Anaplastic oligodendroglioma

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Anaplastic oligodendroglioma
Video of tumor growth simulation (anaplastic oligodendroglioma). Simulation was run for 2600 days since tumor onset (one original cell) with a detection level of 1 cell/mm2.
Specialty Neuro-oncology
Symptoms Epilepsy-Seizures [1]
Usual onsetpeak years are age 45-50 [1]
Durationuntil cure or death
CausesGenerally unknown [1]
Risk factors Generally unknown
Diagnostic method Biopsy
Differential diagnosis Other gliomas
PreventionNot known
TreatmentSurgery, radiation, chemotherapy [1]
Medication Temozolomide [1]
Prognosis Generally fatal after 2-6 years [1]
Frequency0.07 to 0.18 per 100,000 person-years [1]

Anaplastic oligodendroglioma is a neuroepithelial tumor which is believed to originate from oligodendrocytes, a cell type of the glia. In the World Health Organization (WHO) classification of brain tumors, anaplastic oligodendrogliomas are classified as grade III. [2] In the course of the disease, it can degenerate into highly malignant oligodendroglioma, grade IV. [3] The vast majority of oligodendrogliomas occur sporadically, without a confirmed cause and without inheritance within a family.

Contents

Symptoms

Symptoms of anaplastic oligodendroglioma may include: [4]

Pathogenesis

The (malignant) anaplastic oligodendroglioma belongs to the group of diffuse glioma and arises in the central nervous system (brain and spinal cord) from precursor stem cells of the oligodendrocytes. This tumor occurs primarily in middle adulthood with a frequency peak in the 4th and 5th decade of life. [3]

Diagnosis

The most important diagnostic procedure is magnetic resonance imaging (MRI). Occasionally, outside of routine diagnostics, the metabolism in the tissue is shown using positron emission tomography (PET). The diagnosis is confirmed by a fine tissue examination following an operation. Anaplastic oligodendrogliomas often show a loss of genetic material. About 50 to 70% [5] of WHO grade III anaplastic oligodendrogliomas have combined allele losses on the short arm of chromosome 1 (1p) and the long arm of chromosome 19 (19q). This change is mostly referred to as "1p / 19q Co Deletion". It can be seen as favorable for the patient and makes a response to radiation or chemotherapy more likely. The designation of grade III oligodendroglioma (high grade) generally subsumes the previous diagnoses of anaplastic or malignant oligodendroglioma. [2]

Treatment

Surgery can help reduce symptoms caused by the tumor. As complete as possible removal of the tumor visible on the MRI is preferred, provided the location of the tumor allows this. Since typically the cells of an anaplastic oligodendroglioma have already migrated into the surrounding healthy brain tissue at the time of diagnosis, a complete surgical removal of all tumor cells is not possible. The "1p / 19q Codeletion" marker plays an increasingly important role in the selection of therapy and therapy combinations. Because this tumor is an "indolent condition" (a slowly progressive medical condition associated with little or no pain) and the potential morbidity associated with neurosurgery, chemotherapy and radiation therapy, most neuro-oncologists will initially pursue a course of watchful waiting and treat patients symptomatically. Symptomatic treatment often includes the use of anticonvulsants for seizures and steroids for brain swelling. For further treatment, radiation or chemotherapy with temozolomide or a chemotherapy with Procarbazine, CCNU and Vincristine (PCV) has been shown to be effective and was the most commonly used chemotherapy regimen used for treating anaplastic oligodendrogliomas. [3] [6]

Prognosis

5–Year relative survival rate: Age 20–44, 76%. Age 45–54, 67%. Age 55–64, 45%. [7] [8] Procarbazine, lomustine and vincristine have been used since May 1975. For 48 years, new therapeutic options have been regularly tested as part of therapy studies to improve the treatment of anaplastic oligodendroglioma. [9]

Research

As of 2022, a definitive cure is not possible with anaplastic oligodendrogliomas of WHO grade III. A retrospective study on 1054 patients with anaplastic oligodendroglioma, presented during the 2009 ASCO Annual Meeting, suggests that PCV therapy may be effective. Median time to progression for patients with 1p19q co-deletion was longer following PCV alone (7.6 years) than with temozolomide alone (3.3 years); median overall survival was also longer with PCV treatment versus temozolomide treatment (not reached, vs. 7.1 years). [10] A recent long-term study does affirm that radiation combined with adjuvant chemotherapy is significantly more efficacious for anaplastic oligodendroglioma patients with 1p 19q co-deleted tumors and has become the new standard of care. [11] It is possible that radiotherapy may prolong the overall time to progression for non-deleted tumors. If the tumor mass compresses adjacent brain structures, a neurosurgeon will typically remove as much of the tumor as he or she can without damaging other critical, healthy brain structures. Recent studies suggest that radiation does not improve overall survival (even when age, clinical data, histological grading, and type of surgery are considered). [12] [13]

Literature

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">Ependymoma</span> Medical condition

An ependymoma is a tumor that arises from the ependyma, a tissue of the central nervous system. Usually, in pediatric cases the location is intracranial, while in adults it is spinal. The common location of intracranial ependymomas is the fourth ventricle. Rarely, ependymomas can occur in the pelvic cavity.

<span class="mw-page-title-main">Meningioma</span> Type of tumor

Meningioma, also known as meningeal tumor, is typically a slow-growing tumor that forms from the meninges, the membranous layers surrounding the brain and spinal cord. Symptoms depend on the location and occur as a result of the tumor pressing on nearby tissue. Many cases never produce symptoms. Occasionally seizures, dementia, trouble talking, vision problems, one sided weakness, or loss of bladder control may occur.

<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">Procarbazine</span> A chemotherapy medication used for several cancer types

Procarbazine is a chemotherapy medication used for the treatment of Hodgkin's lymphoma and brain cancers. For Hodgkin's it is often used together with chlormethine, vincristine, and prednisone while for brain cancers such as glioblastoma multiforme it is used with lomustine and vincristine. It is typically taken by mouth.

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

Oligoastrocytomas are a subset of brain tumors that present with an appearance of mixed glial cell origin, astrocytoma and oligodendroglioma. However, the term "Oligoastrocytoma" is now considered obsolete by the National Comprehensive Cancer Network stating "the term should no longer be used as such morphologically ambiguous tumors can be reliably resolved into astrocytomas and oligodendrogliomas with molecular testing."

<span class="mw-page-title-main">Primary central nervous system lymphoma</span> Medical condition

Primary central nervous system lymphoma (PCNSL), also termed primary diffuse large B-cell lymphoma of the central nervous system (DLBCL-CNS), is a primary intracranial tumor appearing mostly in patients with severe immunodeficiency. It is a subtype and one of the most aggressive of the diffuse large B-cell lymphomas.

<span class="mw-page-title-main">Lomustine</span> Chemical compound

Lomustine is an alkylating nitrosourea compound used in chemotherapy. It is closely related to semustine and is in the same family as streptozotocin. It is a highly lipid-soluble drug, thus it crosses the blood–brain barrier. This property makes it ideal for treating brain tumors, which is its primary use, although it is also used to treat Hodgkin lymphoma as a second-line option. It has also been used in veterinary practice as a treatment for cancers in cats and dogs.

<span class="mw-page-title-main">Atypical teratoid rhabdoid tumor</span> Medical condition

An atypical teratoid rhabdoid tumor (AT/RT) is a rare tumor usually diagnosed in childhood. Although usually a brain tumor, AT/RT can occur anywhere in the central nervous system (CNS), including the spinal cord. About 60% will be in the posterior cranial fossa. One review estimated 52% in the posterior fossa, 39% are supratentorial primitive neuroectodermal tumors (sPNET), 5% are in the pineal, 2% are spinal, and 2% are multifocal.

Glial tumor is a general term for numerous tumors of the central nervous system, including astrocytomas, ependymal tumors, Oligodendroglioma, and primitive neuroectodermal tumors. The World Health Organization (WHO) classifies tumors into different categories according to severity and recurrence.The first tumor classified as grade I is called pilocytic astrocytoma and it is most commonly observed in children rather than adults. The next tumor is never common in the Dns called diffuse astrocytoma and it is considered a grade II, they are benign, or noncancerous, but can become malignant, meaning cancerous, as the tumor progresses. Grades III and grade IV are considered malignant astrocytomas. Anaplastic astrocytomas are considered by the WHO to be a grade III astrocytoma and Glioblastoma is a grade IV both are referred to high-grade glial tumors.

<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">Esthesioneuroblastoma</span> Medical condition

Esthesioneuroblastoma is a rare cancer of the nasal cavity. Arising from the upper nasal tract, esthesioneuroblastoma is believed to originate from sensory neuroepithelial cells, also known as neuroectodermal olfactory cells.

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">Anaplastic astrocytoma</span> Medical condition

Anaplastic astrocytoma is a rare WHO grade III type of astrocytoma, which is a type of cancer of the brain. In the United States, the annual incidence rate for anaplastic astrocytoma is 0.44 per 100,000 people.

<span class="mw-page-title-main">Temozolomide</span> Cancer medication

Temozolomide, sold under the brand name Temodar among others, is an anticancer medication used to treat brain tumors such as glioblastoma and anaplastic astrocytoma. It is taken by mouth or via intravenous infusion.

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

Astroblastoma is a rare glial tumor derived from the astroblast, a type of cell that closely resembles spongioblastoma and astrocytes. Astroblastoma cells are most likely found in the supratentorial region of the brain that houses the cerebrum, an area responsible for all voluntary movements in the body. It also occurs significantly in the frontal lobe, parietal lobe, and temporal lobe, areas where movement, language creation, memory perception, and environmental surroundings are expressed. These tumors can be present in major brain areas not associated with the main cerebral hemispheres, including the cerebellum, optic nerve, cauda equina, hypothalamus, and brain stem.

Alternating electric field therapy, sometimes called tumor treating fields (TTFields), is a type of electromagnetic field therapy using low-intensity, intermediate frequency electrical fields to treat cancer. TTFields disrupt cell division by disrupting dipole alignment and inducing dielectrophoresis of critical molecules and organelles during mitosis. These anti-mitotic effects lead to cell death, slowing cancer growth. A TTField-treatment device manufactured by the Israeli company Novocure is approved in the United States and Europe for the treatment of newly diagnosed and recurrent glioblastoma multiforme (GBM), malignant pleural mesothelioma (MPM), and is undergoing clinical trials for several other tumor types. Despite earning regulatory approval, the efficacy of this technology remains controversial among medical experts.

Diffuse leptomeningeal glioneuronal tumor (DLGNT) is a rare, primary CNS tumor, classified as distinct entity in 2016 and described as diffuse oligodendroglial-like leptomeningeal tumor of children in the 2016 classification of CNS neoplasms by the WHO., Typically, it's considered juvenile tumors but can occur in adults, the average age of diagnosis is five years. It's characterised by wide leptomeningeal spread with male predominance, like histopathology of neurocytoma, oligodendrocyte-like cytopathology, bland appearance, and severe clinical behaviour. Children's basal cisterns and inter-hemispheric fissures are typically involved in plaque like subarachnoid tumors. A common related intraparenchymal lesion is a spinal lesion. However, in certain situations, superficial parenchyma or Virchow-Robin gaps were affected.
Molecular and genetic investigations frequently show a combination of KIAA1549 and the serine/threonine protein kinase BRAF gene, and also deletions of the short arm of chromosome number 1 and/or the long arm of chromosome number 19.

References

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  3. 1 2 3 Radboud universitair medisch centrum. "highly malignant oligodendroglioma, grade IV" (PDF). www.radboudumc.nl/ (in Dutch). pp. 46–48.
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