Brain Stem Glioma
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A brainstem glioma is a cancerous
glioma A glioma is a type of primary tumor that starts in the glial cells of the brain or spinal cord. They are malignant but some are extremely slow to develop. Gliomas comprise about 30% of all brain and central nervous system tumors and 80% of ...
tumor in the
brainstem The brainstem (or brain stem) is the posterior stalk-like part of the brain that connects the cerebrum with the spinal cord. In the human brain the brainstem is composed of the midbrain, the pons, and the medulla oblongata. The midbrain is conti ...
. Around 75% are diagnosed in children and young adults under the age of twenty, but have been known to affect older adults as well. Brainstem gliomas start in the brain or spinal cord tissue and typically spread throughout the nervous system.


Classification

In children, brainsteam gliomas are classified as either
diffuse intrinsic pontine glioma Diffuse midline glioma, H3 K27-altered (DMG) is a tumour that arises in midline structures of the brain, most commonly the brainstem, thalamus and spinal cord. When located in the pons it is also known as diffuse intrinsic pontine glioma (DIPG). ...
s (DIPGs) or as
focal brain stem glioma Focal or FOCAL may refer to: *Focal (lexicographical website), an Irish lexicographical website *FOCAL (programming language), a programming language for the PDP-8 and similar machines *Focal (HP-41), for programming HP calculators *FOCAL (spacecr ...
s. The latter type are less aggressive and easier to treat.


Signs and symptoms

Common symptoms include, but are not necessarily limited to: * Lack of facial control (droopy eyelids) * Double vision * Headache or headache that gets better after vomiting * Nausea and vomiting * Weakness and fatigue * Seizures * Balance problems * Numbness in face Symptoms can develop slowly and subtly and may go unnoticed for months. In other cases, the symptoms may arise abruptly. A sudden onset of symptoms tends to occur with more rapidly growing, high-grade tumors.


Cause

The cause is still unknown. Researchers have not found any direct genetic link.


Diagnosis

Neuroimaging, such as MRI, is the main diagnostic tool for brain stem gliomas. In very rare cases, surgery and biopsy are performed.


Treatment

Unlike most brain tumors, brainstem glioma is not often treated with
neurosurgery Neurosurgery or neurological surgery, known in common parlance as brain surgery, is the specialty (medicine), medical specialty that focuses on the surgical treatment or rehabilitation of disorders which affect any portion of the nervous system ...
due to complications in vital parts of the brain. More often, it is treated with
chemotherapy Chemotherapy (often abbreviated chemo, sometimes CTX and CTx) is the type of cancer treatment that uses one or more anti-cancer drugs (list of chemotherapeutic agents, chemotherapeutic agents or alkylating agents) in a standard chemotherapy re ...
and/or
radiation therapy Radiation therapy or radiotherapy (RT, RTx, or XRT) is a therapy, treatment using ionizing radiation, generally provided as part of treatment of cancer, cancer therapy to either kill or control the growth of malignancy, malignant cell (biology), ...
(though past use of radiation therapy has yielded mixed results). However, these treatments do produce side effects; most often including nausea, the breakdown of the immune system, and fatigue. Hair loss can occur from both chemotherapy and radiation, but usually grows back after chemotherapy has ceased.
Steroid A steroid is an organic compound with four fused compound, fused rings (designated A, B, C, and D) arranged in a specific molecular configuration. Steroids have two principal biological functions: as important components of cell membranes t ...
s such as Decadron may be required to treat swelling in the brain. Decadron can lead to weight gain and infection. Patients may also experience seizures, which need to be treated to avoid complications. For some patients there is a chance of a neurological breakdown; this can include, but is not limited to, confusion and memory loss. The use of
topotecan Topotecan, sold under the brand name Hycamtin among others, is a chemotherapeutic agent medication that is a topoisomerase inhibitor. It is a synthetic, water-soluble analog of the natural chemical compound camptothecin. It is used in the ...
has been investigated. There are several new clinical trials in process. One such trial is
dendritic cell A dendritic cell (DC) is an antigen-presenting cell (also known as an ''accessory cell'') of the mammalian immune system. A DC's main function is to process antigen material and present it on the cell surface to the T cells of the immune system ...
immunotherapy which uses the patient's tumor cells and white blood cells to produce a chemotherapy that directly attacks the tumor.


Prognosis

The prognosis for children depends on the tumor type; most children with DIPGs live less than 18 months whereas the survival time for most children with focal gliomas is more than 18 months.


Research directions

* Memorial Sloan Kettering Cancer Center: Our hypothesis is that unravelling the genomic alterations of diffuse infiltrating pontine gliomas or DIPGs will lead to improved understanding of the biology of such tumours and improved therapeutic options. * National Institutes of Health: DNA Analysis of Tumor Tissue Samples using Paraffin-Embedded Blocks from Patients with Diffuse Pontine Glioma *St. Jude Children's Research Hospital:Our goal is to perform an extensive genetic analysis of tumor samples obtained from patients with DIPG. * St. Jude Children's Research Hospital:Gene sequencing project discovers mutations tied to deadly brain tumors in young children * UCLA's Jonsson Comprehensive Cancer Center:New system uses nanodiamonds to deliver chemotherapy drugs directly to brain tumors * UT Southwestern Medical Center:Researchers identify a switch that controls growth of most aggressive brain tumor cells * The Institute of Cancer Research, London:Genetic flaw may hold key to deadly childhood brain tumour * Patrick Couvreur, Professor and Director of the Physical Chemistry, Pharmacotechnology and Biopharmacy Unit at Paris-Sud University in France:Fighting cancer with nanotechnology * VU University Medical Center - Amsterdam (the Netherlands): implementation of a multi-institutional diffuse pontine glioma autopsy protocol


References


External links

{{Central nervous system tumors Nervous system neoplasia