Tumor Registry of the United States (CBTRUS) statistical report estimated brain tumor incidence in children 0 to 4 years of age to be 6.18/100,000 population, which exceeds the incidence in children aged 4 to 14 years (5.5/10000) (72). […] Embryonal tumors represent 25% of brain tumors in infants younger than 1 year of age (73; 23). […] Rare embryonal tumors were previously classified as either medulloblastomas or supratentorial primitive neuroectodermal tumors sPNET, and they represent approximately 34% of all embryonal brain tumors of children and adolescents (73; 72).

• #16 Medulloblastoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1181219-overview

  Incidence of medulloblastoma decreases with age. […] Incidence was highest in patients aged 14 years at diagnosis, but patients aged 10-14 years showed increased incidence from 2000 to 2013, and when looking at all age groups the total incidence peaks at ages 9 years and below. […] When looking at CBTRUS and SEER databases covering roughly the same period of time (2000/2001 to 2013) adult patients (20 years of age and older) are about 28% from all medulloblastoma patients. […] Interestingly enough, for the adult group there was a significant rise in incidence rate between 2001 and 2009 with subsequent significant decline in the rate between 2009 and 2013. […] The 5-year and 10-year survival rates among all patients are 73% and 64.7%, respectively. […] Black patients displayed slightly lower survival rates for each year post diagnosis compared to white patients.

• #17 Incidence, Trends, and Survival of Children With Embryonal Tumors – PubMed

  https://pubmed.ncbi.nlm.nih.gov/26304823/

  Central nervous system (CNS) and non-CNS embryonal tumors occur principally in children and are rarely seen in adults. The incidence rates for rare entities such as atypical teratoid/rhabdoid tumors (AT/RT) or primitive neuroectodermal tumors in the CNS are rarely published. Incidence rates for certain subgroups, such as hepatoblastomas, have been increasing in some countries. […] Data of 8337 embryonal tumors, registered in children (0-14 years) between 1991 and 2012 (for AT/RT 2000-2012) in the population-based German Childhood Cancer Registry with complete national coverage were analyzed for incidence rates, time trends, and survival. […] For most entities, the incidence rates were the highest for children 1 year. An important exception was medulloblastomas, which occurred mainly in 1- to 9-year-olds. Neuroblastomas and ganglioneuroblastomas as well as Wilms tumors (nephroblastomas) had the highest age standardized incidence rates (13.7 and 9.4 per million, respectively). A statistically significant increasing trend for hepatoblastomas (annual average percent change 4.6%) was detected. […] This study presents a comprehensive overview of pediatric embryonal tumors from a well-established, complete nationwide cancer registry. Significant increasing trend for hepatoblastomas was detected for the first time in Europe.

• #18 Embryonal tumor with multilayered rosettes | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/embryonal-tumour-with-multilayered-rosettes?lang=us

  Embryonal tumor with multilayered rosettes occurs in children aged 4 years and under, mostly in children under 2 years, and is more common in girls, unlike the other CNS embryonal tumors, in which boys are equally or more commonly affected. […] Embryonal tumors with multilayered rosettes (ETMR) are rare small round blue cell tumor of the central nervous system. They are one of the most aggressive brain tumors usually encountered in children and are WHO grade 4 tumors.

• #19 CNS embryonal tumor with PLAGL amplification | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/cns-embryonal-tumour-with-plagl-amplification

  Embryonal tumor with PLAGL amplification primarily affects children and adolescents, with PLAGL1-amplified tumors occurring more commonly in school-aged children (median age: 10.5 years) and PLAGL2-amplified tumors in younger children (median age: 2 years). Some tumors have been identified in young adults. There is a female predominance among PLAGL1-amplified cases, whereas PLAGL2-amplified cases show a male predominance. […] No standardized treatment protocol exists, however, reported treatment strategies include maximal safe surgical resection, radiotherapy (craniospinal or local) and chemotherapy. Similarly, prognosis is difficult to fully characterize, however outcomes appear variable and perhaps dependent on which gene is amplified: PLAGL1-amplified tumors have a better prognosis, with a 5-year survival rate of 66%; PLAGL2-amplified tumors have a poorer prognosis, with a 5-year survival rate of 25%.

• #20 Medulloblastoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1181219-overview

  Epidemiology data is changing ever since we have a better understanding of molecular and genetic behaviors of these tumors and especially after the new World Health Organization (WHO) classification from 2016. […] Incidence of medulloblastoma is 1.5-2 cases per 100,000 population, with 350 new cases in the United States each year. […] Medulloblastoma accounts for 64.3% of all embryonal tumors in pediatric patients (0-19 years old), according to the Central Brain Tumor Registry of the United States (CBTRUS). […] Males displayed higher incidence rate relative to females (males: 0.16 vs. females: 0.12), except in patients 1 year-old. […] Overall ratio tend to be 1.5:1 for males. […] Among all age group, the reports from CBTRUS citing the embryonal tumor group together, with total incidence rate of 0.25 per 100,000 per year with slight male predominance (0.29 vs. 0.2).

• #21 Medulloblastoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1962091-overview

  Epidemiology data is changing ever since we have a better understanding of molecular and genetic behaviors of these tumors and especially after the new World Health Organization (WHO) classification from 2016. […] Incidence of medulloblastoma is 1.5-2 cases per 100,000 population, with 350 new cases in the United States each year. […] Medulloblastoma accounts for 64.3% of all embryonal tumors in pediatric patients (0-19 years old), according to the Central Brain Tumor Registry of the United States (CBTRUS). […] Males displayed higher incidence rate relative to females (males: 0.16 vs. females: 0.12), except in patients 1 year-old. […] Overall ratio tend to be 1.5:1 for males. […] Among all age group, the reports from CBTRUS citing the embryonal tumor group together, with total incidence rate of 0.25 per 100,000 per year with slight male predominance (0.29 vs. 0.2).

• #22 Embryonal tumor with multilayered rosettes | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/embryonal-tumour-with-multilayered-rosettes?lang=us

  Embryonal tumor with multilayered rosettes occurs in children aged 4 years and under, mostly in children under 2 years, and is more common in girls, unlike the other CNS embryonal tumors, in which boys are equally or more commonly affected. […] Embryonal tumors with multilayered rosettes (ETMR) are rare small round blue cell tumor of the central nervous system. They are one of the most aggressive brain tumors usually encountered in children and are WHO grade 4 tumors.

• #23 CNS embryonal tumor with PLAGL amplification | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/cns-embryonal-tumour-with-plagl-amplification

  Embryonal tumor with PLAGL amplification primarily affects children and adolescents, with PLAGL1-amplified tumors occurring more commonly in school-aged children (median age: 10.5 years) and PLAGL2-amplified tumors in younger children (median age: 2 years). Some tumors have been identified in young adults. There is a female predominance among PLAGL1-amplified cases, whereas PLAGL2-amplified cases show a male predominance. […] No standardized treatment protocol exists, however, reported treatment strategies include maximal safe surgical resection, radiotherapy (craniospinal or local) and chemotherapy. Similarly, prognosis is difficult to fully characterize, however outcomes appear variable and perhaps dependent on which gene is amplified: PLAGL1-amplified tumors have a better prognosis, with a 5-year survival rate of 66%; PLAGL2-amplified tumors have a poorer prognosis, with a 5-year survival rate of 25%.

• #24 Medulloblastoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1962091-overview

  In the collection of data from CBTRUS and SEER, white race was reported in the majority of cases (more than 80%). […] Yet, when comparing black population to white population for the years 2001 to 2013, blacks displayed a non-significant increase in incidence and in mortality risk. […] The 5-year and 10-year survival rates among all patients are 73% and 64.7%, respectively. […] Patients aged 14 years have lower survival rates for each year post diagnosis relative to patients aged 5-9, 10-14, and 20+ years up to 5 years post diagnosis. […] Survival rates for males and females are similar up to 10 years post diagnosis. […] Black patients displayed slightly lower survival rates for each year post diagnosis compared to white patients. […] Yet the reader needs to take into consideration that these survival numbers are from before the adjustment by molecular subtypes.

• #25 Race, ethnicity, and socioeconomic differences in incidence of pediatric embryonal tumors in the United States

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7674242/

  The etiology of childhood cancers and its social patterning remains largely unknown. Accounting for socioeconomic status (SES) when exploring the association between race/ethnicity and cancer incidence is necessary to better understand such etiology. We aimed to investigate differences in the incidence of embryonal tumors (ETs) by SES and race/ethnicity in the United States using population-based registries of the Surveillance, Epidemiology, and End Results (SEER) Program. […] The majority of tumors had lower incidence among non-white children compared to non-Hispanic (NH) white children, after controlling for SES. NH blacks had a higher incidence of Wilms tumor than NH whites (IRR: 1.26, 95% CI: 1.13, 1.39). There was an increasing linear trend (p=0.0001) across increasing SES quintile for embryonal rhabdomyosarcoma after controlling for race/ethnicity.

• #26 Race, ethnicity, and socioeconomic differences in incidence of pediatric embryonal tumors in the United States

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7674242/

  The etiology of childhood cancers and its social patterning remains largely unknown. Accounting for socioeconomic status (SES) when exploring the association between race/ethnicity and cancer incidence is necessary to better understand such etiology. We aimed to investigate differences in the incidence of embryonal tumors (ETs) by SES and race/ethnicity in the United States using population-based registries of the Surveillance, Epidemiology, and End Results (SEER) Program. […] The majority of tumors had lower incidence among non-white children compared to non-Hispanic (NH) white children, after controlling for SES. NH blacks had a higher incidence of Wilms tumor than NH whites (IRR: 1.26, 95% CI: 1.13, 1.39). There was an increasing linear trend (p=0.0001) across increasing SES quintile for embryonal rhabdomyosarcoma after controlling for race/ethnicity.

• #27 Medulloblastoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1962091-overview

  In the collection of data from CBTRUS and SEER, white race was reported in the majority of cases (more than 80%). […] Yet, when comparing black population to white population for the years 2001 to 2013, blacks displayed a non-significant increase in incidence and in mortality risk. […] The 5-year and 10-year survival rates among all patients are 73% and 64.7%, respectively. […] Patients aged 14 years have lower survival rates for each year post diagnosis relative to patients aged 5-9, 10-14, and 20+ years up to 5 years post diagnosis. […] Survival rates for males and females are similar up to 10 years post diagnosis. […] Black patients displayed slightly lower survival rates for each year post diagnosis compared to white patients. […] Yet the reader needs to take into consideration that these survival numbers are from before the adjustment by molecular subtypes.

• #28 Race, ethnicity, and socioeconomic differences in incidence of pediatric embryonal tumors in the United States

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7674242/

  Overall, patterns of incidence of neuroblastoma, retinoblastoma, Wilms, hepatoblastoma, and embryonal rhabdomyosarcoma in non-white children were consistent with those previously reported by Friedrich et al. However, we investigated PNETs and AT/RTs, which were not previously examined, and found lower rates among non-white children compared to white children. […] Interestingly, we identified significant, increasing trends in cancer incidence by SES for embryonal rhabdomyosarcoma and hepatoblastoma. When further investigated to determine whether SES interacts with race/ethnicity, we found a strong linear association between SES and rhabdomyosarcoma as well as Wilms tumor among Hispanics. […] In conclusion, results from this study suggest robust differences in the incidence of several embryonal tumors by race/ethnicity which, having been adjusted for SES, are potentially genetic in origin. Additionally, there is evidence that these differences may be modified by socioeconomic position. Future population-based studies with small-area-based indicators of SES are necessary to confirm these findings as well as to investigate potential risk factors that are socially patterned.

• #29 A Nationwide, Population-Based Epidemiology Study of Primary Central Nervous System Tumors in Korea, 2007-2016: A Comparison with United States Data

  https://www.e-crt.org/journal/view.php?number=3154

  The incidence rate of germ cell tumors among children in Korea was more than twice as high as that in the United States. […] The 5-year RSR of all PCNSTs was 86.4%, whereas that of all malignant tumors was 44.1%. Overall, female patients showed the more favorable survival outcome than male patients for malignant PCNSTs (5-year RSR, 45.8 vs. 42.6). […] The overall ASRs of PCNSTs in Korea and the United States were similar (ASR, 22.01 vs. 23.41); however, large geographical variations in the ASRs of PCNSTs have been reported. […] The relatively lower incidence rate of malignant PCNSTs was observed not only in east Asian countries, but also in Iran and western Asian countries. […] The proportion of non-malignant tumors among all PCNSTs was higher in Korea than in the United States (81% vs. 70%).

• #30 Epidemiology of primary brain tumors in childhood and adolescence in Kuwait | SpringerPlus | Full Text

  https://springerplus.springeropen.com/articles/10.1186/2193-1801-2-58

  The relatively high frequency of primary brain tumors (PBT) observed in childhood and adolescence in Kuwait has necessitated this epidemiological study. […] The most common tumors in childhood were astrocytoma (37%), embryonal tumors (31%), ependymoma (8%), and in adolescence astrocytoma (27%), pituitary adenoma (23%) and glioblastoma (13%). […] Embryonal tumors formed 44% of PBT in early childhood. […] In conclusion, despite the high relative frequency of PBT before the age of 20 years in Kuwait, its incidence rate is apparently low. […] Embryonal tumors are remarkably common in early childhood. […] The objective of this study is epidemiological and represents the first attempt at estimating incidence rates of PBT in children and adolescents in Kuwait and documenting tumor distribution according to age, sex, histological type and anatomical location.

• #31 Race, ethnicity, and socioeconomic differences in incidence of pediatric embryonal tumors in the United States

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7674242/

  The etiology of childhood cancers and its social patterning remains largely unknown. Accounting for socioeconomic status (SES) when exploring the association between race/ethnicity and cancer incidence is necessary to better understand such etiology. We aimed to investigate differences in the incidence of embryonal tumors (ETs) by SES and race/ethnicity in the United States using population-based registries of the Surveillance, Epidemiology, and End Results (SEER) Program. […] The majority of tumors had lower incidence among non-white children compared to non-Hispanic (NH) white children, after controlling for SES. NH blacks had a higher incidence of Wilms tumor than NH whites (IRR: 1.26, 95% CI: 1.13, 1.39). There was an increasing linear trend (p=0.0001) across increasing SES quintile for embryonal rhabdomyosarcoma after controlling for race/ethnicity.

• #32 Race, ethnicity, and socioeconomic differences in incidence of pediatric embryonal tumors in the United States

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7674242/

  Overall, patterns of incidence of neuroblastoma, retinoblastoma, Wilms, hepatoblastoma, and embryonal rhabdomyosarcoma in non-white children were consistent with those previously reported by Friedrich et al. However, we investigated PNETs and AT/RTs, which were not previously examined, and found lower rates among non-white children compared to white children. […] Interestingly, we identified significant, increasing trends in cancer incidence by SES for embryonal rhabdomyosarcoma and hepatoblastoma. When further investigated to determine whether SES interacts with race/ethnicity, we found a strong linear association between SES and rhabdomyosarcoma as well as Wilms tumor among Hispanics. […] In conclusion, results from this study suggest robust differences in the incidence of several embryonal tumors by race/ethnicity which, having been adjusted for SES, are potentially genetic in origin. Additionally, there is evidence that these differences may be modified by socioeconomic position. Future population-based studies with small-area-based indicators of SES are necessary to confirm these findings as well as to investigate potential risk factors that are socially patterned.

• #33 Race, ethnicity, and socioeconomic differences in incidence of pediatric embryonal tumors in the United States

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7674242/

  Overall, patterns of incidence of neuroblastoma, retinoblastoma, Wilms, hepatoblastoma, and embryonal rhabdomyosarcoma in non-white children were consistent with those previously reported by Friedrich et al. However, we investigated PNETs and AT/RTs, which were not previously examined, and found lower rates among non-white children compared to white children. […] Interestingly, we identified significant, increasing trends in cancer incidence by SES for embryonal rhabdomyosarcoma and hepatoblastoma. When further investigated to determine whether SES interacts with race/ethnicity, we found a strong linear association between SES and rhabdomyosarcoma as well as Wilms tumor among Hispanics. […] In conclusion, results from this study suggest robust differences in the incidence of several embryonal tumors by race/ethnicity which, having been adjusted for SES, are potentially genetic in origin. Additionally, there is evidence that these differences may be modified by socioeconomic position. Future population-based studies with small-area-based indicators of SES are necessary to confirm these findings as well as to investigate potential risk factors that are socially patterned.

• #34 Race, ethnicity, and socioeconomic differences in incidence of pediatric embryonal tumors in the United States

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7674242/

  Results from this study suggest differences in the incidence of several ETs by race/ethnicity and that these differences may be modified by SES. Investigation of potential risk factors that are socially patterned are warranted. […] Our study aimed to investigate the differences in the incidence rate of embryonal tumors by census tract-level SES and race/ethnicity, as well as to explore potential effect modification between these factors. […] To our knowledge, our study is the first to have found racial and ethnic differences in the associations between SES and incidence of several embryonal tumors among children in the United States. We found differences in the rates of many embryonal tumors comparing non-white to white children that are robust to adjustment to SES, therefore indicating they may be genetic in origin. Additionally, we found differences in trends of incidence by SES after controlling for race and ethnicity, suggesting potential environmental influences.

• #35 Incidence, Trends, and Survival of Children With Embryonal Tumors – PubMed

  https://pubmed.ncbi.nlm.nih.gov/26304823/

  Central nervous system (CNS) and non-CNS embryonal tumors occur principally in children and are rarely seen in adults. The incidence rates for rare entities such as atypical teratoid/rhabdoid tumors (AT/RT) or primitive neuroectodermal tumors in the CNS are rarely published. Incidence rates for certain subgroups, such as hepatoblastomas, have been increasing in some countries. […] Data of 8337 embryonal tumors, registered in children (0-14 years) between 1991 and 2012 (for AT/RT 2000-2012) in the population-based German Childhood Cancer Registry with complete national coverage were analyzed for incidence rates, time trends, and survival. […] For most entities, the incidence rates were the highest for children 1 year. An important exception was medulloblastomas, which occurred mainly in 1- to 9-year-olds. Neuroblastomas and ganglioneuroblastomas as well as Wilms tumors (nephroblastomas) had the highest age standardized incidence rates (13.7 and 9.4 per million, respectively). A statistically significant increasing trend for hepatoblastomas (annual average percent change 4.6%) was detected. […] This study presents a comprehensive overview of pediatric embryonal tumors from a well-established, complete nationwide cancer registry. Significant increasing trend for hepatoblastomas was detected for the first time in Europe.

• #36 Medulloblastoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1181219-overview

  Incidence of medulloblastoma decreases with age. […] Incidence was highest in patients aged 14 years at diagnosis, but patients aged 10-14 years showed increased incidence from 2000 to 2013, and when looking at all age groups the total incidence peaks at ages 9 years and below. […] When looking at CBTRUS and SEER databases covering roughly the same period of time (2000/2001 to 2013) adult patients (20 years of age and older) are about 28% from all medulloblastoma patients. […] Interestingly enough, for the adult group there was a significant rise in incidence rate between 2001 and 2009 with subsequent significant decline in the rate between 2009 and 2013. […] The 5-year and 10-year survival rates among all patients are 73% and 64.7%, respectively. […] Black patients displayed slightly lower survival rates for each year post diagnosis compared to white patients.

• #37 Medulloblastoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1181219-overview

  Incidence of medulloblastoma decreases with age. […] Incidence was highest in patients aged 14 years at diagnosis, but patients aged 10-14 years showed increased incidence from 2000 to 2013, and when looking at all age groups the total incidence peaks at ages 9 years and below. […] When looking at CBTRUS and SEER databases covering roughly the same period of time (2000/2001 to 2013) adult patients (20 years of age and older) are about 28% from all medulloblastoma patients. […] Interestingly enough, for the adult group there was a significant rise in incidence rate between 2001 and 2009 with subsequent significant decline in the rate between 2009 and 2013. […] The 5-year and 10-year survival rates among all patients are 73% and 64.7%, respectively. […] Black patients displayed slightly lower survival rates for each year post diagnosis compared to white patients.

• #38 Embryonal Tumor: What It Is, Symptoms, Treatment & Prognosis

  https://my.clevelandclinic.org/health/diseases/embryonal-tumors

  Embryonal tumors make up an estimated 12% of all brain tumors in children from birth to age 14 in the United States. Among these, the most common, medulloblastoma, accounts for over 68% of all embryonal tumors. Atypical teratoid/rhabdoid tumors account for 17%. […] These tumors are most common among babies and children. They can affect adults but its rare. For example, atypical teratoid/rhabdoid tumors affect more male children younger than 3 years old. Embryonal tumors with multilayered rosettes are more common among female children younger than 4 years old. […] Treatment for embryonal tumors varies based on the type, size and location of the tumor. Common treatment options may include: Removal surgery, Radiation therapy, Chemotherapy, Targeted therapy (medications). […] Your child’s prognosis varies depending on the following: Tumor type, Tumor location, Whether or not the cancer spreads, Age, Results of surgery or previous treatment, General health.

• #39 Medulloblastoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1962091-overview

  In the collection of data from CBTRUS and SEER, white race was reported in the majority of cases (more than 80%). […] Yet, when comparing black population to white population for the years 2001 to 2013, blacks displayed a non-significant increase in incidence and in mortality risk. […] The 5-year and 10-year survival rates among all patients are 73% and 64.7%, respectively. […] Patients aged 14 years have lower survival rates for each year post diagnosis relative to patients aged 5-9, 10-14, and 20+ years up to 5 years post diagnosis. […] Survival rates for males and females are similar up to 10 years post diagnosis. […] Black patients displayed slightly lower survival rates for each year post diagnosis compared to white patients. […] Yet the reader needs to take into consideration that these survival numbers are from before the adjustment by molecular subtypes.

• #40 Medulloblastoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1962091-overview

  When looking at the new classification (even before changing from 4 subtypes to 5), certain unsettled issues in epidemiology can become clearer. […] The group of infants 1 year of age has a much poorer prognosis. […] In previous works it was described that the age group of children less than 4 years old are divided mainly to SHH (more than 50%) and group 3 (~40%). […] Whereas SHH pathway-driven tumors usually lead to a fair survival rate of 75% in 5 years for children below 3 years of age, group 3 for the same age group is having significantly worse survival rates. […] This accounts for the discrepancy between the old survival rates in CBTRUS of about 48% for children 1 year old and 62% for children between 1 and 4 years of age, and the more positive picture that sometime can be seen in daily life.

• #41 Childhood Medulloblastoma and Other CNS Embryonal Tumors (PDQ®) – NCI

  https://www.cancer.gov/types/brain/hp/child-cns-embryonal-treatment-pdq

  Various clinical and biological parameters have been associated with the likelihood of disease control of embryonal tumors after treatment. […] It has become increasingly clear, especially for medulloblastomas, that outcome is also related to the molecular characteristics of the tumor, but this has not been definitively shown for other embryonal tumors. […] Prognosis is poor for patients with medulloepithelioma and ETMR, with 5-year survival rates ranging between 0% and 30%. […] Patients with disseminated CNS disease at diagnosis are at highest risk of disease relapse. […] Nonmedulloblastoma embryonal tumors and pineoblastomas may also be disseminated at the time of diagnosis, although the incidence may be somewhat less than for medulloblastomas, with dissemination at diagnosis in approximately 10% to 20% of patients.

• #42 Childhood Medulloblastoma and Other Central Nervous System Embryonal Tumors Treatment (PDQ®): Treatment – Health Professional Information [NCI] – Health Information Library | PeaceHealth

  https://www.peacehealth.org/medical-topics/id/ncicdr0000548358

  The 2021 WHO classification of embryonal tumors is as follows: Medulloblastoma, Other CNS embryonal tumors. […] Patients with disseminated CNS disease at diagnosis are at highest risk of disease relapse. […] Nonmedulloblastoma embryonal tumors and pineoblastomas may also be disseminated at the time of diagnosis, although the incidence of dissemination may be somewhat less than that of medulloblastomas, with dissemination at diagnosis being documented in approximately 10% to 20% of patients. […] The significance of many of these factors have been shown to be predictive for medulloblastomas, although some are used to assign risk, to some degree, for other embryonal tumors. […] Prognosis is poor for patients with medulloepithelioma and ETMR, with 5-year survival rates ranging between 0% and 30%.

• #43 Embryonal Tumor: What It Is, Symptoms, Treatment & Prognosis

  https://my.clevelandclinic.org/health/diseases/embryonal-tumors

  Some embryonal tumors are aggressive, spread easily and can return after treatment. Others may be benign (noncancerous). Your child’s healthcare provider will be able to give you the most accurate prognosis. […] The life expectancy for children with this condition varies. For example, one study reported that around 33% of children diagnosed with medulloblastoma don’t survive due to complications. The survival rate for an aggressive embryonal tumor with multilayered rosettes is 12 months after a diagnosis. […] Your child’s life expectancy may differ from others with the same type of tumor based on the tumor size and location. Your child’s healthcare providers will give you the most up-to-date information about treatment options and available care to increase your child’s chance of survival.

• #44

  https://braintumourresearch.org/pages/types-of-brain-tumours-embryonal-tumours?srsltid=AfmBOop8FQuL2sjPK42i3HUyvGZHT0YtnEjwcRSxRcd2WnXIiEazaWYW

  Embryonal tumours represent 20% to 25% of primary paediatric central nervous system (CNS) tumours, with medulloblastoma accounting for the majority of these tumours. […] Embryonal tumours are up to ten times more likely to be diagnosed in a child under 9 years old than they are to be diagnosed in an adult. […] The prognosis for each type of embryonal tumour is included in the relevant section above, but overall survival rates for embryonal tumours (not including medulloblastoma) are generally poor, ranging from less than 5% to 50% of patients surviving more than 5 years from diagnosis.

• #45 Childhood Medulloblastoma and Other CNS Embryonal Tumors (PDQ®) – NCI

  https://www.cancer.gov/types/brain/hp/child-cns-embryonal-treatment-pdq

  Various clinical and biological parameters have been associated with the likelihood of disease control of embryonal tumors after treatment. […] It has become increasingly clear, especially for medulloblastomas, that outcome is also related to the molecular characteristics of the tumor, but this has not been definitively shown for other embryonal tumors. […] Prognosis is poor for patients with medulloepithelioma and ETMR, with 5-year survival rates ranging between 0% and 30%. […] Patients with disseminated CNS disease at diagnosis are at highest risk of disease relapse. […] Nonmedulloblastoma embryonal tumors and pineoblastomas may also be disseminated at the time of diagnosis, although the incidence may be somewhat less than for medulloblastomas, with dissemination at diagnosis in approximately 10% to 20% of patients.

• #46 Childhood Medulloblastoma and Other Central Nervous System Embryonal Tumors Treatment (PDQ®): Treatment – Health Professional Information [NCI] – Health Information Library | PeaceHealth

  https://www.peacehealth.org/medical-topics/id/ncicdr0000548358

  Various clinical and biological parameters have been associated with the likelihood of disease control of embryonal tumors after treatment. […] The development of surveillance strategies other than imaging for patients with medulloblastoma is the subject of ongoing research. […] Surveillance imaging of the brain and spine is usually undertaken at routine intervals during and after treatment. […] The frequency of such imaging, designed to detect recurrent disease at an early, asymptomatic state, has been arbitrarily determined and has not been shown to clearly influence survival. […] Relapse in children with embryonal tumors is most likely to occur within the first 18 months of diagnosis. […] For most children, treatment is palliative, and disease control is transient in patients previously treated with radiation therapy and chemotherapy, with more than 80% of patients progressing within 2 years.

• #47 Medulloblastoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1962091-overview

  When looking at the new classification (even before changing from 4 subtypes to 5), certain unsettled issues in epidemiology can become clearer. […] The group of infants 1 year of age has a much poorer prognosis. […] In previous works it was described that the age group of children less than 4 years old are divided mainly to SHH (more than 50%) and group 3 (~40%). […] Whereas SHH pathway-driven tumors usually lead to a fair survival rate of 75% in 5 years for children below 3 years of age, group 3 for the same age group is having significantly worse survival rates. […] This accounts for the discrepancy between the old survival rates in CBTRUS of about 48% for children 1 year old and 62% for children between 1 and 4 years of age, and the more positive picture that sometime can be seen in daily life.

• #48 Childhood Medulloblastoma and Other CNS Embryonal Tumors (PDQ®) – NCI

  https://www.cancer.gov/types/brain/hp/child-cns-embryonal-treatment-pdq

  Various clinical and biological parameters have been associated with the likelihood of disease control of embryonal tumors after treatment. […] It has become increasingly clear, especially for medulloblastomas, that outcome is also related to the molecular characteristics of the tumor, but this has not been definitively shown for other embryonal tumors. […] Prognosis is poor for patients with medulloepithelioma and ETMR, with 5-year survival rates ranging between 0% and 30%. […] Patients with disseminated CNS disease at diagnosis are at highest risk of disease relapse. […] Nonmedulloblastoma embryonal tumors and pineoblastomas may also be disseminated at the time of diagnosis, although the incidence may be somewhat less than for medulloblastomas, with dissemination at diagnosis in approximately 10% to 20% of patients.

• #49 Childhood Medulloblastoma and Other Central Nervous System Embryonal Tumors Treatment (PDQ®): Treatment – Health Professional Information [NCI] – Health Information Library | PeaceHealth

  https://www.peacehealth.org/medical-topics/id/ncicdr0000548358

  The 2021 WHO classification of embryonal tumors is as follows: Medulloblastoma, Other CNS embryonal tumors. […] Patients with disseminated CNS disease at diagnosis are at highest risk of disease relapse. […] Nonmedulloblastoma embryonal tumors and pineoblastomas may also be disseminated at the time of diagnosis, although the incidence of dissemination may be somewhat less than that of medulloblastomas, with dissemination at diagnosis being documented in approximately 10% to 20% of patients. […] The significance of many of these factors have been shown to be predictive for medulloblastomas, although some are used to assign risk, to some degree, for other embryonal tumors. […] Prognosis is poor for patients with medulloepithelioma and ETMR, with 5-year survival rates ranging between 0% and 30%.

• #50 Medulloblastoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1962091-overview

  When looking at the new classification (even before changing from 4 subtypes to 5), certain unsettled issues in epidemiology can become clearer. […] The group of infants 1 year of age has a much poorer prognosis. […] In previous works it was described that the age group of children less than 4 years old are divided mainly to SHH (more than 50%) and group 3 (~40%). […] Whereas SHH pathway-driven tumors usually lead to a fair survival rate of 75% in 5 years for children below 3 years of age, group 3 for the same age group is having significantly worse survival rates. […] This accounts for the discrepancy between the old survival rates in CBTRUS of about 48% for children 1 year old and 62% for children between 1 and 4 years of age, and the more positive picture that sometime can be seen in daily life.

• #51 Childhood Medulloblastoma and Other CNS Embryonal Tumors (PDQ®) – NCI

  https://www.cancer.gov/types/brain/hp/child-cns-embryonal-treatment-pdq

  Various clinical and biological parameters have been associated with the likelihood of disease control of embryonal tumors after treatment. […] It has become increasingly clear, especially for medulloblastomas, that outcome is also related to the molecular characteristics of the tumor, but this has not been definitively shown for other embryonal tumors. […] Prognosis is poor for patients with medulloepithelioma and ETMR, with 5-year survival rates ranging between 0% and 30%. […] Patients with disseminated CNS disease at diagnosis are at highest risk of disease relapse. […] Nonmedulloblastoma embryonal tumors and pineoblastomas may also be disseminated at the time of diagnosis, although the incidence may be somewhat less than for medulloblastomas, with dissemination at diagnosis in approximately 10% to 20% of patients.

• #52 Childhood Medulloblastoma and Other Central Nervous System Embryonal Tumors Treatment (PDQ®): Treatment – Health Professional Information [NCI] – Health Information Library | PeaceHealth

  https://www.peacehealth.org/medical-topics/id/ncicdr0000548358

  Various clinical and biological parameters have been associated with the likelihood of disease control of embryonal tumors after treatment. […] The development of surveillance strategies other than imaging for patients with medulloblastoma is the subject of ongoing research. […] Surveillance imaging of the brain and spine is usually undertaken at routine intervals during and after treatment. […] The frequency of such imaging, designed to detect recurrent disease at an early, asymptomatic state, has been arbitrarily determined and has not been shown to clearly influence survival. […] Relapse in children with embryonal tumors is most likely to occur within the first 18 months of diagnosis. […] For most children, treatment is palliative, and disease control is transient in patients previously treated with radiation therapy and chemotherapy, with more than 80% of patients progressing within 2 years.

• #53 Added value of diffusion weighted imaging in pediatric central nervous system embryonal tumors surveillance | Oncotarget

  https://www.oncotarget.com/article/19553/text/

  The rationale behind surveillance imaging is that early detection of tumor recurrence may potentially allow for prompt intervention and thus improved outcome. […] However, screening for and diagnosing recurrent disease poses significant challenges. […] Our results show that DWI was able to pick up a significantly higher number of focal CNS ET relapses when compared to conventional MRI. […] Sensitivity for focal relapsing lesions raised from 77% of conventional MRI to 96% of DWI (p=0.0003). […] In conclusion, DWI represents a valuable diagnostic tool for the surveillance of embryonal tumors after treatment and can provide helpful information for clinical management of these patients.

• #54 Childhood Medulloblastoma and Other Central Nervous System Embryonal Tumors Treatment (PDQ®): Treatment – Health Professional Information [NCI] – Health Information Library | PeaceHealth

  https://www.peacehealth.org/medical-topics/id/ncicdr0000548358

  Various clinical and biological parameters have been associated with the likelihood of disease control of embryonal tumors after treatment. […] The development of surveillance strategies other than imaging for patients with medulloblastoma is the subject of ongoing research. […] Surveillance imaging of the brain and spine is usually undertaken at routine intervals during and after treatment. […] The frequency of such imaging, designed to detect recurrent disease at an early, asymptomatic state, has been arbitrarily determined and has not been shown to clearly influence survival. […] Relapse in children with embryonal tumors is most likely to occur within the first 18 months of diagnosis. […] For most children, treatment is palliative, and disease control is transient in patients previously treated with radiation therapy and chemotherapy, with more than 80% of patients progressing within 2 years.

• #55 Childhood Medulloblastoma and Other Central Nervous System Embryonal Tumors Treatment (PDQ®): Treatment – Health Professional Information [NCI] | Kaiser Permanente

  https://healthy.kaiserpermanente.org/health-wellness/health-encyclopedia/he.childhood-medulloblastoma-and-other-central-nervous-system-embryonal-tumors-treatment-pdq%C2%AE-treatment-health-professional-information-nci.ncicdr0000548358

  An accurate diagnosis is critical for patients with embryonal tumors. […] The significance of many of these factors have been shown to be predictive for medulloblastomas, although some are used to assign risk, to some degree, for other embryonal tumors. […] The development of surveillance strategies other than imaging for patients with medulloblastoma is the subject of ongoing research. […] Surveillance imaging of the brain and spine is usually undertaken at routine intervals during and after treatment. […] The frequency of such imaging, designed to detect recurrent disease at an early, asymptomatic state, has been arbitrarily determined and has not been shown to clearly influence survival. […] The optimal treatment of childhood nonmedulloblastoma embryonal tumors remains unclear and under study.

• #56 Childhood Medulloblastoma and Other CNS Embryonal Tumors (PDQ®) – NCI

  https://www.cancer.gov/types/brain/hp/child-cns-embryonal-treatment-pdq

  Embryonal tumors account for approximately 20% of primary CNS tumors (malignant CNS neoplasms and pilocytic astrocytomas) arising in children. These tumors occur along the pediatric age spectrum but tend to cluster early in life. The incidence of embryonal tumors in children aged 1 to 9 years is fivefold to tenfold higher than in adults. […] Medulloblastomas comprise the vast majority of pediatric embryonal tumors. By definition, they arise in the posterior fossa, where they constitute approximately 40% of all posterior fossa tumors. Other forms of embryonal tumors each make up 2% or less of all childhood brain tumors. […] Imaging studies and CSF analysis are included in the diagnostic and staging evaluation. […] After surgery, lumbar CSF analysis is performed, if deemed safe. Neuroimaging and CSF evaluation are considered complementary because as many as 10% of patients have evidence of free-floating tumor cells in the CSF without clear evidence of leptomeningeal disease on MRI scan.

• #57 Embryonal tumours | Brain and spinal cord tumours | Cancer Research UK

  https://www.cancerresearchuk.org/about-cancer/brain-tumours/types/embryonal-tumours

  Embryonal tumours are rare. Less than 1 out of every 100 brain tumours (less than 1%) diagnosed in England between 1995 and 2017 were embryonal tumours. They are more common in children than in adults. […] You have regular appointments with your doctor or nurse after treatment finishes. Your doctor examines you at each appointment. They ask how you are feeling, whether you have had any symptoms or side effects, and if you are worried about anything. […] Doctors are always trying to improve the diagnosis and treatment of brain tumours. As part of your treatment, your doctor might ask you to take part in a clinical trial. This might be to test a new treatment or look at different combinations of existing treatments.

• #58 Added value of diffusion weighted imaging in pediatric central nervous system embryonal tumors surveillance | Oncotarget

  https://www.oncotarget.com/article/19553/text/

  Diffusion weighted imaging (DWI) has an established role in primary CNS embryonal tumor (ET) characterization; however, its diagnostic utility in detecting relapse has never been determined. […] We aimed to compare DWI and conventional MRI sensitivity in CNS ET recurrence detection, and to evaluate the DWI properties of contrast-enhancing radiation induced lesions (RIL). […] Focal recurrence detection sensitivity of DWI (96%) was significantly higher than conventional MRI (77%) and CE-MRI alone (51%) (p=0.0003 and p0.0001). […] In conclusion, DWI is a valuable complementary technique allowing for improved detection of focal relapse in CNS ET patients, particularly in children with classic medulloblastoma, and may assist in differentiating recurrence from RIL. […] Standard surveillance for CNS ET includes neuroimaging and clinical evaluations at regular intervals.

• #59 Childhood Medulloblastoma and Other Central Nervous System Embryonal Tumors Treatment (PDQ®): Treatment – Health Professional Information [NCI] – Health Information Library | PeaceHealth

  https://www.peacehealth.org/medical-topics/id/ncicdr0000548358

  Various clinical and biological parameters have been associated with the likelihood of disease control of embryonal tumors after treatment. […] The development of surveillance strategies other than imaging for patients with medulloblastoma is the subject of ongoing research. […] Surveillance imaging of the brain and spine is usually undertaken at routine intervals during and after treatment. […] The frequency of such imaging, designed to detect recurrent disease at an early, asymptomatic state, has been arbitrarily determined and has not been shown to clearly influence survival. […] Relapse in children with embryonal tumors is most likely to occur within the first 18 months of diagnosis. […] For most children, treatment is palliative, and disease control is transient in patients previously treated with radiation therapy and chemotherapy, with more than 80% of patients progressing within 2 years.

• #60 Childhood Medulloblastoma and Other Central Nervous System Embryonal Tumors Treatment (PDQ®): Treatment – Health Professional Information [NCI] – Health Information Library | PeaceHealth

  https://www.peacehealth.org/medical-topics/id/ncicdr0000548358

  Various clinical and biological parameters have been associated with the likelihood of disease control of embryonal tumors after treatment. […] The development of surveillance strategies other than imaging for patients with medulloblastoma is the subject of ongoing research. […] Surveillance imaging of the brain and spine is usually undertaken at routine intervals during and after treatment. […] The frequency of such imaging, designed to detect recurrent disease at an early, asymptomatic state, has been arbitrarily determined and has not been shown to clearly influence survival. […] Relapse in children with embryonal tumors is most likely to occur within the first 18 months of diagnosis. […] For most children, treatment is palliative, and disease control is transient in patients previously treated with radiation therapy and chemotherapy, with more than 80% of patients progressing within 2 years.

• #61

  https://journals.lww.com/ijno/fulltext/2021/04001/diagnosis_and_management_of_central_nervous_system.28.aspx

  Subsequently, with the efforts by several research groups, understanding of the biology of embryonal tumors has exponentially grown, with the recognition of significant molecular heterogeneity within these embryonal tumors, resulting in the integration of molecular data in their classification in the WHO 2016 update classification of CNS tumors. […] Following the completion of planned treatment, it is essential to follow all patients periodically for early detection of relapse (recurrence/progression), if any, as well as timely identification and appropriate management of treatment-related morbidity. […] Medulloblastoma has a negative impact on health-related quality of life across the globe. […] Survivors often suffer from significant treatment-related toxicities from each of the individual modalities including surgery, RT, and chemotherapy. […] It is crucial to perform a detailed systemic examination with timely referral to appropriate healthcare specialists for management of such late effects.

• #62

  https://journals.lww.com/ijno/fulltext/2021/04001/diagnosis_and_management_of_central_nervous_system.28.aspx

  Subsequently, with the efforts by several research groups, understanding of the biology of embryonal tumors has exponentially grown, with the recognition of significant molecular heterogeneity within these embryonal tumors, resulting in the integration of molecular data in their classification in the WHO 2016 update classification of CNS tumors. […] Following the completion of planned treatment, it is essential to follow all patients periodically for early detection of relapse (recurrence/progression), if any, as well as timely identification and appropriate management of treatment-related morbidity. […] Medulloblastoma has a negative impact on health-related quality of life across the globe. […] Survivors often suffer from significant treatment-related toxicities from each of the individual modalities including surgery, RT, and chemotherapy. […] It is crucial to perform a detailed systemic examination with timely referral to appropriate healthcare specialists for management of such late effects.

• #63

  https://journals.lww.com/ijno/fulltext/2021/04001/diagnosis_and_management_of_central_nervous_system.28.aspx

  Central nervous system (CNS) embryonal tumors exhibit significant biological heterogeneity and pose challenges in diagnosis and clinical management. […] Advances in understanding the molecular alterations of these tumors, using genomic and epigenomic platforms, have led to refinement in their diagnosis, classification, and guiding clinical management. […] Our understanding of embryonal tumors has evolved enormously since the initial recognition of these tumors. However, what has remained constant is the fact that embryonal tumors pose a challenge in diagnosis as well as management and are generally associated with a poor prognosis, excepting for an favorable biology observed in localized medulloblastoma, which has an excellent long-term survival. […] The entity embryonal tumor with abundant neuropil and true rosettes (ETANTR) was described in this fascicle although not included in the classification.

• #64

  https://journals.lww.com/ijno/fulltext/2021/04001/diagnosis_and_management_of_central_nervous_system.28.aspx

  Subsequently, with the efforts by several research groups, understanding of the biology of embryonal tumors has exponentially grown, with the recognition of significant molecular heterogeneity within these embryonal tumors, resulting in the integration of molecular data in their classification in the WHO 2016 update classification of CNS tumors. […] Following the completion of planned treatment, it is essential to follow all patients periodically for early detection of relapse (recurrence/progression), if any, as well as timely identification and appropriate management of treatment-related morbidity. […] Medulloblastoma has a negative impact on health-related quality of life across the globe. […] Survivors often suffer from significant treatment-related toxicities from each of the individual modalities including surgery, RT, and chemotherapy. […] It is crucial to perform a detailed systemic examination with timely referral to appropriate healthcare specialists for management of such late effects.

• #65 Childhood Medulloblastoma and Other CNS Embryonal Tumors (PDQ®) – NCI

  https://www.cancer.gov/types/brain/hp/child-cns-embryonal-treatment-pdq

  The optimal treatment of childhood nonmedulloblastoma embryonal tumors remains unclear and under study. […] For more information about the treatment of CNS AT/RTs, see Childhood Central Nervous System Atypical Teratoid/Rhabdoid Tumor Treatment. […] The 2021 World Health Organization (WHO) Classification of Tumors of the Central Nervous System (CNS) is listed below in the Cellular and Molecular Classification section. All nonmedulloblastoma tumors of neuroectodermal origin that lack the specific histopathological features or molecular alterations that define other CNS tumors are classified as CNS embryonal tumors. […] The optimal treatment of patients with CNS neuroblastoma, FOXR2-activated tumors has not been confirmed by prospective studies.

• #66

  https://journals.lww.com/ijpm/fulltext/2022/65001/embryonal_tumors_in_the_who_cns5_classification__a.11.aspx

  Embryonal tumors are a heterogenous group of neoplasms mostly defined by recurrent genetic driver events. […] The importance of molecular characteristics in CNS embryonal tumors is well represented by the identification of different molecular groups and subgroups in medulloblastoma. […] The other embryonal tumors listed in WHO CNS5 are AT/RT; Embryonal tumor with multilayered rosettes (ETMR); CNS neuroblastoma, FOXR2- activated, and CNS tumor with BCOR internal tandem duplication (ITD). […] The most important change in CNS5 is the identification of three molecular sub-groups defined by DNA methylation and/or gene expression profiling. […] The inclusion in WHO CNS5 of CNS tumors with BCOR ITD as embryonal tumors, maybe provisional, in view of the fact that these neoplasms are not definitively neuroectodermal.

• #67 Medulloblastoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1962091-overview

  When looking at the new classification (even before changing from 4 subtypes to 5), certain unsettled issues in epidemiology can become clearer. […] The group of infants 1 year of age has a much poorer prognosis. […] In previous works it was described that the age group of children less than 4 years old are divided mainly to SHH (more than 50%) and group 3 (~40%). […] Whereas SHH pathway-driven tumors usually lead to a fair survival rate of 75% in 5 years for children below 3 years of age, group 3 for the same age group is having significantly worse survival rates. […] This accounts for the discrepancy between the old survival rates in CBTRUS of about 48% for children 1 year old and 62% for children between 1 and 4 years of age, and the more positive picture that sometime can be seen in daily life.

• #68

  https://journals.lww.com/ijpm/fulltext/2022/65001/embryonal_tumors_in_the_who_cns5_classification__a.11.aspx

  Embryonal tumors are a heterogenous group of neoplasms mostly defined by recurrent genetic driver events. […] The importance of molecular characteristics in CNS embryonal tumors is well represented by the identification of different molecular groups and subgroups in medulloblastoma. […] The other embryonal tumors listed in WHO CNS5 are AT/RT; Embryonal tumor with multilayered rosettes (ETMR); CNS neuroblastoma, FOXR2- activated, and CNS tumor with BCOR internal tandem duplication (ITD). […] The most important change in CNS5 is the identification of three molecular sub-groups defined by DNA methylation and/or gene expression profiling. […] The inclusion in WHO CNS5 of CNS tumors with BCOR ITD as embryonal tumors, maybe provisional, in view of the fact that these neoplasms are not definitively neuroectodermal.

• #69

  https://journals.lww.com/ijpm/fulltext/2022/65001/embryonal_tumors_in_the_who_cns5_classification__a.11.aspx

  Embryonal tumors are a heterogenous group of neoplasms mostly defined by recurrent genetic driver events. […] The importance of molecular characteristics in CNS embryonal tumors is well represented by the identification of different molecular groups and subgroups in medulloblastoma. […] The other embryonal tumors listed in WHO CNS5 are AT/RT; Embryonal tumor with multilayered rosettes (ETMR); CNS neuroblastoma, FOXR2- activated, and CNS tumor with BCOR internal tandem duplication (ITD). […] The most important change in CNS5 is the identification of three molecular sub-groups defined by DNA methylation and/or gene expression profiling. […] The inclusion in WHO CNS5 of CNS tumors with BCOR ITD as embryonal tumors, maybe provisional, in view of the fact that these neoplasms are not definitively neuroectodermal.

• #70 Incidence, Trends, and Survival of Children With Embryonal Tumors – PubMed

  https://pubmed.ncbi.nlm.nih.gov/26304823/

  Central nervous system (CNS) and non-CNS embryonal tumors occur principally in children and are rarely seen in adults. The incidence rates for rare entities such as atypical teratoid/rhabdoid tumors (AT/RT) or primitive neuroectodermal tumors in the CNS are rarely published. Incidence rates for certain subgroups, such as hepatoblastomas, have been increasing in some countries. […] Data of 8337 embryonal tumors, registered in children (0-14 years) between 1991 and 2012 (for AT/RT 2000-2012) in the population-based German Childhood Cancer Registry with complete national coverage were analyzed for incidence rates, time trends, and survival. […] For most entities, the incidence rates were the highest for children 1 year. An important exception was medulloblastomas, which occurred mainly in 1- to 9-year-olds. Neuroblastomas and ganglioneuroblastomas as well as Wilms tumors (nephroblastomas) had the highest age standardized incidence rates (13.7 and 9.4 per million, respectively). A statistically significant increasing trend for hepatoblastomas (annual average percent change 4.6%) was detected. […] This study presents a comprehensive overview of pediatric embryonal tumors from a well-established, complete nationwide cancer registry. Significant increasing trend for hepatoblastomas was detected for the first time in Europe.

• #71 Medulloblastoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1962091-overview

  Epidemiology data is changing ever since we have a better understanding of molecular and genetic behaviors of these tumors and especially after the new World Health Organization (WHO) classification from 2016. […] Incidence of medulloblastoma is 1.5-2 cases per 100,000 population, with 350 new cases in the United States each year. […] Medulloblastoma accounts for 64.3% of all embryonal tumors in pediatric patients (0-19 years old), according to the Central Brain Tumor Registry of the United States (CBTRUS). […] Males displayed higher incidence rate relative to females (males: 0.16 vs. females: 0.12), except in patients 1 year-old. […] Overall ratio tend to be 1.5:1 for males. […] Among all age group, the reports from CBTRUS citing the embryonal tumor group together, with total incidence rate of 0.25 per 100,000 per year with slight male predominance (0.29 vs. 0.2).

• #72 Race, ethnicity, and socioeconomic differences in incidence of pediatric embryonal tumors in the United States

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7674242/

  The etiology of childhood cancers and its social patterning remains largely unknown. Accounting for socioeconomic status (SES) when exploring the association between race/ethnicity and cancer incidence is necessary to better understand such etiology. We aimed to investigate differences in the incidence of embryonal tumors (ETs) by SES and race/ethnicity in the United States using population-based registries of the Surveillance, Epidemiology, and End Results (SEER) Program. […] The majority of tumors had lower incidence among non-white children compared to non-Hispanic (NH) white children, after controlling for SES. NH blacks had a higher incidence of Wilms tumor than NH whites (IRR: 1.26, 95% CI: 1.13, 1.39). There was an increasing linear trend (p=0.0001) across increasing SES quintile for embryonal rhabdomyosarcoma after controlling for race/ethnicity.

• #73 Incidence, Trends, and Survival of Children With Embryonal Tumors – PubMed

  https://pubmed.ncbi.nlm.nih.gov/26304823/

  Central nervous system (CNS) and non-CNS embryonal tumors occur principally in children and are rarely seen in adults. The incidence rates for rare entities such as atypical teratoid/rhabdoid tumors (AT/RT) or primitive neuroectodermal tumors in the CNS are rarely published. Incidence rates for certain subgroups, such as hepatoblastomas, have been increasing in some countries. […] Data of 8337 embryonal tumors, registered in children (0-14 years) between 1991 and 2012 (for AT/RT 2000-2012) in the population-based German Childhood Cancer Registry with complete national coverage were analyzed for incidence rates, time trends, and survival. […] For most entities, the incidence rates were the highest for children 1 year. An important exception was medulloblastomas, which occurred mainly in 1- to 9-year-olds. Neuroblastomas and ganglioneuroblastomas as well as Wilms tumors (nephroblastomas) had the highest age standardized incidence rates (13.7 and 9.4 per million, respectively). A statistically significant increasing trend for hepatoblastomas (annual average percent change 4.6%) was detected. […] This study presents a comprehensive overview of pediatric embryonal tumors from a well-established, complete nationwide cancer registry. Significant increasing trend for hepatoblastomas was detected for the first time in Europe.

• #74 A Nationwide, Population-Based Epidemiology Study of Primary Central Nervous System Tumors in Korea, 2007-2016: A Comparison with United States Data

  https://www.e-crt.org/journal/view.php?number=3154

  The purpose of this study was to determine the epidemiologic characteristics and survival of patients with primary brain and other central nervous system (CNS) tumors in Korea and to compare our findings with those from the United States. […] We collected data on primary brain and CNS tumors diagnosed between 2007 and 2016 from the Korea Central Cancer Registry. The age-standardized incidence rates (ASRs) and 5-year relative survival rates (RSRs) were evaluated. […] A total of 115,050 primary brain and CNS tumors were identified, and the ASR of all tumors was 22.01 per 100,000 individuals, which was lower than the 23.41 in the United States. However, the ASR of malignant tumors was significantly lower herein (4.27) than in the United States (7.08). […] In Korea, malignant brain and other CNS tumors have a lower incidence and better survival outcome.

• #75 :: BTRT :: Brain Tumor Research and Treatment

  https://btrt.org/DOIx.php?id=10.14791/btrt.2017.5.1.16

  The incidence of meningiomas was over 3.5 times higher in females than in males. […] The overall incidence according to biological behavior is shown in Fig. 1. Tumors classified as benign, uncertain, and malignant tumors accounted for 73.2%, 8.8%, and 18.0% of all primary CNS tumors, respectively. […] The incidence according to the originating site is shown in Fig. 2. Meninges (35.2%) were the most common site of primary brain tumors, followed by the brain parenchyma (29.0%), sellar region (21.1%), and cranial and spinal nerves (14.7%). […] Children numbering 578 under 19 years of age were diagnosed with brain tumors in 2013, and crude rates of primary brain tumors in children was 5.27 per 100,000 person-years. […] The most common histology in children included neuroepithelial tumors, germ cell tumors, and tumors of the sellar region. Neuroepithelial tumors accounted for 38.6% of all tumors in children. Embryonal/primitive/medulloblastoma was the most common tumor among neuroepithelial tumors in children. […] In conclusion, we updated the nationwide statistics of brain and CNS tumors in the Republic of Korea. Updated incidence of brain tumors will help to assess disease burden, facilitate etiologic studies, and establish cancer prevention and control strategies.

• #76 A Nationwide, Population-Based Epidemiology Study of Primary Central Nervous System Tumors in Korea, 2007-2016: A Comparison with United States Data

  https://www.e-crt.org/journal/view.php?number=3154

  The purpose of this study was to determine the epidemiologic characteristics and survival of patients with primary brain and other central nervous system (CNS) tumors in Korea and to compare our findings with those from the United States. […] We collected data on primary brain and CNS tumors diagnosed between 2007 and 2016 from the Korea Central Cancer Registry. The age-standardized incidence rates (ASRs) and 5-year relative survival rates (RSRs) were evaluated. […] A total of 115,050 primary brain and CNS tumors were identified, and the ASR of all tumors was 22.01 per 100,000 individuals, which was lower than the 23.41 in the United States. However, the ASR of malignant tumors was significantly lower herein (4.27) than in the United States (7.08). […] In Korea, malignant brain and other CNS tumors have a lower incidence and better survival outcome.

• #77 Rare brain embryonal tumors in infancy and early childhood | MedLink Neurology

  https://www.medlink.com/articles/rare-brain-embryonal-tumors-in-infancy-and-early-childhood

  Due to varied diagnostic practices and scarce clinical data, disease features and determinants of outcomes for these tumors are poorly defined. Moreover, the development of much-needed innovative therapies is warranted for rare pediatric embryonal tumors. […] ETMRs may likely represent a relevant proportion of the previous category CNS-PNET; however, there is a lack of data on the true incidence and prevalence of this rare entity due in part to the historical misdiagnoses for these tumors and the lack of molecular tools for its diagnostic confirmation until recent years. Based on the prospective German P-HIT Trial, Juhnke and colleagues estimated the incidence of ETMR to be 1.35 per 1 million children aged 1 to 4 years (41).

• #78

  https://journals.lww.com/ijno/fulltext/2021/04001/diagnosis_and_management_of_central_nervous_system.28.aspx

  Central nervous system (CNS) embryonal tumors exhibit significant biological heterogeneity and pose challenges in diagnosis and clinical management. […] Advances in understanding the molecular alterations of these tumors, using genomic and epigenomic platforms, have led to refinement in their diagnosis, classification, and guiding clinical management. […] Our understanding of embryonal tumors has evolved enormously since the initial recognition of these tumors. However, what has remained constant is the fact that embryonal tumors pose a challenge in diagnosis as well as management and are generally associated with a poor prognosis, excepting for an favorable biology observed in localized medulloblastoma, which has an excellent long-term survival. […] The entity embryonal tumor with abundant neuropil and true rosettes (ETANTR) was described in this fascicle although not included in the classification.

• #79 Medulloblastoma: Practice Essentials, Background, Pathophysiology

  https://emedicine.medscape.com/article/1962091-overview

  Epidemiology data is changing ever since we have a better understanding of molecular and genetic behaviors of these tumors and especially after the new World Health Organization (WHO) classification from 2016. […] Incidence of medulloblastoma is 1.5-2 cases per 100,000 population, with 350 new cases in the United States each year. […] Medulloblastoma accounts for 64.3% of all embryonal tumors in pediatric patients (0-19 years old), according to the Central Brain Tumor Registry of the United States (CBTRUS). […] Males displayed higher incidence rate relative to females (males: 0.16 vs. females: 0.12), except in patients 1 year-old. […] Overall ratio tend to be 1.5:1 for males. […] Among all age group, the reports from CBTRUS citing the embryonal tumor group together, with total incidence rate of 0.25 per 100,000 per year with slight male predominance (0.29 vs. 0.2).

• #80

  https://journals.lww.com/ijpm/fulltext/2022/65001/embryonal_tumors_in_the_who_cns5_classification__a.11.aspx

  With this term, WHO CNS5 defines a CNS tumor with embryonal histology and immunophenotype in which no alteration that would classify it as one of the molecularly defined CNS embryonal tumors can be detected or cases that, for any reason, are not susceptible to analysis. […] For nonmedulloblastoma tumors, a useful approach could be to start using three simple antibodies (synaptophysin, vimentin, and Olig2). […] On one hand, the discovery of numerous molecular subgroups opens the possibility to the development of more specific target therapies, however, clinical trials on a small group of patients would not provide significant results.

• #81 Rare brain embryonal tumors in infancy and early childhood | MedLink Neurology

  https://www.medlink.com/articles/rare-brain-embryonal-tumors-in-infancy-and-early-childhood

  Due to varied diagnostic practices and scarce clinical data, disease features and determinants of outcomes for these tumors are poorly defined. Moreover, the development of much-needed innovative therapies is warranted for rare pediatric embryonal tumors. […] ETMRs may likely represent a relevant proportion of the previous category CNS-PNET; however, there is a lack of data on the true incidence and prevalence of this rare entity due in part to the historical misdiagnoses for these tumors and the lack of molecular tools for its diagnostic confirmation until recent years. Based on the prospective German P-HIT Trial, Juhnke and colleagues estimated the incidence of ETMR to be 1.35 per 1 million children aged 1 to 4 years (41).

• #82

  https://journals.lww.com/ijno/fulltext/2021/04001/diagnosis_and_management_of_central_nervous_system.28.aspx

  Central nervous system (CNS) embryonal tumors exhibit significant biological heterogeneity and pose challenges in diagnosis and clinical management. […] Advances in understanding the molecular alterations of these tumors, using genomic and epigenomic platforms, have led to refinement in their diagnosis, classification, and guiding clinical management. […] Our understanding of embryonal tumors has evolved enormously since the initial recognition of these tumors. However, what has remained constant is the fact that embryonal tumors pose a challenge in diagnosis as well as management and are generally associated with a poor prognosis, excepting for an favorable biology observed in localized medulloblastoma, which has an excellent long-term survival. […] The entity embryonal tumor with abundant neuropil and true rosettes (ETANTR) was described in this fascicle although not included in the classification.

• #83

  https://journals.lww.com/ijpm/fulltext/2022/65001/embryonal_tumors_in_the_who_cns5_classification__a.11.aspx

  With this term, WHO CNS5 defines a CNS tumor with embryonal histology and immunophenotype in which no alteration that would classify it as one of the molecularly defined CNS embryonal tumors can be detected or cases that, for any reason, are not susceptible to analysis. […] For nonmedulloblastoma tumors, a useful approach could be to start using three simple antibodies (synaptophysin, vimentin, and Olig2). […] On one hand, the discovery of numerous molecular subgroups opens the possibility to the development of more specific target therapies, however, clinical trials on a small group of patients would not provide significant results.

• #84 Rare brain embryonal tumors in infancy and early childhood | MedLink Neurology

  https://www.medlink.com/articles/rare-brain-embryonal-tumors-in-infancy-and-early-childhood

  Due to varied diagnostic practices and scarce clinical data, disease features and determinants of outcomes for these tumors are poorly defined. Moreover, the development of much-needed innovative therapies is warranted for rare pediatric embryonal tumors. […] ETMRs may likely represent a relevant proportion of the previous category CNS-PNET; however, there is a lack of data on the true incidence and prevalence of this rare entity due in part to the historical misdiagnoses for these tumors and the lack of molecular tools for its diagnostic confirmation until recent years. Based on the prospective German P-HIT Trial, Juhnke and colleagues estimated the incidence of ETMR to be 1.35 per 1 million children aged 1 to 4 years (41).

• #85

  https://journals.lww.com/ijno/fulltext/2021/04001/diagnosis_and_management_of_central_nervous_system.28.aspx

  Central nervous system (CNS) embryonal tumors exhibit significant biological heterogeneity and pose challenges in diagnosis and clinical management. […] Advances in understanding the molecular alterations of these tumors, using genomic and epigenomic platforms, have led to refinement in their diagnosis, classification, and guiding clinical management. […] Our understanding of embryonal tumors has evolved enormously since the initial recognition of these tumors. However, what has remained constant is the fact that embryonal tumors pose a challenge in diagnosis as well as management and are generally associated with a poor prognosis, excepting for an favorable biology observed in localized medulloblastoma, which has an excellent long-term survival. […] The entity embryonal tumor with abundant neuropil and true rosettes (ETANTR) was described in this fascicle although not included in the classification.

• #86

  https://journals.lww.com/ijpm/fulltext/2022/65001/embryonal_tumors_in_the_who_cns5_classification__a.11.aspx

  Embryonal tumors are a heterogenous group of neoplasms mostly defined by recurrent genetic driver events. […] The importance of molecular characteristics in CNS embryonal tumors is well represented by the identification of different molecular groups and subgroups in medulloblastoma. […] The other embryonal tumors listed in WHO CNS5 are AT/RT; Embryonal tumor with multilayered rosettes (ETMR); CNS neuroblastoma, FOXR2- activated, and CNS tumor with BCOR internal tandem duplication (ITD). […] The most important change in CNS5 is the identification of three molecular sub-groups defined by DNA methylation and/or gene expression profiling. […] The inclusion in WHO CNS5 of CNS tumors with BCOR ITD as embryonal tumors, maybe provisional, in view of the fact that these neoplasms are not definitively neuroectodermal.

• #87

  https://journals.lww.com/ijpm/fulltext/2022/65001/embryonal_tumors_in_the_who_cns5_classification__a.11.aspx

  With this term, WHO CNS5 defines a CNS tumor with embryonal histology and immunophenotype in which no alteration that would classify it as one of the molecularly defined CNS embryonal tumors can be detected or cases that, for any reason, are not susceptible to analysis. […] For nonmedulloblastoma tumors, a useful approach could be to start using three simple antibodies (synaptophysin, vimentin, and Olig2). […] On one hand, the discovery of numerous molecular subgroups opens the possibility to the development of more specific target therapies, however, clinical trials on a small group of patients would not provide significant results.

• #88 Race, ethnicity, and socioeconomic differences in incidence of pediatric embryonal tumors in the United States

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7674242/

  Overall, patterns of incidence of neuroblastoma, retinoblastoma, Wilms, hepatoblastoma, and embryonal rhabdomyosarcoma in non-white children were consistent with those previously reported by Friedrich et al. However, we investigated PNETs and AT/RTs, which were not previously examined, and found lower rates among non-white children compared to white children. […] Interestingly, we identified significant, increasing trends in cancer incidence by SES for embryonal rhabdomyosarcoma and hepatoblastoma. When further investigated to determine whether SES interacts with race/ethnicity, we found a strong linear association between SES and rhabdomyosarcoma as well as Wilms tumor among Hispanics. […] In conclusion, results from this study suggest robust differences in the incidence of several embryonal tumors by race/ethnicity which, having been adjusted for SES, are potentially genetic in origin. Additionally, there is evidence that these differences may be modified by socioeconomic position. Future population-based studies with small-area-based indicators of SES are necessary to confirm these findings as well as to investigate potential risk factors that are socially patterned.

• #89

  https://journals.lww.com/ijpm/fulltext/2022/65001/embryonal_tumors_in_the_who_cns5_classification__a.11.aspx

  With this term, WHO CNS5 defines a CNS tumor with embryonal histology and immunophenotype in which no alteration that would classify it as one of the molecularly defined CNS embryonal tumors can be detected or cases that, for any reason, are not susceptible to analysis. […] For nonmedulloblastoma tumors, a useful approach could be to start using three simple antibodies (synaptophysin, vimentin, and Olig2). […] On one hand, the discovery of numerous molecular subgroups opens the possibility to the development of more specific target therapies, however, clinical trials on a small group of patients would not provide significant results.

• #90 Race, ethnicity, and socioeconomic differences in incidence of pediatric embryonal tumors in the United States

  https://pmc.ncbi.nlm.nih.gov/articles/PMC7674242/

  Overall, patterns of incidence of neuroblastoma, retinoblastoma, Wilms, hepatoblastoma, and embryonal rhabdomyosarcoma in non-white children were consistent with those previously reported by Friedrich et al. However, we investigated PNETs and AT/RTs, which were not previously examined, and found lower rates among non-white children compared to white children. […] Interestingly, we identified significant, increasing trends in cancer incidence by SES for embryonal rhabdomyosarcoma and hepatoblastoma. When further investigated to determine whether SES interacts with race/ethnicity, we found a strong linear association between SES and rhabdomyosarcoma as well as Wilms tumor among Hispanics. […] In conclusion, results from this study suggest robust differences in the incidence of several embryonal tumors by race/ethnicity which, having been adjusted for SES, are potentially genetic in origin. Additionally, there is evidence that these differences may be modified by socioeconomic position. Future population-based studies with small-area-based indicators of SES are necessary to confirm these findings as well as to investigate potential risk factors that are socially patterned.

• #91 Added value of diffusion weighted imaging in pediatric central nervous system embryonal tumors surveillance | Oncotarget

  https://www.oncotarget.com/article/19553/text/

  The rationale behind surveillance imaging is that early detection of tumor recurrence may potentially allow for prompt intervention and thus improved outcome. […] However, screening for and diagnosing recurrent disease poses significant challenges. […] Our results show that DWI was able to pick up a significantly higher number of focal CNS ET relapses when compared to conventional MRI. […] Sensitivity for focal relapsing lesions raised from 77% of conventional MRI to 96% of DWI (p=0.0003). […] In conclusion, DWI represents a valuable diagnostic tool for the surveillance of embryonal tumors after treatment and can provide helpful information for clinical management of these patients.

• #92 Incidence of childhood CNS tumours in Britain and variation in rates by definition of malignant behaviour: population-based study | BMC Cancer | Full Text

  https://bmccancer.biomedcentral.com/articles/10.1186/s12885-019-5344-7

  Age-standardised incidence in 2001-10 was 40.1 per million. Astrocytomas accounted for 41%, embryonal tumours for 17%, other gliomas for 10%, ependymomas for 7%, rarer subtypes for 20% and unspecified tumours for 5%. […] Embryonal tumours accounted for 17% of registrations. Within this subgroup, 73% were medulloblastoma, 15% were embryonal CNS tumour NOS and 11% were atypical teratoid/rhabdoid tumours (ATRT). […] Total incidence of childhood CNS tumours in Britain in the first decade of the new millennium was fairly similar to that in other large western countries. Deficits of some, predominantly low-grade, tumours or differences in their age distribution compared with the United States and the Nordic countries are compatible with delayed diagnosis in Britain. Complete registration of all types of CNS tumour, regardless of behaviour, is essential for assessing burden of disease and changes over time in incidence and survival. This is particularly important for pilocytic astrocytoma, the most common of all childhood CNS tumours, because of the recent change in its behaviour code from malignant to non-malignant and changes over time in the proportion of astrocytomas for which subtype has been specified.

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