Nervous system tumor

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Nervous system tumor
Glioblastoma - MR coronal with contrast.jpg
MRI scan showing a glioblastoma with contrast enhancement
Specialty Neurosurgery, oncology


A nervous system tumor is a tumor that arises within the nervous system, either the central nervous system (CNS) or the peripheral nervous system (PNS). [1] [2] Nervous system primary tumors include various types of brain tumor and spinal tumors, such as gliomas, and meningiomas (of the CNS), and schwannomas (of the PNS) and can be either benign or malignant. [1]

Contents

There are over 120 types of brain and spinal cord tumors. [2] In the CNS a tumor may be a malignant secondary tumor having metastasised (spread from a primary site in the body). Secondary tumors are more common in adults. [2]

Treatment and prognosis depend on factors such as the type of tumor, location, and molecular characteristics. [1]

Types

Primary tumors can affect either the peripheral nervous system (PNS) or the central nervous system (CNS). They may be either benign or malignant. A nerve sheath tumor may be found in both the CNS and PNS. There are over 120 types of brain and spinal cord tumor. [2] A secondary tumor may be found in the CNS that has spread from another part of the body. [1] [2]

In the CNS

Primary tumors in the CNS originate within the brain or spinal cord. [3] Some common types include:

In the PNS

Primary tumors in the PNS originate in the peripheral nerves and nerve sheaths. These tumors are often benign but can sometimes be malignant. Common types of nerve sheath tumors include:

Secondary tumors

Secondary tumors in the CNS, or metastatic tumors, occur when cancer cells spread to the brain or spinal cord from a primary tumor in another part of the body. These tumors are more common than primary CNS tumors in adults and often originate from cancers of the lung, breast, skin, kidney, or colon. [8]

Diagnosis

Diagnosis typically begins with a comprehensive medical history, and a neurological examination. The goal of the evaluation is to identify any neurological changes that may suggest the presence of a tumor. The assessment considers factors such as the nature, progression, and duration of the presented symptoms. [9]

Nervous system tumors can have a wide range of presentations, and a clinical evaluation is often followed by neuroimaging to confirm or rule out the presence of a tumor. Further testing, such as a biopsy, may be necessary to determine the tumor type and grade. [2] [9]

Imaging

Advances in imaging technologies, and molecular diagnostics, have significantly improved the accuracy of diagnoses in the CNS, and made possible effective targeted therapies. [10] [11] [3]

Magnetic resonance imaging (MRI) with contrast is the primary imaging modality for diagnosing brain and spinal cord tumors due to its high-resolution visualization of soft tissues. MRI helps identify the tumor's location, size, and potential impact on surrounding structures. In emergency situations or when MRI is contraindicated, a CT scan can be used as an alternative. [12]

Additional imaging techniques can provide further insights. [2]

Biopsy

A definitive diagnosis often requires a tissue biopsy. The biopsy can be performed through an open surgical procedure or minimally invasive stereotactic surgery. [13] Histopathological examination determines the tumor type, grade, and molecular characteristics, which are critical for guiding treatment decisions. [14]

In some cases, a liquid biopsy by way of a lumbar puncture (spinal tap) may be performed to analyze cerebrospinal fluid (CSF). This can help detect tumor cells, especially in cases of suspected brain tumor metastasis, or primary CNS lymphoma. The presence of tumor markers or abnormal cells in the CSF can provide diagnostic information that complements imaging and biopsy findings. [2]

Advances in molecular diagnostics allow for the identification of genetic and molecular markers, improving the precision of tumor classification and enabling targeted therapies. [15]

Epidemiology

Nervous system neoplasms vary in incidence and type based on factors such as age, tumor origin, and malignancy. Overall, brain and spinal tumors are more common in females due to the higher prevalence of meningiomas. [10]

Gliomas are the most common malignant brain tumor accounting for approximately 81% of malignant brain tumors and 26% of all brain tumors [16] They are the most common brain tumor in children up to 14 years (25.7%), the next most commonly found are pilocytic astrocytomas (17.5%), and embryonal tumors (15.7%). [16] The overall incidence rate of brain tumors in children is 6.2 per 100,000. [10] Glioblastomas are the most aggressive malignant glioma and make up approximately 47.7% of all gliomas, and are more commonly found in males. [10] [16] Their incidence rate is 3.23 per 100,000 people. [10] The 5-year survival rate for glioblastoma is only 6.8%. [10]

Meningiomas, which develop in the meninges, are common brain tumors, representing approximately 37% of all brain tumors. [16] The incidence rate for meningiomas is 9.51 per 100,000 people. [10] Unlike gliomas, more than 98% of meningiomas are benign. [16] The 5-year survival rate for nonmalignant meningiomas is approximately 91%. [10]

The distribution and behavior of nervous system neoplasms differ significantly between adults and children. Gliomas are more frequent and aggressive in adults, while certain benign tumors like pilocytic astrocytomas are more common in children. Notably, adults are more likely to develop secondary (metastatic) tumors that spread to the nervous system from cancers originating in other parts of the body. [1]

See also

Related Research Articles

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

A brain tumor occurs when a group of cells within the brain turn cancerous and grow out of control, creating a mass. 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">Nervous tissue</span> Main component of the nervous system

Nervous tissue, also called neural tissue, is the main tissue component of the nervous system. The nervous system regulates and controls body functions and activity. It consists of two parts: the central nervous system (CNS) comprising the brain and spinal cord, and the peripheral nervous system (PNS) comprising the branching peripheral nerves. It is composed of neurons, also known as nerve cells, which receive and transmit impulses to and from it, and neuroglia, also known as glial cells or glia, which assist the propagation of the nerve impulse as well as provide nutrients to the neurons.

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

A glioma is a type of primary tumor that starts in the glial cells of the brain or spinal cord. They are cancerous but some are extremely slow to develop. Gliomas comprise about 30 percent of all brain tumors and central nervous system tumours, and 80 percent of all malignant brain tumours.

Spinal tumors are neoplasms located in either the vertebral column or the spinal cord. There are three main types of spinal tumors classified based on their location: extradural and intradural. Extradural tumors are located outside the dura mater lining and are most commonly metastatic. Intradural tumors are located inside the dura mater lining and are further subdivided into intramedullary and extramedullary tumors. Intradural-intramedullary tumors are located within the dura and spinal cord parenchyma, while intradural-extramedullary tumors are located within the dura but outside the spinal cord parenchyma. The most common presenting symptom of spinal tumors is nocturnal back pain. Other common symptoms include muscle weakness, sensory loss, and difficulty walking. Loss of bowel and bladder control may occur during the later stages of the disease.

<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">Meningioma</span> Tumor of the membranes surrounding the brain and spinal cord (meninges)

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">Neurofibromatosis type II</span> Type of neurofibromatosis disease

Neurofibromatosis type II is a genetic condition that may be inherited or may arise spontaneously, and causes benign tumors of the brain, spinal cord, and peripheral nerves. The types of tumors frequently associated with NF2 include vestibular schwannomas, meningiomas, and ependymomas. The main manifestation of the condition is the development of bilateral benign brain tumors in the nerve sheath of the cranial nerve VIII, which is the "auditory-vestibular nerve" that transmits sensory information from the inner ear to the brain. Besides, other benign brain and spinal tumors occur. Symptoms depend on the presence, localisation and growth of the tumor(s). Many people with this condition also experience vision problems. Neurofibromatosis type II is caused by mutations of the "Merlin" gene, which seems to influence the form and movement of cells. The principal treatments consist of neurosurgical removal of the tumors and surgical treatment of the eye lesions. Historically the underlying disorder has not had any therapy due to the cell function caused by the genetic mutation.

<span class="mw-page-title-main">Neurofibroma</span> Benign nerve-sheath tumor in the peripheral nervous system

A neurofibroma is a benign nerve-sheath tumor in the peripheral nervous system. In 90% of cases, they are found as stand-alone tumors, while the remainder are found in persons with neurofibromatosis type I (NF1), an autosomal-dominant genetically inherited disease. They can result in a range of symptoms from physical disfiguration and pain to cognitive disability.

<span class="mw-page-title-main">Pilocytic astrocytoma</span> Benign brain tumor originating in astrocyte cells

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">Schwannoma</span> Benign nerve-sheath tumor composed of Schwann cells

A schwannoma is a usually benign nerve sheath tumor composed of Schwann cells, which normally produce the insulating myelin sheath covering peripheral nerves.

<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">Nerve sheath tumor</span> Medical condition

A nerve sheath tumor is a type of tumor of the nervous system which is made up primarily of the myelin surrounding nerves. Nerve sheath tumors can be benign or malignant, and may affect both the peripheral and central nervous systems. There are three main types of nerve sheath tumors: schwannomas, neurofibromas, and malignant peripheral nerve sheath tumors.

<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">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.

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.

Within medical ophthalmology, Intraocular schwannoma, also termed uveal schwannoma, is a type of schwannoma found in the eye. These tumors are almost always benign in nature and while malignant forms have been documented in other areas of the body, this has not been reported in the uveal region. Composed of Schwann cells, these masses are generally slow growing and can be found in the peripheral nerve tract, often around the head and neck.

<span class="mw-page-title-main">Outline of the human nervous system</span> Overview of and topical guide to the human nervous system

The following diagram is provided as an overview of and topical guide to the human nervous system:

A central nervous system tumor is an abnormal growth of cells from the tissues of the brain or spinal cord. CNS tumor is a generic term encompassing over 120 distinct tumor types. Common symptoms of CNS tumors include vomiting, headache, changes in vision, nausea, and seizures. A CNS tumor can be detected and classified via neurological examination, medical imaging, such as x-ray imaging, magnetic resonance imaging (MRI) or computed tomography (CT), or after analysis of a biopsy.

<span class="mw-page-title-main">Peripheral nerve tumor</span> Medical condition

Peripheral nerve tumors, also called tumors of peripheral nerves or tumors of the peripheral nervous system, are a diverse category with a range of morphological characteristics and biological potential. They are categorized as either benign or malignant peripheral nerve sheath tumors.

References

  1. 1 2 3 4 5 "Adult Central Nervous System Tumors Treatment (PDQ®)". National Cancer Institute. 2024-03-29. Retrieved 2024-06-07.
  2. 1 2 3 4 5 6 7 8 9 10 "Brain and Spinal Cord Tumors". National Institute of Neurological Disorders and Stroke. Retrieved 2024-06-07.
  3. 1 2 Louis, David N.; Perry, Arie; Wesseling, Petra; Brat, Daniel J.; Ellison, David W.; Figarella-Branger, Dominique; Hawkins, Cynthia; Ng, Ho-Keung; Pfister, Stefan M.; Reifenberger, Guido; Soerensen, Neil; Von Deimling, Andreas (2021). "The 2021 WHO Classification of Tumors of the Central Nervous System: A Summary". Neuro-Oncology. 23 (8): 1231–1251. doi:10.1093/neuonc/noab106. PMC   8328013 . PMID   33408488.
  4. 1 2 3 "Rare Brain and Spine Tumors". NCI. 17 September 2018. Retrieved 2024-06-08.
  5. ↑ "Vestibular Schwannoma (Acoustic Neuroma) and Neurofibromatosis". National Institute on Deafness and Other Communication Disorders. 6 March 2017. Retrieved 2024-06-07.
  6. ↑ Belakhoua, SM; Rodriguez, FJ (16 February 2021). "Diagnostic Pathology of Tumors of Peripheral Nerve". Neurosurgery. 88 (3): 443–456. doi:10.1093/neuros/nyab021. PMID   33588442.
  7. ↑ "Malignant Peripheral Nerve Sheath Tumor (MPNST)". NCI. 22 July 2020. Retrieved 2024-06-08.
  8. ↑ Brenner, Alex W.; Patel, Akash J. (2022). "Review of Current Principles of the Diagnosis and Management of Brain Metastases". Frontiers in Oncology. 12. doi: 10.1155/2022/4635806 . PMC   9171239 . PMID   35720039.
  9. 1 2 Thomas, Reena P.; Iv, Michael (2024). "Clinical Features of Brain Tumors and Complications of Their Treatment". In Bradley, W. J. (ed.). Neurology: Principles and Practice (3rd ed.). Elsevier. ISBN   9780323642613.
  10. 1 2 3 4 5 6 7 8 Ostrom, Quinn T.; Cioffi, George; Greenlee, Rebecca T.; Kruchko, Clair; Barnholtz-Sloan, Jill S. (2021). "CBTRUS Statistical Report: Primary Brain and Other Central Nervous System Tumors Diagnosed in the United States in 2015–2019". Neuro-Oncology. 23 (12): 1961–1985. doi:10.1093/neuonc/noab200. PMC   9533228 . PMID   36196752.
  11. ↑ "Brain and Spinal Cord Tumor Research". National Cancer Institute. 12 July 2022. Retrieved 2024-06-07.
  12. ↑ Wong, Eric T.; Wu, Julian K. (2023-10-19). Wen, Patrick Y.; Eichler, April F. (eds.). "Overview of the Clinical Features and Diagnosis of Brain Tumors in Adults". UpToDate. Retrieved 2024-06-08.
  13. ↑ Jackson, R.J.; Fuller, G.N.; Abi-Said, D.; Lang, F.F.; Gokaslan, Z.L.; Shi, W.M.; Wildrick, D.M.; Sawaya, R. (2001). "Limitations of Stereotactic Biopsy in the Initial Management of Gliomas". Neuro-Oncology. 3 (3): 193–200. doi:10.1093/neuonc/3.3.193. PMC   1920616 . PMID   11465400.
  14. ↑ Ballester, Leomar Y.; Huse, Jason T. (2024). "Pathology and Molecular Genetics". In Bradley, W. J. (ed.). Bradley and Daroff's Neurology in Clinical Practice (8th ed.). Elsevier. pp. 1076–1098.e2. ISBN   9780323642613.
  15. ↑ Pan, Chong; Diplas, Basilios H.; Chen, Xiaonan; Geng, Ying; Sheng, Hongtao; Liang, Hao; Tang, Kening; Wu, Yang; Zhang, Peng; Chen, Long; Yang, Hai; Xiao, Jian; Yan, Huajun; Zhou, Wei; Zhang, Junchao; Wang, Jun; Yan, Hui (2019). "Molecular profiling of tumors of the brainstem by sequencing of CSF-derived circulating tumor DNA". Acta Neuropathologica. 137 (2): 297–306. doi:10.1007/s00401-018-1936-6. PMC   7523750 . PMID   30460397.
  16. 1 2 3 4 5 Michaud, Dominique S. (2024). "Epidemiology of Brain Tumors". In Bradley, W. J. (ed.). Neurology: Principles and Practice (3rd ed.). Elsevier. ISBN   9780323642613.
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