Gliomatosis cerebri

Last updated
Gliomatosis cerebri
Other namesInfiltrative diffuse astrocytosis
Gliomatosis cerebri.jpg
Axial fluid-attenuated inversion recovery MRI image demonstrating tumor-related infiltration involving both temporal lobes (Short arrow), and the substantia nigra (Long arrow).
Specialty Neuro-oncology, Neurosurgery

Gliomatosis cerebri is a rare growth pattern of some brain tumors, impacting at least three cerebral lobes, mostly with bilateral involvement of the cerebral hemispheres. It can be seen in some types of diffuse glioma, most notably glioblastoma. It consists of infiltrative threads that spread deeply into the brain, making them very difficult to remove with surgery or treat with radiation and is associated with poor prognosis.

Contents

Previously it was considered to be a separate type of tumor, but this view has been overturned by molecular and genetic diagnostics.

Definition

As defined by the WHO, gliomatosis cerebri spans at least three cerebral lobes, usually involves both hemispheres and can also spread deeply into the gray matter or into infratentorial structures like the brainstem, cerebelleum or spinal cord. [1]

Until the 4th revision of the WHO classification of tumours of the central nervous system of 2016, gliomatosis cerebri was considered to be a distinct entity. Due to advances in molecular diganostics, the lack of a specific genetic profile and poor prognostic value, this defintion is now considered obsolete. [1]

Causes

Gliomatosis cerebri is most often caused by glioblastoma, but can also arise from astrocytoma, oligodendroglioma or other types of diffuse glioma. [2] Other pathologies such als vasculitis, encephalitis or leukoencephalopathy may also cause similar radiological findings. [3]

Notes

  1. 1 2 Louis, David N.; Ohgaki, Hiroko; Wiestler, Otmar D.; Cavenee, Webster K., eds. (2016). WHO classification of tumours of the central nervous system. World Health Organization classification of tumours (Revised 4th ed.). Lyon: International Agency for Research on Cancer. p. 23. ISBN   978-92-832-4492-9.{{cite book}}: CS1 maint: date and year (link)
  2. Kwon, Mi Jung; Kang, So Young; Cho, Haeyon; Lee, Jung Il; Kim, Sung Tae; Suh, Yeon-Lim (March 2020). "Clinical relevance of molecular subgrouping of gliomatosis cerebri per 2016 WHO classification: a clinicopathological study of 89 cases". Brain Pathology. 30 (2): 235–245. doi:10.1111/bpa.12782. ISSN   1015-6305. PMC   8018049 . PMID   31435963.
  3. Rudà, Roberta; Bertero, Luca; Sanson, Marc (February 2014). "Gliomatosis Cerebri: A Review". Current Treatment Options in Neurology. 16 (2): 273. doi:10.1007/s11940-013-0273-2. hdl:2318/1701662. ISSN   1092-8480. PMID   24390814. S2CID   25043219.

Related Research Articles

<span class="mw-page-title-main">Brain tumor</span> Neoplasm in the brain

A brain tumor occurs when abnormal cells form within the brain. There are two main types of tumors: malignant (cancerous) tumors and benign (non-cancerous) tumors. These can be further classified as primary tumors, which start within the brain, and secondary tumors, which most commonly have spread from tumors located outside the brain, known as brain metastasis tumors. All types of brain tumors may produce symptoms that vary depending on the size of the tumor and the part of the brain that is involved. Where symptoms exist, they may include headaches, seizures, problems with vision, vomiting and mental changes. Other symptoms may include difficulty walking, speaking, with sensations, or unconsciousness.

<span class="mw-page-title-main">Glioma</span> Tumour of the glial cells of the brain or spine

A glioma is a type of tumor that starts in the glial cells of the brain or the spine. Gliomas comprise about 30 percent of all brain tumors and central nervous system tumours, and 80 percent of all malignant brain tumours.

<span class="mw-page-title-main">Oligodendroglioma</span> Medical condition

Oligodendrogliomas are a type of glioma that are believed to originate from the oligodendrocytes of the brain or from a glial precursor cell. They occur primarily in adults but are also found in children.

<span class="mw-page-title-main">Glioblastoma</span> Aggressive type of brain cancer

Glioblastoma, previously known as glioblastoma multiforme (GBM), is the most aggressive and most common type of cancer that originates in the brain, and has very poor prognosis for survival. Initial signs and symptoms of glioblastoma are nonspecific. They may include headaches, personality changes, nausea, and symptoms similar to those of a stroke. Symptoms often worsen rapidly and may progress to unconsciousness.

<span class="mw-page-title-main">Astrocytoma</span> Medical condition

Astrocytoma is a type of brain tumor. Astrocytomas originate from a specific kind of star-shaped glial cell in the cerebrum called an astrocyte. This type of tumor does not usually spread outside the brain and spinal cord and it does not usually affect other organs. After glioblastomas, astrocytomas are the second most common glioma and can occur in most parts of the brain and occasionally in the spinal cord.

<span class="mw-page-title-main">Central neurogenic hyperventilation</span> Abnormal pattern of breathing

Central neurogenic hyperventilation (CNH) is an abnormal pattern of breathing characterized by deep and rapid breaths at a rate of at least 25 breaths per minute. Increasing irregularity of this respiratory rate generally is a sign that the patient will enter into coma. CNH is unrelated to other forms of hyperventilation, like Kussmaul's respirations. CNH is the human body's response to reduced carbon dioxide levels in the blood. This reduction in carbon dioxide is caused by contraction of cranial arteries from damage caused by lesions in the brain stem. However, the mechanism by which CNH arises as a result from these lesions is still very poorly understood. Current research has yet to provide an effective means of treatment for the rare number of patients who are diagnosed with this condition.

<span class="mw-page-title-main">Pilocytic astrocytoma</span> Medical condition

Pilocytic astrocytoma is a brain tumor that occurs most commonly in children and young adults. They usually arise in the cerebellum, near the brainstem, in the hypothalamic region, or the optic chiasm, but they may occur in any area where astrocytes are present, including the cerebral hemispheres and the spinal cord. These tumors are usually slow growing and benign, corresponding to WHO malignancy grade 1.

<span class="mw-page-title-main">Blastoma</span> Type of cancer arising from precursor cells

A blastoma is a type of cancer, more common in children, that is caused by malignancies in precursor cells, often called blasts. Examples are nephroblastoma, medulloblastoma, and retinoblastoma. The suffix -blastoma is used to imply a tumor of primitive, incompletely differentiated cells, e.g., chondroblastoma is composed of cells resembling the precursor of chondrocytes.

<span class="mw-page-title-main">Gemistocyte</span> Swollen and reactive astrocyte

A gemistocyte is a swollen, reactive astrocyte.

<span class="mw-page-title-main">Gliosarcoma</span> Medical condition

Gliosarcoma is a rare type of glioma, a cancer of the brain that comes from glial, or supportive, brain cells, as opposed to the neural brain cells. Gliosarcoma is a malignant cancer, and is defined as a glioblastoma consisting of gliomatous and sarcomatous components. Primary gliosarcoma (PGS) is classified as a grade IV tumor and a subtype of glioblastoma multiforme in the 2007 World Health Organization classification system (GBM). Because of a lack of specific and clear diagnostic criteria, the word "gliosarcoma" was frequently used to refer to glial tumours with mesenchymal properties, such as the ability to make collagen and reticulin.

<span class="mw-page-title-main">Brainstem glioma</span> Medical condition

A brainstem glioma is a cancerous glioma tumor in the brainstem. 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.

<span class="mw-page-title-main">WHO classification of tumours of the central nervous system</span>

The WHOclassification of tumours of the central nervous system is a World Health Organization Blue Book that defines, describes and classifies tumours of the central nervous system (CNS).

<span class="mw-page-title-main">Giant-cell glioblastoma</span> Tumor of the central nervous system

The giant-cell glioblastoma is a histological variant of glioblastoma, presenting a prevalence of bizarre, multinucleated giant cells.

<span class="mw-page-title-main">Grading of the tumors of the central nervous system</span>

The concept of grading of the tumors of the central nervous system, agreeing for such the regulation of the "progressiveness" of these neoplasias, dates back to 1926 and was introduced by P. Bailey and H. Cushing, in the elaboration of what turned out the first systematic classification of gliomas.
In the following, the grading systems present in the current literature are introduced. Then, through a table, the more relevant are compared.

<span class="mw-page-title-main">Medulloepithelioma</span> Medical condition

Medulloepithelioma is a rare, primitive, fast-growing brain tumour thought to stem from cells of the embryonic medullary cavity. Tumours originating in the ciliary body of the eye are referred to as embryonal medulloepitheliomas, or diktyomas.

Neuro-oncology is the study of brain and spinal cord neoplasms, many of which are very dangerous and life-threatening. Among the malignant brain cancers, gliomas of the brainstem and pons, glioblastoma multiforme, and high-grade astrocytoma/oligodendroglioma are among the worst. In these cases, untreated survival usually amounts to only a few months, and survival with current radiation and chemotherapy treatments may extend that time from around a year to a year and a half, possibly two or more, depending on the patient's condition, immune function, treatments used, and the specific type of malignant brain neoplasm. Surgery may in some cases be curative, but, as a general rule, malignant brain cancers tend to regenerate and emerge from remission easily, especially highly malignant cases. In such cases, the goal is to excise as much of the mass and as much of the tumor margin as possible without endangering vital functions or other important cognitive abilities. The Journal of Neuro-Oncology is the longest continuously published journal in the field and serves as a leading reference to those practicing in the area of neuro-oncology.

<span class="mw-page-title-main">Fibrillary astrocytoma</span> Type of brain tumor

Fibrillary astrocytomas are a group of primary slow-growing brain tumors that typically occur in adults between the ages of 20 and 50.

<span class="mw-page-title-main">IDH1</span> Protein-coding gene in the species Homo sapiens

Isocitrate dehydrogenase 1 (NADP+), soluble is an enzyme that in humans is encoded by the IDH1 gene on chromosome 2. Isocitrate dehydrogenases catalyze the oxidative decarboxylation of isocitrate to 2-oxoglutarate. These enzymes belong to two distinct subclasses, one of which uses NAD+ as the electron acceptor and the other NADP+. Five isocitrate dehydrogenases have been reported: three NAD+-dependent isocitrate dehydrogenases, which localize to the mitochondrial matrix, and two NADP+-dependent isocitrate dehydrogenases, one of which is mitochondrial and the other predominantly cytosolic. Each NADP+-dependent isozyme is a homodimer. The protein encoded by this gene is the NADP+-dependent isocitrate dehydrogenase found in the cytoplasm and peroxisomes. It contains the PTS-1 peroxisomal targeting signal sequence. The presence of this enzyme in peroxisomes suggests roles in the regeneration of NADPH for intraperoxisomal reductions, such as the conversion of 2,4-dienoyl-CoAs to 3-enoyl-CoAs, as well as in peroxisomal reactions that consume 2-oxoglutarate, namely the alpha-hydroxylation of phytanic acid. The cytoplasmic enzyme serves a significant role in cytoplasmic NADPH production. Alternatively spliced transcript variants encoding the same protein have been found for this gene. [provided by RefSeq, Sep 2013]

<span class="mw-page-title-main">Diffuse midline glioma</span> Highly aggressive brain tumor, mostly found in children

Diffuse midline glioma, H3 K27-altered (DMG) is a fatal 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).

<span class="mw-page-title-main">Angiocentric glioma</span>

Angiocentric glioma (AG) refers to a rare neuroepithelial tumor when the superficial brain malignant cells enclose the brain vessels, commonly found in children and young adults. Initially identified in 2005 by Wang and his team from the University of Texas, AG was classified as Grade I by 2007 WHO Classification of Tumors of the Central Nervous System due to its benign clinical behavior, low proliferation index, and curative properties. AG primarily affects children and young adults at an average initial diagnosis age of 16 years old. Over 85% AG patients experience intractable seizures since childhood, especially partial epilepsy.