Materiały źródłowe

• #1 Medulloblastoma – StatPearls – NCBI Bookshelf

  https://www.ncbi.nlm.nih.gov/books/NBK431069/

  Medulloblastoma is the most common malignant brain tumor in children, constituting nearly 20 percent of all pediatric brain tumors. […] Examining data from the Surveillance, Epidemiology, and End-Results (SEER) database from 1973 through 2007, the annual incidence for medulloblastoma was reported at six per million children, in other words, approximately 450 new pediatric cases per year. Children age 4 to 9 years old had the highest incidence at 44%, followed by adolescents (10 to 16 years old) at 23%, and only a 12% incidence in infants/toddlers (0 to 3 years old). Pediatric incidence was calculated to be ten-fold higher than adult incidence. Medulloblastoma affected males 1.5 times more than females.

• #2 Medulloblastoma | Treatment & Management | Point of Care

  https://www.statpearls.com/point-of-care/24908

  Medulloblastoma is the most common malignant brain tumor in children constituting nearly 20% of all pediatric brain tumors. […] Examining data from the Surveillance, Epidemiology, and End-Results (SEER) database from 1973 through 2007, the annual incidence for medulloblastoma was reported at six per million children, in other words, approximately 450 new pediatric cases per year. Children age 4 to 9 years old had the highest incidence at 44%, followed by adolescents (10 to 16 years old) at 23%, and only a 12% incidence in infants/toddlers (0 to 3 years old). Pediatric incidence was calculated to be ten-fold higher than adult incidence. Medulloblastoma affected males 1.5 times more than females.

• #3 Medulloblastoma Statistics | medulloblastoma.org

  https://medulloblastoma.org/medulloblastoma-statistics/

  Brain tumors are now the most common cancer and cause of cancer-related death in children less than 15 years of age. […] Children comprise 70% of all medulloblastoma cases. […] Approximately 435 patients are diagnosed each year in the US, with more than 70% of them being pediatric. […] Medulloblastoma accounts for approximately 20% of all childhood brain tumors and 63% of intracranial embryonic tumors. These tumors can arise throughout childhood and into adulthood with an overall annual incidence of approximately 5 cases per 1 million in the pediatric population. […] 75% of medulloblastomas occur in children under the age of 10, with incidence peaking in children 14 and 59 years of age. […] Medulloblastoma is more common in males, affecting approximately 1.7 times more males than females, although sex predilection as well as age-specific incidence vary by subgroup. […] Medulloblastoma is far more rare in adulthood, with a reported incidence of 0.05 cases per 100,000 population.

• #4 Medulloblastoma | MedLink Neurology

  https://www.medlink.com/articles/medulloblastoma

  Medulloblastoma occurs throughout the world, and there is no evidence that the tumor is more likely to occur in any ethnic or racial subpopulation. The tumor occurs in slightly higher incidence in males than females (a ratio of 1.3 to 1). However, as noted previously, some biological subgroups have gender differences. The tumor tends to occur in the first decade of life with a bimodal distribution, peaking at 3 to 4 years of age and again later at 8 to 10 years of age. However, the lesion may occur at any time, including adulthood. […] The tumor is the most common malignant primary central nervous system neoplasm occurring in childhood. It makes up approximately 20% of all childhood brain tumors and 30% to 40% of all posterior fossa childhood brain tumors. Up to 10% of children harbor germ-line mutations, which likely predispose to tumor development.

• #5 Clinical presentation, diagnosis, and risk stratification of medulloblastoma – UpToDate

  https://www.uptodate.com/contents/clinical-presentation-diagnosis-and-risk-stratification-of-medulloblastoma/print

  Medulloblastomas are the most common malignant brain tumor of childhood and occur in the cerebellum. The epidemiology, clinical presentation, diagnosis, and risk stratification of medulloblastoma in children and adults will be discussed here. […] Approximately 500 children are diagnosed with a medulloblastoma each year in the United States. Medulloblastoma is the most common malignant brain tumor of childhood, accounting for approximately 10 percent of all primary tumors of the central nervous system among children less than 19 years of age. The peak incidence is between five and nine years of age. Approximately 70 percent of patients are diagnosed before the age of 20. There is a slight increase in incidence between the ages of 20 to 24 years, and the disease is rare after the fourth decade, consistent with its embryonal origin.

• #6 Medulloblastoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/medulloblastoma?lang=us

  Medulloblastomas are the most common malignant brain tumors of childhood, most often presenting as midline masses in the roof of the 4th ventricle (at the superior medullary velum) with associated mass-effect and hydrocephalus. […] Overall medulloblastomas, are the most common malignant brain tumor and account for 12-25% of all pediatric CNS tumors, and 30-40% of pediatric posterior fossa tumors. […] They are also seen in adults but only account for 0.4-1.0% of adult brain tumors. […] Taken as a group, there is a moderate male predilection with a M:F ratio of 2:1, although this is only true of group 3 and 4 tumors. […] They usually present in childhood with 77% of cases before the age of 19. […] When diagnosed in adulthood, they typically present in the 3rd and 4th decades and are more likely to arise in atypical locations. […] Importantly the age of presentation and gender ratio is influenced by tumor genomics.

• #7 Pediatric Medulloblastoma: Practice Essentials, Pathophysiology, Etiology

  https://emedicine.medscape.com/article/987886-overview

  Medulloblastoma is the most common malignant brain tumor in children, accounting for up to 25% of primary CNS neoplasms and approximately 50% of all posterior fossa tumors. […] Approximately 250 new patients are diagnosed annually. Among pediatric patients aged 0-14 years, medulloblastomas represent 68.8% of all embryonal tumors. […] In general, brain tumors occur at a rate of 2.5-4 per 100,000 at-risk children per year. Of these, approximately 18% are medulloblastoma. […] Data from the Surveillance, Epidemiology, and End Results (SEER) program showed that patients aged 0-14 years in the United States have an incidence rate per million population of 5.7 in Whites and 5 in Blacks. […] US incidence per 1 million population for patients aged 0-14 years is 6.1 for boys and 4.5 for girls. […] Peak age of incidence is during the first decade of life. Approximately 80% of patients are diagnosed in the first 15 years of life.

• #8 Medulloblastoma: Diagnosis and Treatment – NCI

  https://www.cancer.gov/rare-brain-spine-tumor/tumors/medulloblastoma

  Seventy percent of medulloblastoma cases occur in children and 30 percent occur in adults. […] Medulloblastomas occur more often in males than females. They are most common in non-Hispanic white and Hispanic people. An estimated 6,070 people are living with this tumor in the United States. […] The relative five-year survival rate for medulloblastoma is 80.6 percent. However, many factors can affect prognosis. These include the tumor molecular group, staging, extent of resection, the persons age and health when diagnosed, and how they respond to treatment.

• #9 Medulloblastoma in adults – reviewing the literature from a surgeon’s point of view | Aging

  https://www.aging-us.com/article/202568/text

  Medulloblastoma is a common primary brain tumor in children but it is a rare cancer in adult patients. […] Adult medulloblastoma occurs at a rate of 0.6 per one million people per year. […] There is a slight disparity between male and female patients, and patients with a fair skin tone are more likely to have a medulloblastoma. […] The incidence of medulloblastoma among all age groups is estimated at about 1.5 per million per year and the incidence of all primitive embryonal tumors is estimated to be about 2 per million per year. […] The incidence in adults is about 0.6 per million per year. […] 1.5% of posterior fossa craniotomies among adult patients are for medulloblastoma, and the dispersion between males and females is about 1.5/1 for all embryonal tumors. […] There is a slight disparity in the incidence of medulloblastoma between patients with different cultural backgrounds according to the SEER-register, and adults with a fair skin tone are more likely to have a medulloblastoma.

• #10 Medulloblastoma epidemiology and demographics – wikidoc

  https://www.wikidoc.org/index.php/Medulloblastoma_epidemiology_and_demographics

  Medulloblastoma is the second most common brain tumor among the pediatric population. The overall age adjusted incidence rate of medulloblastoma is approximately 0.71 per 100,000 individuals in the United States. The incidence of medulloblastoma decreases with age; the median age at diagnosis is between 5 to 7 years. Males are more commonly affected with medulloblastoma than females. The male to female ratio is approximately 1.44 to 1. […] Medulloblastoma is considered a rare tumor among adults, as it comprises fewer than 2% of all brain tumors among that age group. […] The overall age adjusted incidence rate of medulloblastoma among males is approximately 0.84 per 100,000 individuals in the United States. The overall age adjusted incidence rate of medulloblastoma among females is approximately 0.58 per 100,000 individuals in the United States. […] There is no racial predilection to medulloblastoma.

• #11 Medulloblastoma in adults – reviewing the literature from a surgeon’s point of view | Aging

  https://www.aging-us.com/article/202568/text

  Most adult patients with medulloblastoma seem to be diagnosed between 20 and 40 years of age. […] The aim of this review is to provide a short overview of this rare cancer, focusing on imaging features, opportunities for surgical treatment, molecular pathology, adjuvant treatment, and prognostic factors. […] Surveillance and follow-up examinations are of great importance to achieve the best long-term prognosis for medulloblastoma patients after the first tumor treatment. […] Follow-up examinations should be carried out every three months during the first 5 years, including cerebral and spinal imaging as well as cerebrospinal fluid examination. […] It is sensible to perform follow-up examinations until 10 years after diagnosis. […] Brandes et al. found a high risk for recurrent disease, even after 7-10 years after completed tumor treatment.

• #12 Deep learning models for predicting the survival of patients with medulloblastoma based on a surveillance, epidemiology, and end results analysis | Scientific Reports

  https://www.nature.com/articles/s41598-024-65367-9

  To our knowledge, there is a lack of research combining deep learning techniques with the study of medulloblastoma. Therefore, this study aimed to fill this research gap by utilizing data obtained from the Surveillance, Epidemiology, and End Results (SEER) database, which contains information on patients diagnosed with medulloblastoma in the United States. […] This study analyzed data from 2,322 medulloblastoma patients registered in the SEER database between 2000 and 2019. […] The predictive model was generated by partitioning the complete dataset into two mutually exclusive subsets. […] The survival of medulloblastoma patients was significantly affected by non-surgical treatment, LC, white race, tumor size >3.4 cm, total resection, age <3 years, chemotherapy, and radiotherapy. [...] The study results demonstrated that the DeepSurv model outperformed both the CoxPH and RSF models, as indicated by its higher C-index in both the training and testing sets. [...] These findings collectively suggest that the DeepSurv model is more accurate in predicting the survival of patients with medulloblastoma.

• #13 Surveillance scanning of children with medulloblastoma – PubMed

  https://pubmed.ncbi.nlm.nih.gov/8114859/

  Background: The standard follow-up care for children with medulloblastoma includes regular clinical evaluations and surveillance scanning of the central nervous system with computed tomography or magnetic resonance imaging. […] Among children with medulloblastoma, surveillance scanning is of little clinical value. Scanning detected a minority of recurrences, and no patient who had a recurrence survived.

• #14 Surveillance neuroimaging of intracranial medulloblastoma in children: how effective, how often, and for how long? in: Journal of Neurosurgery Volume 99 Issue 2 (2003) Journals

  https://thejns.org/view/journals/j-neurosurg/99/2/article-p280.xml

  The goal of this paper was to review brain and spine images obtained in children with medulloblastomas to determine the risk factors for tumor recurrence and to assess the impact of surveillance imaging on patient outcomes among patients who remain alive 1 month postsurgery. […] Surveillance imaging revealed 10 of the first 53 recurrences and 15 of all 68 recurrences. […] Surveillance imaging is associated with an increase in survival in children with medulloblastomas. More frequent surveillance imaging in children with incomplete tumor excision and recurrent disease may further improve the length of survival.

• #15 Imaging and Medulloblastoma | IntechOpen

  https://www.intechopen.com/chapters/1212487

  Even though MB is the most common primary CNS tumor associated with extra neuroaxis metastasis (ENM), the reported incidence (25%) is rare, with 1% having ENM at presentation. […] The applications and roles of MRI for diagnosis, staging, quantifying residual disease, post-treatment response assessment, periodic surveillance, and prediction of the molecular subgrouping in managing MB are summarized in Table 1. […] The main objective of imaging during follow-up is the detection of early recurrences. […] Most guidelines recommend MRI scans in the initial 23 years following treatment once every 3 months. After 3 years the frequency can be extended to once every 6 months until 5 years followed by yearly MRIs. […] The differences in the incidence, pattern, and time to first relapse among the molecular subgroups are becoming evident.

• #16 Medulloblastoma | IntechOpen

  https://www.intechopen.com/chapters/62364

  Surveillance is important as relapses encountered on surveillance imaging can be better dealt with and show improved survival as paralleled to those identified by reemergence of the clinical symptoms. Though consensus about the schedule is debated, generally clinical and radiographic follow-up is recommended at three-month intervals during the first year after completion of scheduled therapy, at three to four-month intervals in the second year, every 6 months during the third year, and annually thereafter. It is generally agreed that surveillance imaging of the brain should be complemented with full spinal MRI as it often might prove to be crucial and beneficial for the patient.

• #17 Data Show Similar Patterns of Relapse in Standard- and High-Risk Medulloblastoma – Cancer Therapy Advisor

  https://www.cancertherapyadvisor.com/reports/medulloblastoma-patterns-relapse-data-similar-high-risk-treatment/

  Patterns of relapse are similar in patients with standard-risk and high-risk medulloblastoma, a review suggests. […] Patterns of relapse are similar between patients with standard-risk medulloblastoma and those with high-risk medulloblastoma, according to research presented in a poster at the Society for Neuro-Oncology 27th Annual Meeting. […] The researchers identified 8 multicenter clinical trials in which post-therapy surveillance (with MRI or lumbar puncture) was performed every 3-6 months for 2 years. […] Surveillance of the entire neuraxis is important for relapse detection and should be incorporated for both high- and standard-risk patients. Radiographic surveillance every 3 months for the first 2 years is important to detect majority of relapses.

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

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

  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. […] Various clinical and biological parameters have been associated with the likelihood of disease control of embryonal tumors after treatment. 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. […] 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. […] An accurate diagnosis is critical for patients with embryonal tumors.

• #19 Medulloblastoma: Practice Essentials, Background, Pathophysiology

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

  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. […] 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.

• #20

  https://journals.lww.com/md-journal/fulltext/2020/10090/survival_difference_between_brainstem_and.19.aspx

  To investigate the prognoses associated with different locations of medulloblastoma (MB) in terms of survival through a case-control study and evaluate the prognostic factors for MB. […] The Surveillance, Epidemiology, and End Results database was used to identify MB patients diagnosed from 1975 to 2016. […] Brainstem location detrimentally affected the survival time of MB patients in both the matched cohort (hazard ratios =8.14, 95% confidence interval =5.9811.08) and the unmatched cohort (hazard ratios =1.44, 95% confidence interval =1.201.74). […] This study uncovers a survival advantage for cMB patients versus bMB patients. Additionally, prognostic factors include age, extent of surgical resection, and receipt of radiotherapy or chemotherapy. […] This study uncovers a survival advantage for cMB patients over bMB patients. Additionally, prognostic factors include age, extent of surgical resection, radiotherapy status and chemotherapy status.

• #21 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 (see Table 1). […] 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. […] Patients with disseminated CNS disease at diagnosis are at highest risk of disease relapse. Ten percent to 40% of patients with medulloblastoma have CNS dissemination at diagnosis. Infants have the highest incidence and adolescents and adults have the lowest incidence of CNS dissemination.

• #22 Medulloblastoma in adults – reviewing the literature from a surgeon’s point of view | Aging

  https://www.aging-us.com/article/202568/text

  In the SEER database, the survival of adult medulloblastoma patients was 79.9% at 2 years, 64.9% at 5 years and 52.1% at 10 years. […] Other investigations have revealed a number of factors influencing survival for adult medulloblastoma. […] These include the male sex, molecular subtype of the tumor, Chang risk stratification, presence or absence of metastases, extent of resection, irradiation dose, maintenance chemotherapy, and Karnofsky performance status. […] WNT-positive tumors appear to have an excellent prognosis among all age groups. […] SHH-tumors only show superior survival if the histological subtype is desmoplastic.

• #23 Treatment and prognosis of medulloblastoma – UpToDate

  https://www.uptodate.com/contents/treatment-and-prognosis-of-medulloblastoma

  Medulloblastoma is the most common malignant brain tumor of childhood and occurs in the posterior fossa, predominantly in the cerebellum. […] Long-term survival is now achieved in approximately three-quarters of all patients with medulloblastoma, though specific risk groups have varied survival outcomes. […] The treatment and prognosis of medulloblastoma in children and adults, as well as the delayed complications of therapy, are discussed here. […] The optimal initial treatment of patients with medulloblastoma includes both general measures to alleviate increased intracranial pressure and specific therapy directed against the tumor. […] Based upon results from multiple cooperative group trials, the preferred approach in most patients includes a combination of maximal safe surgical resection, radiation therapy (RT) to both the tumor site and the craniospinal axis, and systemic chemotherapy.

• #24 Medulloblastoma | IntechOpen

  https://www.intechopen.com/chapters/62364

  Among the pediatric brain tumors, medulloblastoma (MB) is the most common solid variety and entirely occur in the posterior fossa with tendency to seed into CSF spaces. […] Generally it is recognized that medulloblastoma is the most common malignant brain tumor in children. However, recent data shows that as a group, high-grade gliomas are marginally more prevalent. Approximately 350 new pediatric cases of medulloblastoma are diagnosed in the United States every year and that represents about 30% of pediatric brain tumors and 7–10% of all brain tumors. […] Survival of medulloblastoma patients have become better over the last few decades nonetheless in patients with relapse, the outcome is still miserable. Long-term survival rates have progressively improved from 22% by 1950s to 85% in 2015 with current approaches.

• #25 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. […] 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.

• #26

  https://braintumourresearch.org/pages/types-of-brain-tumours-medulloblastoma?srsltid=AfmBOopmg8eFMN9zYOq7Mj5BoNBCQzrvk_Nr9wCeeq7OKZn8xDwARSzz

  Medulloblastoma is the most common malignant or high-grade paediatric brain tumour. At least 75% of medulloblastomas occur in the cerebellum at the back and base of the brain. […] Medulloblastoma brain cancer is usually diagnosed as a grade 3 or grade 4 tumour. It is more common in boys than girls and is rarer in adults. […] The likelihood of a complete cure depends on various factors, such as the age and overall health of the patient, the size and location of the tumour, and whether cancer has spread to other parts of the body. With modern treatment methods, the cure rate for medulloblastoma can be as high as 70-80% for children and 50-60% for adults. […] Survival rates in children with medulloblastoma depend on the patient’s age and if the tumour has spread. If the disease has not spread, survival rates are above 60%, but if it has spread to the spinal cord, the survival rate is reduced.

• #27 Comparing children and adults with medulloblastoma: a SEER based analysis | Oncotarget

  https://www.oncotarget.com/article/23773/text/

  Medulloblastoma (MB) is a brain malignancy, which commonly occurs in children, but is rare in adults. The Surveillance, Epidemiology, and End Results (SEER) database was used to compare survival, clinical features, and prognostic factors of children and adults with MB from 1992 to 2013. […] The estimated survival rates for children diagnosed with MB for 2, 5, and 10 years were 85.6%, 75.5%, and 67.9%, respectively; the corresponding estimates for adults were 84.9%, 74.2%, and 67.3%. […] The majority of reported survival rates and prognostic factors were based on single-institution comprising small series, or clinical studies that group together adult MB children MB, thus, the results may be inconclusive or may not permit a definite assessment of the prognostic role of clinical and pathologic factors to guide the therapy for adults.

• #28 Long-term outcome of posterior fossa medulloblastoma in patients surviving more than 20 years following primary treatment in childhood | Scientific Reports

  https://www.nature.com/articles/s41598-020-66328-8

  The aim of the study was to analyze the long-term outcome (20 years) after treatment of posterior fossa medulloblastoma (MB) in childhood. […] Observed 20- and 30-year survival rates were 51% and 44%, respectively. […] Despite successful treatment of MB in childhood and satisfactory tumor control during the first 20 years following primary treatment, our data indicates that even long-term survivors may die from tumor recurrence. However, the main factors causing late mortality and morbidity in long-term survivors seem to be the complications related to radiotherapy given in childhood. […] The present series provides long-term outcome data from a subset of patients surviving 20 years following primary treatment for posterior fossa MB in childhood. […] Our main finding from the exceptionally long follow-up is that despite satisfactory control of tumor disease in long-term survivors during the first 20 years, they still may die from tumor recurrences even after decades of observation, but most of them from complications related to treatment.

• #29 Orphanet: Medulloblastoma

  https://www.orpha.net/en/disease/detail/616

  Medulloblastoma (MB) is the most common malignant brain tumor in childhood. Annual incidence varies according to age a group, and is estimated in the USA at 0.47/100,000 in children (0-14 years of age), 0.11/100,000 in adolescents and young adults, 0.02/100,000 in adults (over 40 years of age). Males are more affected than females. […] The overall survival rates are now 80% in standard risk patients, and 30-60 % in high-risk patients. Relapse occurs in nearly 75% of pediatric cases within 2 years.

• #30 Long-term outcome of posterior fossa medulloblastoma in patients surviving more than 20 years following primary treatment in childhood | Scientific Reports

  https://www.nature.com/articles/s41598-020-66328-8

  This is a critical point as it illustrates the time-dependent feature of secondary morbidity related to treatment in long-term survivors, and stresses the need for lifelong follow-up of these patients. […] Taken together, the view of MB as potentially curable disease is counteracted by a life-long risk of developing complications related to the treatment given in childhood. Along with the obvious risk for tumor relapse even decades after primary treatment, this prompts the need for close, life-long follow-up of long-term survivors.

• #31 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).

• #32 Medulloblastoma: DNA Points the Way – St. Jude Children’s Research Hospital

  https://www.stjude.org/about-st-jude/stories/promise-magazine/summer-2018/medulloblastoma-dna-points-the-way.html

  New international research co-led by St. Jude, the European Molecular Biology Laboratory and German Cancer Research Center in Heidelberg, and the Hospital for Sick Children in Toronto, has transformed our understanding of the inherited risks for medulloblastoma. […] Until now, scientists believed most cases of medulloblastoma occurred by chance and did not run in families. But in the largest study yet of genetic susceptibility in a pediatric brain tumor, scientists learned that inherited gene mutations often play a role in its development. […] The discovery has prompted new recommendations for genetic testing and counseling of medulloblastoma patients and their families. […] The cancers odds of appearing due to inherited genes vary depending on the diseases four subgroups. These subgroups are named WNT, sonic hedgehog, Group 3 and Group 4.

• #33 Current medulloblastoma subgroup specific clinical trials – Thompson – Translational Pediatrics

  https://tp.amegroups.org/article/view/40870/html

  Medulloblastoma is the most common malignant pediatric brain tumor, with approximately 1,800 new cases diagnosed in the United States every year (1). […] Medulloblastoma is not a single entity, but rather is comprised of at least four molecular subgroups (7) and 12 subtypes (8). These four molecular subgroups which include wingless (WNT), sonic hedgehog (SHH), Group 3, and Group 4 and have distinct features including tumor drivers, prognosis, and patient demographics (7). […] Given these differences in subgroups and the high incidence of adjuvant therapy-related morbidity, there has recently been significant interest in tailoring adjuvant therapy with the goal of reducing toxic chemotherapy and radiation while optimizing patient outcomes. […] In this review, we have highlighted nine clinical trials which use medulloblastoma molecular subgrouping to tailor adjuvant therapies. […] Future studies will undoubtedly offer more targeted therapies as preclinic work continues to drive the field forward.

• #34 Medulloblastoma research | Taylor Lab

  https://lab.research.sickkids.ca/taylor/medulloblastoma/

  Medulloblastoma is an embryonal tumour of the cerebellum that was thought to represent a single disease entity. […] Data from the Taylor Lab and others, and accepted in an international consensus statement, Taylor et. al 2011 have agreed that MB is in fact comprised of four molecularly distinct diseases that have their own epidemiology, demographics, clinical presentation, transcriptional profile, somatic genetic events, and clinical outcomes: […] More recently we have demonstrated that medulloblastoma is even more heterogenic than thought and may in fact be up to 12 different diseases […] and that there is clinically significant heterogeneity in metastatic medulloblastomas […] We have also shown that these cerebellar tumours are a disorder of early brain development, a finding which provides a proximate explanation for the peak incidence of cerebellar tumours in early childhood. […] Medulloblastoma patients show circulating tumor cells in the blood at diagnosis and these circulating tumor cells can home to the meninges to form metastases.

• #35 Clinical presentation, diagnosis, and risk stratification of medulloblastoma – UpToDate

  https://www.uptodate.com/contents/clinical-presentation-diagnosis-and-risk-stratification-of-medulloblastoma/print

  Approximately 5 to 6 percent of medulloblastomas overall, with significant variation by molecular subtype, occur in association with a cancer predisposition syndrome such as nevoid basal cell carcinoma syndrome (NBCCS), caused by germline mutations in the patched-1 (PTCH1) gene; Li-Fraumeni syndrome, caused by mutations in the tumor protein p53 (TP53) gene; or familial adenomatous polyposis (FAP), caused by inactivating mutations in the adenomatous polyposis coli (APC) gene.

• #36 Epidemiology of Medulloblastomas in Children – ISPN Guide

  https://ispn.guide/tumors-of-the-nervous-system-in-children/infratentorial-tumors-in-children/medulloblastomas-in-children-homepage/epidemiology-of-medulloblastomas-in-children/

  Medulloblastomas are the most common malignant pediatric brain tumor, accounting for 15-20% of pediatric brain tumors and 30-55% of posterior fossa tumors (13, 19, 20). […] The mean age at presentation is 5-7 years, and 80% of patients are younger than 15 years (13). […] Medulloblastomas are more common in males than females, with the male:female ratio ranging from 1.4:1 to greater than 2:1, depending on the series (3, 8). […] No environmental factors have been definitively associated with medulloblastoma incidence (15). […] Gorlins syndrome: 5% incidence of medulloblastoma, also associated with multiple nevoid and basal cell carcinomas, multiple skeletal and cutaneous anomalies, hydrocephalus, calcification of the dura, and developmental delay (15). […] Chemotherapy for Medulloblastomas in Children: Chemotherapy Protocols for Medulloblastomas in Children. […] Outcome of Chemotherapy for Medulloblastomas in Children.

• #37 Medulloblastoma: DNA Points the Way – St. Jude Children’s Research Hospital

  https://www.stjude.org/about-st-jude/stories/promise-magazine/summer-2018/medulloblastoma-dna-points-the-way.html

  The findings have important implications for the treatment and surveillance of patients and their families. […] Somewhere between one in five and one in six sonic hedgehog medulloblastoma patients have a clear hereditary predisposition based on mutations in these six genes. Thats an important finding that has significant implications for patients treatment and their surveillanceas well as for their siblings and parents. […] Northcott and his team have created detailed recommendations guiding clinicians on which types of other cancers to watch for in medulloblastoma patientsand in some cases, their relativesbased on genes and family history. […] The study spotlights an urgent need to make genetic counseling and testing the standard of care for many medulloblastoma patients, particularly those in the sonic hedgehog and WNT subgroups. […] The screenings can help patients and families understand and manage their lifetime cancer risk, Robinson says. But the new research greatly broadens the scope of patients who will be pegged for such consideration.

• #38 Deep learning models for predicting the survival of patients with medulloblastoma based on a surveillance, epidemiology, and end results analysis | Scientific Reports

  https://www.nature.com/articles/s41598-024-65367-9

  Medulloblastoma is a malignant neuroepithelial tumor of the central nervous system. Accurate prediction of prognosis is essential for therapeutic decisions in medulloblastoma patients. […] It is the most common type of paediatric embryonal tumor, with an incidence ranging from 5 to 11 cases per 1 million individuals. […] Multimodal therapy, which includes surgery, external beam irradiation, and/or cytotoxic chemotherapy, can result in survival rates ranging from 50 to 80% based on clinical staging. […] Certain prognostic features, such as age at diagnosis, extent of resection, histological subtype, and molecular subgroup classification, have been found to affect survival predictions in individual patients. […] Previous studies have used the Cox proportional-hazards model (CoxPH) to evaluate the survival rate of medulloblastoma patients.

• #39 Deep learning models for predicting the survival of patients with medulloblastoma based on a surveillance, epidemiology, and end results analysis | Scientific Reports

  https://www.nature.com/articles/s41598-024-65367-9

  To our knowledge, there is a lack of research combining deep learning techniques with the study of medulloblastoma. Therefore, this study aimed to fill this research gap by utilizing data obtained from the Surveillance, Epidemiology, and End Results (SEER) database, which contains information on patients diagnosed with medulloblastoma in the United States. […] This study analyzed data from 2,322 medulloblastoma patients registered in the SEER database between 2000 and 2019. […] The predictive model was generated by partitioning the complete dataset into two mutually exclusive subsets. […] The survival of medulloblastoma patients was significantly affected by non-surgical treatment, LC, white race, tumor size >3.4 cm, total resection, age <3 years, chemotherapy, and radiotherapy. [...] The study results demonstrated that the DeepSurv model outperformed both the CoxPH and RSF models, as indicated by its higher C-index in both the training and testing sets. [...] These findings collectively suggest that the DeepSurv model is more accurate in predicting the survival of patients with medulloblastoma.

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

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

  The presence of brain stem involvement in children with medulloblastoma was found to be a prognostic factor. […] The 2021 WHO classification of embryonal tumors is as follows: Medulloblastoma, molecularly defined; Medulloblastoma, histologically defined; Other CNS embryonal tumors. […] Surveillance imaging of the brain and spine is usually undertaken at routine intervals during and after treatment. […] The development of surveillance strategies other than imaging for patients with medulloblastoma is the subject of ongoing research.

• #41 Childhood medulloblastoma in Morocco (middle-income country): therapeutic outcomes and survival – Jaafari – Therapeutic Radiology and Oncology

  https://tro.amegroups.org/article/view/7981/html

  Medulloblastoma is one of the most common malignant brain tumors of childhood, the management of medulloblastoma in low and middle-income countries (LMIC) is difficult and there is a lack of epidemiological data and mean features about this childhood pathology in Morocco. […] The incidence of medulloblastoma is estimated to be 0.7 per 100,000 children per year, with a male predominance. […] In Morocco, according to the Great Casablanca Register of Cancer, more than 538 cases of brain tumors have been reported in children under 15 years old, with rate of brain tumors accounting for 16% of cases. […] National epidemiological studies of primary brain tumors found that medulloblastoma and pilocytic astrocytoma were the most common types, accounting together for nearly half of the cases. […] This retrospective study provides epidemiological data and the main features of this pathology in Morocco.

• #42 Childhood medulloblastoma in Morocco (middle-income country): therapeutic outcomes and survival – Jaafari – Therapeutic Radiology and Oncology

  https://tro.amegroups.org/article/view/7981/html

  The study found that the overall survival rates for patients with medulloblastoma in Morocco were low, with a 5-year overall survival rate of 50%. […] The study also found that the delay radiotherapy and the presence of metastasis were significant prognostic factors. […] There is limited information available on childhood medulloblastoma in Morocco. […] A better understanding of the therapeutic outcomes and survival rates for this disease in Morocco. […] It would be useful to improve the management of childhood medulloblastoma in Morocco and other low and middle-income countries.

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