Pediatric low-grade glioma (pLGG) is a group of brain tumors that typically occur in children and are less aggressive than their high-grade forms. These tumors can arise in various areas of the central nervous system, including the optic nerves, cerebral cortex, and brainstem. pLGG are slow-growing, resulting in long asymptomatic periods for patients. However, as the disease progresses, they can cause neurological impairment and poor quality of life, requiring prompt diagnosis and treatment adjustments. Most cases of these tumors are diagnosed in childhood, with the peak incidence occurring between 5 and 10 years of age.
History of the disease and interesting historical facts
Gliomas were first described in medical literature back in the 19th century, when doctors began systematically studying brain tumors. One of the first researchers to study childhood gliomas was the German pathologist W. Mayer. In 1925, he described the features of the macroscopic and microscopic structure of these tumors. Since then, more and more data has appeared on the types and pathogenesis of gliomas, which has allowed doctors to more accurately diagnose and treat this disease. Interestingly, in the 1990s, several classifications were developed that helped to divide these tumors into subtypes, which significantly improved the understanding of their biology and clinical manifestations. In recent years, genetic research has found a link between certain mutations and the development of gliomas, which opens up new horizons for diagnosis and treatment.
Epidemiology
The incidence of low-grade gliomas in childhood varies by region and ethnic group. According to statistics, gliomas account for about 20% of all childhood brain tumors and 4-5% of all childhood neoplasms. The incidence is more common in boys than in girls, with a ratio of approximately 2:1. The main age group affected by the disease is children aged 5-10 years. Epidemiological studies show that the number of reported cases of gliomas in children has been steadily increasing in recent years, possibly due to improved diagnostic methods and greater awareness of diseases of the central nervous system.
Genetic predisposition to this disease
Studies show that there is a certain genetic predisposition to the development of low-grade childhood gliomas. In particular, mutations in the BRAF, FGFR1, and TP53 genes are important markers associated with the occurrence of pLGG. BRAF mutations, in particular, are the most common genetic changes observed in this form of tumors. Specific subtypes of changes in the BRAF gene can indicate different subtypes of gliomas. For example, BRAF V600E mutations are found in 30-40% patients with gliomas. In addition, a number of chromosomal aberrations have been identified, such as deletions on 1p and 19q, which may also indicate a predisposition to gliomas. The development of molecular genetic diagnostic methods has allowed us to deepen our understanding of the pathogenesis of these tumors and even predict their behavior.
Risk factors for the development of this disease
Although the exact causes of low-grade gliomas remain unclear, there are some known risk factors that may predispose to their development. These include:
- Genetic predisposition – having a family history of gliomas may increase the risk of the disease in subsequent generations.
- Physical factors – exposure to ionizing radiation, such as in radiotherapy to the head to treat other diseases.
- Chemical factors – exposure to certain chemicals, such as formaldehyde, may be associated with an increased risk of tumors.
- Other conditions – Certain genetic syndromes, such as Neifertiti syndrome or neurofibromatosis, may increase the likelihood of developing gliomas.
Diagnosis of this disease
Diagnosis of low-grade childhood gliomas is based on a comprehensive approach, including clinical manifestations, instrumental methods and laboratory tests. The main symptoms may include:
- >Headaches are often the most common symptom.
- Visual disturbances – such as double vision or decreased visual acuity.
- Epileptic seizures – may develop as a result of brain damage.
- Cognitive impairment – difficulty learning or remembering.
Laboratory tests may include:
- General and biochemical blood tests – to identify the ratio of cells and possible signs of inflammation.
- Molecular genetic testing – to identify mutations in genes associated with gliomas.
The main radiological diagnostic method is magnetic resonance imaging (MRI), which allows visualization of the tumor and its spread. In the early stages, images may show minor changes, which makes early referral to a specialist important. Computed tomography (CT) may also be used to assess structural changes. Differential diagnostics include exclusion of other neurological diseases, such as metastases or infectious processes.
Treatment
Treatment of low-grade childhood gliomas depends on the location, size, and clinical presentation. General treatment approaches include:
- Observation – in cases of slow growth and asymptomatic progression, active waiting is possible.
- Surgical treatment may be indicated in the presence of surgically accessible tumors for their removal.
- Pharmacological treatment – the use of chemotherapy drugs may be recommended in case of relapse or progression of the disease.
- Radiotherapy – may be used as an adjunct to surgery in cases of residual tumors.
The main medications may be temozolomide-based drugs, in combination with other cytostatics in case of relapses. Baclofen, as well as various anticonvulsants, may be prescribed to control symptoms.
List of medications used to treat this disease
Drug treatment for pLGG may include the following medications:
- Temozolomide
- Prozac
- Epirubicin
- Lomustine
- Vincristine
Each prescription must be carefully calculated taking into account the patient's health and individual characteristics.
Disease monitoring
Monitoring of a patient with pLGG includes regular follow-up examinations, such as MRI, and assessment of neurological function. The prognosis for children with low-grade gliomas is generally good, but depends on a number of factors, including complete tumor removal and the patient’s age. Complications can range from minor neurological impairment to serious complications, such as the development of new neurological symptoms or recurrence of the disease.
Age-related features of the disease
Childhood low-grade glioma is detected mainly in children under 10 years of age, but cases have been reported in adolescents. In children, the disease may manifest itself more acutely, while in adolescents, clinical symptoms may be hidden behind general changes in health. At a younger age, the prognosis is often more optimistic, but is characterized by a threat to neuropsychological functions, which may suffer significantly. Adulthood of patients with pLGG also requires a special approach to treatment, since new, previously unseen, health and adaptation problems may arise.
Questions and Answers
- What are the main symptoms of pLGG?The main symptoms include headaches, visual disturbances, epileptic seizures and cognitive impairment.
- How is low grade glioma diagnosed?Diagnosis is based on clinical data, MRI, laboratory tests and molecular genetic analysis.
- What is the treatment for this disease?Treatment may include observation, surgery, chemotherapy, and radiation therapy depending on the individual case.
- What is the prognosis for pLGG?The prognosis is favorable in most cases, but depends on many factors, including complete removal of the tumor.
- What are the risk factors associated with this disease?Risk factors include genetic predisposition, exposure to radiation, and certain chemicals.
