Diagnosis[ edit ] Like for most brainstem tumors, diagnosing diffuse intrinsic pontine glioma usually involves non-invasive brain imaging like MRI, in addition to neurologic physical exam. Biopsies and other procedures are very uncommon. However, symptoms usually recur after 6 to 9 months and progress rapidly. By nature, these tumors invade diffusely throughout the brain stem, growing between normal nerve cells. Aggressive surgery would cause severe damage to neural structures vital for arm and leg movement, eye movement, swallowing, breathing, and even consciousness.
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A brain glioma can cause headaches , vomiting , seizures , and cranial nerve disorders as a result of increased intracranial pressure. A glioma of the optic nerve can cause visual loss. Spinal cord gliomas can cause pain , weakness , or numbness in the extremities.
Gliomas do not usually metastasize by the bloodstream, but they can spread via the cerebrospinal fluid and cause "drop metastases" to the spinal cord. Hereditary disorders such as neurofibromatoses type 1 and type 2 and tuberous sclerosis complex are known to predispose to their development.
Researchers have observed in some studies that brain tumor patients or their mothers have generally consumed more cured foods also known as Curing than control groups. Recently, Drs. Lee, Wrensch and others found that adults with glioma were more likely to consume diets high in cured foods and low in vitamin C-rich fruits and vegetables, and to consume diets high in nitrites and low in vitamin C.
The effect was more pronounced in men than women. However, the pattern of increased risk with increased consumption of cured foods, and decreased risk with greater consumption of fruits, vegetables, and antioxidant vitamins is compatible with other cancer studies that show increased consumption of vegetables and possibly of fruits is associated with decreased cancer risk.
Infection with cytomegalovirus[ edit ] Other causes[ edit ] Though some studies have shown that farmers have higher rates of gliomas compared to the general population, exposure to farm animals or manure is not associated with glioma.
More consistent data show that architects, surveyors, retail workers, butchers, and engineers have higher rates of gliomas. DNA damages are a likely major primary cause of progression to cancer in general. Furthermore, incomplete DNA repair can give rise to epigenetic alterations or epimutations.
In the case of 12 gliomas On the other hand, Cohen et al. Increased oxidative damage to DNA could be mutagenic. Often, tumor growth causes a breakdown of the blood—brain barrier in the vicinity of the tumor. As a rule, high-grade gliomas almost always grow back even after complete surgical excision, so are commonly called recurrent cancer of the brain.
Tumor suppressor protein 53 p53 is mutated early in the disease. When p53 itself is mutated, other mutations can survive. Phosphatase and tensin homolog PTEN , another tumor suppressor gene, is itself lost or mutated. Epidermal growth factor receptor , a growth factor that normally stimulates cells to divide, is amplified and stimulates cells to divide too much. Together, these mutations lead to cells dividing uncontrollably, a hallmark of cancer.
In , mutations in IDH1 and IDH2 were found to be part of the mechanism and associated with a less favorable prognosis.
Ecco come attaccare un raro tumore del cervello che colpisce i bambini
The brain stem controls breathing, heart rate and the nerves and muscles that help us see, hear, walk, talk and eat. These tumors are called gliomas because they grow from glial cells, a type of supportive cell in the brain. What are the stages of DIPG? DIPG falls into the Glioma staging system, so they can be classified according to the four stages below based on how the cells look under the microscope. The grades are from the least severe to the most severe.
Glioma pontino intrínseco difuso (GPID)
Si tratta di tumori poco comuni, a istologia benigna e guaribili molto spesso con la sola chirurgia. Astrocitoma pilocitico retrobulbare del nervo ottico. Recidive tarde sono relativamente comuni, per cui tali pazienti andrebbero seguiti per almeno 15 anni. I risultati di alcuni studi evidenziano che la massima escissione del tumore fornisce i migliori risultati. Tuttavia questa motivazione continua a risultare opinabile. Immagine RM T1-pesata in piano orizzontale di oligodendroglioma. Immagine RM T2-pesata in piano orizzontale di oligodendroglioma.
Diffuse intrinsic pontine glioma
Per loro natura, questi tumori invadono diffusamente tutto il tronco cerebrale, sviluppandosi tra le cellule nervose normali. La chirurgia aggressiva causerebbe gravi danni alle strutture neurali vitali deputate al movimento delle braccia e delle gambe, degli occhi, della deglutizione, della respirazione e persino della coscienza. Radioterapia[ modifica modifica wikitesto ] Radioterapia per un giovane paziente adulto con glioma pontino intrinseco diffuso. Chemioterapia e altre terapie basate su farmaci[ modifica modifica wikitesto ] Il ruolo della chemioterapia nello DIPG rimane poco chiaro. La chemioterapia intensiva o ad alte dosi con trapianto di midollo osseo autologo o reimmissione di cellule staminali del sangue periferico non ha dimostrato alcuna efficacia negli gliomi del tronco cerebrale.
Diffuse Intrinsic Pontine Glioma (DIPG)