Diffuse Intrinsic Pontine Glioma

Diffuse Intrinsic Pontine Glioma

Article
Brain & Nerve
Kids' Zone
+4
Contributed byKrish Tangella MD, MBADec 27, 2020

The topic Diffuse Intrinsic Pontine Glioma you are seeking is a synonym, or alternative name, or is closely related to the medical condition Diffuse Midline Glioma H3-K27M Mutant.

Quick Summary:

  • A diffuse midline glioma is a form of central nervous system (CNS) tumor. A CNS tumor may be described as a mass of abnormally growing cells arising from the brain tissue (brain parenchyma and meninges) or the spinal cord (central spine). The brain along-with the spinal cord constitute the central nervous system (CNS) of the body
  • Broadly, CNS tumors are classified as benign (non-cancerous) and malignant. Malignant tumors are cancerous and can spread or metastasize to other regions of the body. They generally exhibit rapid growth and, in some cases, may present symptoms during the early stages of development
  • The World Health Organization (WHO) classifies central nervous system tumors according to their grade and histological subtypes. The diagnosis of a tumor subtype is made by the pathologist after examining a tissue biopsy of the tumor under a microscope. This WHO classification system is used by healthcare professionals all over the world in diagnosing and treating these tumors
  • Diffuse Midline Glioma H3-K27M Mutant is a high-grade pediatric brain tumor that arises from the glial cells (a type of brain cells). This high-grade glioma shows astrocytic differentiation, on examination of a tissue biopsy by a pathologist under the microscope, and K27M mutation in either H3F3A or HIST1H3B/C. The tumor mostly arises from the brainstems in children. Per WHO, the tumor forms a part of “diffuse astrocytic and oligodendroglial tumors”
  • The H3K27M-Mutant Diffuse Midline Glioma is a WHO grade IV brain tumor that was previously referred to as Diffuse Intrinsic Pontine Glioma (DIPG). Grade IV tumors are highly-malignant, exhibit aggressive behavior, and grow rapidly. These tumors are known to infiltrate widely and destroy tissues, causing necrosis. They are also known to recur sooner following treatment
  • The cause of formation of Diffuse Midline Glioma H3-K27M Mutant is not well-established. It is reported that a combination of several factors may play a role in their formation, including genetic, environmental, and lifestyle-related. Individuals with a family history of Li-Fraumeni syndrome and neurofibromatosis type 1 are at an increased risk for diffuse midline gliomas. Other risk factors may include the presence of certain genetic disorders, exposure to ionized radiation, viral infections, and even head trauma
  • The signs and symptoms of Diffuse Midline Glioma H3-K27M Mutant may depend on the location of the tumor. Individuals may experience headaches, weakness in different parts of the body, convulsions, vision and speech disturbances. Large-sized tumors may compress adjacent brain tissue resulting in complications. Rarely, tumors may metastasize to sites outside the brain
  • The treatment modalities for Diffuse Midline Glioma H3-K27M Mutant may include surgery, radiation therapy, and chemotherapy. The prognosis is determined by a wide variety of factors, such as age of the individual, tumor size, and overall health status. Nevertheless, in many cases, the prognosis is poor since it is a high-grade (WHO grade IV) and aggressive malignancy

Please find comprehensive information on Diffuse Midline Glioma H3-K27M Mutant regarding definition, distribution, risk factors, causes, signs & symptoms, diagnosis, complications, treatment, prevention, prognosis, and additional useful information HERE.

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Krish Tangella MD, MBA picture
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Krish Tangella MD, MBA

Pathology, Medical Editorial Board, DoveMed Team

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