In 2000 the incidence of tumors was 6.7 per 100,000 persons.
More than 41,000 cases per year in the U.S.
CNS tumors are the most common kind of solid tumors, diagnosed in children, the second most common in adolescents, and the eighth most common in adult adults.
CNS tumors ranked first among all cancers in terms of average years of life lost.
Account for 1.35% of al cancers and 2.2% of all cancer related deaths.
Worldwide approximately 176,000 cases per year are diagnosed and an estimated annual mortality of 128,000.
Most common malignancies in children younger than 15 years.
CNS tumors represent the leading cause of death due to cancer in children, surpassing leukemia.
The incidence for childhood CNS tumors is approximately 55 cases per million children per year, in the most common tumor types are astrocytoma and medulloblastoma.
In children 60% occur in the posterior fossa.
Gliomas make up approximately 51% of all CNS tumors.
Routine advanced, multi dimensional molecular profiling is now required to provide optimal standard of care for patients with CNS tumors.
Molecular testing for central nervous system (CNS) tumors involves a variety of advanced techniques that are essential for accurate diagnosis, prognostication, and treatment planning.
The methods include: Next-Generation Sequencing (NGS): This technique allows for the detection of single nucleotide variations, small insertions/deletions, and gene fusions. It is particularly useful for identifying mutations in genes such as IDH1/IDH2, TP53, and BRAF, which are critical for the classification and management of gliomas and other CNS tumors.
DNA Methylation Profiling: This method is used for tumor classification and subgrouping, especially in complex cases like medulloblastomas and ependymomas. It provides high diagnostic accuracy and helps in distinguishing between different tumor types based on their epigenetic signatures.
Copy Number Profiling: This technique identifies genomic alterations such as 1p/19q codeletion, which is a defining feature of oligodendrogliomas.
It is also used to detect other chromosomal abnormalities that are relevant for diagnosis and prognosis.
Fusion Transcript Detection: This method identifies gene fusions that are characteristic of certain CNS tumors, such as the RELA fusion in ependymomas.
It is necessary for the diagnosis and potential targeted therapy.
Guidelines both incorporate molecular signatures into their diagnostic criteria, underscoring the necessity of these tests for accurate tumor classification and management.
Molecular testing enhances diagnostic precision, informs prognosis, and guides therapeutic decisions, including eligibility for clinical trials and targeted therapies.
Patients suffer with impaired cognition, fatigue, mood disorders, sleep disturbances, sexual dysfunction along with neurologic signs and symptoms.
Postoperative chemotherapy is standard management in treatment of infants and very young children to delay or avoid the use of radiation therapy, because of its adverse neuropsychological and neuroendocrine effects on these children.
Use of cisplatin based chemotherapy in children less than 3 years of age with such lesions have a response rates of 40% and median time to progression 6-9 months (Duffner).