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Brain Tumors in Children
Central nervous system tumors encompass many distinct diseases that develop in the brain and, more rarely, in the spinal cord. They account for about one-quarter of all cancers diagnosed in children aged 0 to 17 in France and are the most common solid tumors in children. Approximately 500 new cases are diagnosed each year in children under 18.
These tumors are very different from one another. Their location, rate of progression, microscopic appearance, and—above all—their molecular characteristics determine the diagnosis, treatment, and prognosis. Current classifications are therefore no longer based solely on the appearance of the cells. They combine pathological and biological data to define each disease as precisely as possible.
Key Takeaways
There is no single prognosis that applies to all childhood brain tumors. Some are very often curable, while others remain difficult to treat. The exact type of tumor, its location, extent, biological characteristics, and the child’s age are all key factors.
Tumors that differ from those in adults
Pediatric brain tumors are not simply early-onset versions of the tumors seen in adults. They may arise from different cells, carry different molecular abnormalities, and respond differently to treatment. Their management therefore requires coordinated expertise in pediatrics, neuropathology, neuroradiology, neurosurgery, oncology, and radiation oncology.
The terms “benign” and “malignant” should be used with caution when referring to the central nervous system. A slow-growing tumor can have serious consequences if it compresses a critical area or cannot be safely removed. Conversely, some malignant tumors can be cured with an appropriate combination of treatments.
The Main Types of Tumors
The World Health Organization’s classification distinguishes between numerous pediatric conditions. These can be grouped into broad categories, each comprising several diseases that differ in their clinical presentation and treatment.
They arise from glial cells or related cells. They include low-grade gliomas, which often grow slowly, and more aggressive high-grade pediatric gliomas. Their molecular profile plays an increasingly important role in diagnosis and treatment selection.
These include, in particular, medulloblastoma, certain atypical teratoid and rhabdoid tumors (ATRT), and embryonal tumors with multilayered rosettes (ETMR). Some of these tumors can spread through the cerebrospinal fluid.
They develop from cells that line the cavities containing cerebrospinal fluid. Their location and molecular subtype help determine the prognosis and treatment strategy.
They most commonly occur in the pineal or suprasellar regions. They include germinomas and non-germinomatous forms. Blood or cerebrospinal fluid markers may aid in the diagnosis.
They originate in structures that produce cerebrospinal fluid. They include papillomas, atypical papillomas, and carcinomas. Certain forms, particularly carcinomas, may prompt an investigation into a genetic predisposition.
Craniopharyngiomas, meningiomas, certain glioneural tumors, and other rare conditions require a treatment strategy tailored to their location, biology, and impact on the child’s development.
A classification system that is now integrated
For certain tumors, a comprehensive diagnosis involves microscopic examination, immunohistochemistry, and molecular testing. In select cases, analyzing the DNA methylation profile can help clarify the tumor type.
What symptoms might indicate a brain tumor?
Symptoms depend primarily on the child’s age, the tumor’s location, and how quickly it is progressing. They are often nonspecific and may be related to many more common causes. However, if they persist, worsen, recur, or occur in combination, medical advice should be sought.
Persistent headaches, sometimes worse in the morning; repeated vomiting; unusual drowsiness; irritability; or a rapid increase in head circumference in infants.
Difficulty walking, loss of balance, new clumsiness, weakness on one side of the body, speech or swallowing difficulties, or facial asymmetry.
Double vision, recent strabismus, decreased vision, abnormal eye movements, slowed growth, puberty disorders, or excessive thirst and very frequent urination.
Regression in learning, loss of the ability to walk, behavioral changes, decline in newly acquired skills, unusual academic difficulties, or epileptic seizures.
When to Seek Immediate Medical Attention
Rapid worsening of symptoms, repeated vomiting accompanied by a headache, unusual drowsiness, a seizure, weakness in a limb, or sudden problems with balance, vision, speech, or consciousness require prompt medical evaluation. If you experience sudden or severe symptoms, contact emergency services.
How is the diagnosis made?
The clinical and neurological examination guides the initial diagnostic tests. A brain MRI is the gold standard for locating the tumor, assessing its relationship to neighboring structures, and planning the treatment strategy. Depending on the suspected tumor type, an MRI of the spinal cord may be performed to complete the workup. CT scans remain useful in certain emergency situations or for evaluating calcifications, but they do not replace MRI for characterizing a central nervous system tumor.
The definitive diagnosis is most often based on the analysis of a specimen obtained during surgery or a biopsy. Neuropathology determines the tumor’s characteristics, and molecular analyses look for features useful for its classification, for assessing the prognosis, and, in certain situations, for selecting targeted therapy.
A lumbar puncture may be indicated to detect tumor cells or markers in the cerebrospinal fluid. It is not performed routinely and should only be considered after assessing safety considerations, particularly in the presence of intracranial hypertension.
A diagnosis discussed as a group
The case is reviewed during a multidisciplinary pediatric consultation meeting. The imaging findings, neuropathological diagnosis, molecular biology results, extent of the disease, and the child’s condition are evaluated together to develop a personalized treatment strategy.
Treatments
Treatment depends on the specific type of tumor, its location, extent, molecular profile, the child’s age, and functional risks. It may involve a combination of neurosurgery, radiation therapy, chemotherapy, targeted therapies, and supportive care. For certain slow-growing tumors, close monitoring may be recommended when immediate treatment would pose more risks than benefits.
Neurosurgery
Surgery may have several objectives: obtaining a biopsy to confirm the diagnosis, reducing tumor volume, treating a complication related to cerebrospinal fluid flow, or removing the tumor. The goal is not always complete resection at all costs. When a tumor is adherent to an area that controls an essential function, the team weighs the expected benefit against the risk of neurological sequelae.
Planning relies on imaging and techniques that enhance surgical precision. Depending on the tumor’s location, the neurosurgical pathway may be organized in collaboration with a specialized partner team and then coordinated with the pediatric oncology team for follow-up treatment.
Radiation Therapy
Radiation therapy uses radiation to destroy tumor cells. It can target the area that was operated on or any residual tumor. For certain diseases that can spread through the cerebrospinal fluid, craniospinal radiation may be necessary.
Current techniques aim to tailor the dose to the volume to be treated and minimize exposure to healthy tissue. Intensity-modulated radiation therapy, stereotactic techniques, and proton therapy may be considered depending on the indication. In certain selected situations, proton therapy can reduce the dose received by tissues located beyond the target. However, it is not the best option for every child, and its use is evaluated on a case-by-case basis.
Chemotherapy
Chemotherapy is part of many treatment protocols, either alone or in combination with surgery and radiation therapy. It can shrink the tumor before another treatment, treat widespread disease, reduce the risk of relapse, or allow radiation therapy to be deferred in certain very young children.
The drugs, their doses, and their schedule vary greatly depending on the disease. Some strategies use conventional doses, while others employ intensive chemotherapy with hematopoietic stem cell support.
Targeted Therapies and Clinical Trials
Molecular analysis can identify an abnormality that plays a role in the tumor’s development. When a drug targets this abnormality or the relevant biological pathway, targeted therapy may be offered for certain indications, sometimes as part of a clinical trial.
These treatments do not systematically replace conventional treatments. Their role depends on the type of tumor, evidence of their efficacy, the child’s age, treatments already received, and their availability within an appropriate protocol or authorization.
Preventing and Managing Long-Term Effects
The disease and its treatments can have neurological, motor, cognitive, sensory, endocrine, or psychosocial consequences. The risk depends, in particular, on the tumor’s location, age at the time of treatment, surgery, the volumes irradiated, the doses received, and the medications used.
A neuropsychological evaluation and academic accommodations may be recommended in cases of difficulties with attention, memory, processing speed, or organization.
Endocrinological follow-up may be necessary when the tumor or treatments involve the hypothalamic-pituitary region or affect certain glands.
Hearing and vision evaluations, as well as physical therapy, occupational therapy, psychomotor therapy, and speech therapy, are provided as needed.
The child, his or her parents, and siblings can receive psychological, social, and educational support during treatment and after it ends.
Follow-up does not end when treatment is completed
Follow-up care is designed to monitor for a possible relapse, as well as for the long-term effects of the disease and its treatments. Long-term follow-up, tailored to individual risks, allows for the early detection of problems and the provision of necessary care.
Patient Care at Gustave Roussy
At Gustave Roussy, the treatment strategy is discussed by a multidisciplinary team specializing in pediatric oncology. Depending on the situation, the care pathway involves pediatric oncologists, neuroradiologists, neuropathologists, partner neurosurgeons, radiation oncologists, molecular biologists, anesthesiologists, pain management specialists, and palliative care professionals.
Support is tailored throughout the care journey. It may include psychological support, pain management, nutrition, rehabilitation, neuropsychology, educational support, adapted physical activity, social support, information on fertility preservation, and long-term follow-up.
When the disease is rare, recurrent, or difficult to treat, in-depth molecular analysis and access to a clinical trial may be discussed. Eligibility always depends on the exact diagnosis, the clinical situation, and the specific criteria of the protocol.
Research and Innovation
Research in pediatric neuro-oncology aims to better understand the origin of tumors, refine their classification, identify biomarkers, develop experimental models, and evaluate more effective and less toxic treatments. The integration of molecular biology into diagnosis already makes it possible to distinguish between diseases that were previously grouped under a single name.
The main challenges include tumors with a poor prognosis, relapses, treatment resistance, and reducing long-term effects in children who have been cured. Clinical trials may focus on new drugs, new drug combinations, or strategies tailored to the tumor’s molecular profile.