Brain stem glioma is a very rare condition. It affects around fifty children a year in France. The care is particular due to its position. What are the symptoms in adults? In the child? What are the treatments? What life expectancy? Dr. Florence Laigle-Donadey, neuro-oncologist.
Brainstem glioma is a very rare tumor located in a region of the nervous system that connects the brain to the spinal cord. In France, about one fifty children are affected by this disease every year, as many girls as boys. Adults are less affected, with a few dozen cases per year. The complexity of affection, the rarity of cases and the uncertainties surrounding this tumor require a specific support patients with brain stem glioma. What is this brain stem glioma? What are the symptoms ? As the tend to ?
Definition: what is brain stem glioma?
“It’s about a brain tumor belonging to the category of gliomasthat is, it arises from cells, other than neurons, which form the architecture of the brain“indicates Dr. Florence Laigle-Donadey, a neuro-oncologist. She is in a very particular region, the brain stem. This structure controls the vital functions.
What are the symptoms of a brain stem glioma?
The peculiarity of this tumor is that it affects the brain stem, a structure of small volume, crossed by many nerve fibers and occupied by nuclei at the origin of the cranial nerves. “The region contributes to functions as diverse as breathing, cardiovascular regulation, sight, hearing, swallowing, speech articulation (being able to speak in an audible voice), fine motor skills, balance, bladder control etc” develops the neuro-oncologist. These functions can then be changed. “It depends on the patient, he can feel visual disturbances, difficulty swallowing or difficulty with balance, to a different extent “ details Dr. Florence Laigle-Donadey.
What are the causes of a brain stem glioma?
There is, to date, no known cause, as in the case of gliomas of other sites. “However, in rare cases, the role of anterior irradiation of the brain (radiation therapy) for another reason it may have favored an acquired predisposition to develop glioma in some patients“, continues our expert.”There might even be hereditary predispositions in the context of often known family illnesses “. But at the moment there is no way to establish with certainty the causes.
“We do not perform surgery for this tumor because the area is too delicate”
Those are the symptoms reported by the patient who will guide the need to exercise CT scan followed by MRI brain to view the affected brain region and highlight this tumor. “MRI will give a suspected diagnosis: to confirm this it will be necessary to carry out a biopsy but since this region is particularly difficult to approach surgically, the operating risks are taken into account. Support will be provided by a highly specialized team and sometimes it is possible to consider that there are too many risks for the biopsy“emphasizes Dr. Florence Laigle-Donadey. Since this tumor is a very rare disease, cases are discussed during multidisciplinary consultation meetings (RCP), among experts from all over France (neuro oncologists, radiotherapists, neuroradiologists, neurosurgeons, etc.) to define in particular the risk and whether or not to perform a biopsyas well as the treatment to be implemented.
Treatments may vary. We will often offer to treatment with radiotherapy or chemotherapy or a combination of both treatments to reduce tumor size and improve symptoms. “Treatment will depend on the case, the nature of the tumor, the context (whether the tumor has just been diagnosed or whether it is recurrent). It is important to have a tumor analysis to know the degree of aggression ” support our expert. “We are seeing advances in the management of gliomas, we know them better in terms of the molecular abnormalities present in their DNA and we are able to classify tumors based on their genetic profile in order to find adequate treatments from the new therapies developed in oncology” says the neuro-oncologist. Management differs between children and adults and according to the evolution of the disease. “The disease is often more aggressive in children. In adults, the disease usually progresses more slowly. In principle, no surgery is performed. removal of this tumor except in the rare cases of so-called circumscribed gliomas, whose limits are easily identifiable because the area is too delicate with a high risk of complications and post-operative sequelae “adds Dr. Florence Laigle-Donadey.
What is the life expectancy of a patient with a brain stem glioma?
It’s a illness which varies depending on whether you are an adult or a child. “Brain stem glioma is not a curable disease, but there ares active treatments to achieve prolonged remissions sometimes for several years. The risk of recurrence remains unpredictable“.”It is a serious disease, but the goal of treatment is to improve symptoms and make the disease chronic, maintaining a good quality of life. In parallel, research is also proceeding“Defends our interlocutor.
Thanks to Dr. Florence Laigle-Donadey, neuro-oncologist of the Department of Neuro-oncology at the Pitié-Salpêtrière Hospital in Paris and coordinator of the GILTRAD national network for adult brain stem tumors.