Central nervous system tumor | |
---|---|
Specialty | Oncology, neurology |
A central nervous system tumor (CNS tumor) is an abnormal growth of cells from the tissues of the brain or spinal cord. [1] CNS tumor is a generic term encompassing over 120 distinct tumor types. [2] Common symptoms of CNS tumors include vomiting, headache, changes in vision, nausea, and seizures. [3] 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. [4]
Below is a list of the most common pediatric CNS- tumors (children's brain tumors). The list is based on the WHO classification of CNS tumors and on the pediatric cancer register Deutsches Kinderkrebsregister | Übersicht [5] and Surveillance, Epidemiology, and End Results Program. [6] The American Cancer Society estimated the number of new cases of pediatric CNS tumors in the US in 2019 to be 23,820, and the number of deaths attributable to CNS tumors to be 17,760. [7]
The incidence rates of the most common brain tumors for adult patients is very different, with meningiomas being the most common tumor, accounting for 38% of all cases, and with a much higher mortality each year. [8]
Tumor classification | Frequency as % of total CNS tumors |
---|---|
Pilocytic astrocytomas | ~30% |
Diffuse astrocytomas | ~12% |
Anaplastic astrocytomas | ~2% |
Glioblastomas | ~3% |
Oligodendroglial tumors | ~1.5% |
Ependymal tumor | ~9% |
Medulloblastomas | ~20% |
Pineal tumors | ~1.5% |
Meningeal tumors | ~1.2% |
Germ cell tumors | ~3% |
The most common symptoms of CNS tumors are headache, vomiting, and nausea. Symptoms vary depending on the tumor and may include unsteady gait, slowed speech, memory loss, loss of hearing and vision, problems with memory, narrowing of visual field, and back pain. Symptoms may also vary greatly between individuals with the same tumor type.
In pediatric patients, symptoms may include: [9]
Some symptoms in adults are specific to the location of the tumor:
The causes of CNS tumors are poorly understood. A few risk factors are known, including radiation exposure, genetic disorder, a family history of CNS tumors, immunodeficiency, stress and a history of previous cancers. As with all cancers, the risk of developing a CNS tumor increases with age. [10]
A number of genetic disorders increase the risk of specific types of CNS tumors. These include tuberous sclerosis, Von Hippel-Lindau disease, Li-Fraumeni syndrome, Gorlin syndrome, Turcot syndrome, Cowden Syndrome and neurofibromatosis types 1 and 2 (NF1/NF2). [11] Patients with NF1 have higher risks of having schwannomas, meningiomas, and some types of gliomas. NF2 is correlated with vestibular schwannomas.
There are no recommended tests to diagnose CNS tumors. [12] The tumor is usually found when patients develop symptoms and visit the doctor. The first step in diagnosis is usually a neurological exam, including tests for reflexes, muscle strength, vision and eye movements, and balance and alertness. If the results are abnormal, additional tests carried out by a specialist such as a neurosurgeon or a neurologist may be recommended.
Adults and children both undergo a similar set of tests to diagnose CNS tumors, including:
Most patients who have CNS tumors do not have a family record of the disease.
Imaging is an important method for diagnosing CNS tumors and determining their location. The location is informative both for identifying the type of tumor and for determining how to treat it. Magnetic resonance imaging (MRI) and computed tomography (CT) scans are the most commonly used imaging technologies for diagnosis of CNS tumors. MRI provides better detail and detection of tumor-infiltrated areas. [13]
Magnetic resonance imaging uses strong magnetic fields, magnetic field gradients, and radio waves to generate images of the structure of the brain. In perfusion MRI a contrast agent, such as gadolinium compounds, may be used to study the structure of the blood vessels around the tumor that provide nutrients and remove waste. [13] The contrast agent may be taken by mouth or injected into the patient before the scan.
Magnetic resonance spectroscopy (MRS) is a variant of MRI used to measure biochemical changes in the brain. Comparing the metabolites detected in normal brain tissue with those in affected brain tissue can help determine the type of tumor and estimate how quickly it is growing. [13]
Computed tomography (CT) technologies include X-ray CT, positron emission tomography (PET) and single-photon emission computed tomography (SPECT). X-ray CT scans take many X-ray measurements from different angles to produce virtual "slices" of specific areas of a scanned object that can then be reassembled into a complete image of the object. Such scans can detect tumors by the swelling and anatomical distortion they cause, or by surrounding edema. While CT scans are widely available and produce images rapidly, MRI scans provide better anatomic detail of brain structures and detection of tumor-infiltrated areas. [13]
A biopsy is the definitive way to diagnose CNS tumors. Because of the difficulty of accessing brain tissue, and the risk of damage to the brain, biopsies may be guided by computer and imaging in a stereotactic surgery procedure. A stereotactic biopsy is performed under local anesthesia or general anesthesia. After the MRI or a CT scan, the scalp or scalp contours are marked to show the position of where to drill or cut the scalp. An image guidance system is then used to provide assistance in directing a needle into the tumor to collect a small tissue sample. The sample is analyzed by a pathologist or neuropathologist to determine whether the tumor is benign or malignant and identify the type of tumor. Biopsies can also be performed as part of an operation to remove the tumor mass. [14]
Blood tests such as a complete blood count (CBC) can provide insight into the progress of a tumor by measuring the number of blood cell types such as white blood cells, red blood cells and platelets. Blood chemistry is also used to check the health of the liver, kidneys and other organs. Many tumors shed microscopic extracellular vesicles into the bloodstream that can be used to monitor the progress of a cancer or its response to therapy. [15] [16]
CNS tumors are typically treated using one or more of the following options:
Treatment of CNS tumors frequently involves a team of doctors working together, including neurosurgeons, neurologists, medical oncologists, radiation oncologists and endocrinologists.
Surgeries are used both to diagnose and to treat CNS tumors. Removal of tumor tissues helps decrease the pressure of the tumor on nearby parts of the brain. [17] The main goal of surgery is to remove as much as possible of the tumor mass while preserving normal brain function, and to relieve the symptoms caused by the tumor such as headache, nausea and vomiting. [18] Some tumors are deep-seated and unsafe to remove, and in these cases the role of surgery may be limited to obtaining a diagnostic biopsy. [18] After the surgery, chemotherapy or radiation therapy may be used to destroy the remaining cancer cells.
Radiation therapy uses high energy rays to destroy cancer cells or to shrink tumors. The kind of rays used are x-rays, gamma rays, electron beams or protons.
According to the National Cancer Institute, [1] there are two types of radiation therapy:
External radiation therapy or teletherapy uses a machine that sends a focused beam of radiation directed at the location of the tumor in the body. The radiation may be delivered from several angles or in a shaped beam to maximize the dose delivered to the tumor while reducing the harm to healthy parts of the body. [1] Treatment is commonly given daily for 4–8 weeks. [19]
In internal radiation therapy, the source of radiation is inserted into the patient's body. This may be done by placing a solid source of radiation adjacent to the tumor in the form of a seed, ribbon or capsule (brachytherapy) [1] or by giving the patient a liquid source of radiation that travels through the body and kills cancer cells (system therapy). In this case the radiation is usually given in the form of injections, ingesting a capsule or through an intravenous line.[ citation needed ]
Chemotherapy is a treatment that uses a tumor-killing drug to prevent the growth of cancer cells by stopping them from dividing. It is often used after surgery or as the first line of treatment. The drug may be given systemically, by injection into a vein or by mouth, or may be injected into the fluid that surrounds the brain and spinal cord to allow the drug to reach the tumor without crossing the blood–brain barrier (intrathecal administration). [1]
Common side effects of chemotherapy include:
An increasing number of drugs are available that promise to target a tumor specifically, reducing harm to normal cells. These therapies are matched to the specific tumor, and include antibodies that bind to specific surface molecules found primarily on the tumor, or small molecules that target proteins mutated in the tumor. Targeted therapies may block enzymes or other proteins necessary for cancer cell proliferation, deliver toxic substances directly to cancer cells, help with immune system function, or prevent the tumor from obtaining the nutrients it needs. [21] For example, bevacizumab is a targeted therapy drug used against various cancers, including glioblastoma, that blocks the blood supply to and therefore the proliferation of cancerous tumors.[ citation needed ] Checkpoint inhibitors, which prevent the tumor from blocking the action of tumor-killing cells of the immune system. are also being tested for CNS tumor therapy. [22] Although targeted therapy may have fewer side effects than other forms of cancer treatment, side effects are still frequent and may include high blood pressure, fatigue, increased risk of infection, or diarrhea.[ citation needed ]
All treatments for CNS tumors have significant risks and side-effects. In cases where tumors are slow growing and do not cause symptoms, it may be preferable to closely watch the patient's condition without any treatment, until new test results or symptoms indicate that the patient's condition has worsened. [17]
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.
Myelitis is inflammation of the spinal cord which can disrupt the normal responses from the brain to the rest of the body, and from the rest of the body to the brain. Inflammation in the spinal cord can cause the myelin and axon to be damaged resulting in symptoms such as paralysis and sensory loss. Myelitis is classified to several categories depending on the area or the cause of the lesion; however, any inflammatory attack on the spinal cord is often referred to as transverse myelitis.
A bone tumor is an abnormal growth of tissue in bone, traditionally classified as noncancerous (benign) or cancerous (malignant). Cancerous bone tumors usually originate from a cancer in another part of the body such as from lung, breast, thyroid, kidney and prostate. There may be a lump, pain, or neurological signs from pressure. A bone tumor might present with a pathologic fracture. Other symptoms may include fatigue, fever, weight loss, anemia and nausea. Sometimes there are no symptoms and the tumour is found when investigating another problem.
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.
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 floor of the fourth ventricle. Rarely, ependymomas can occur in the pelvic cavity.
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.
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."
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.
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.
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.
Leptomeningeal cancer is a rare complication of cancer in which the disease spreads from the original tumor site to the meninges surrounding the brain and spinal cord. This leads to an inflammatory response, hence the alternative names neoplastic meningitis (NM), malignant meningitis, or carcinomatous meningitis. The term leptomeningeal describes the thin meninges, the arachnoid and the pia mater, between which the cerebrospinal fluid is located. The disorder was originally reported by Eberth in 1870. It is also known as leptomeningeal carcinomatosis, leptomeningeal disease (LMD), leptomeningeal metastasis, meningeal metastasis and meningeal carcinomatosis.
A central nervous system cyst is a type of cyst that presents and affects part of the central nervous system (CNS). They are usually benign and filled with either cerebrospinal fluid, blood, or tumor cells. CNS cysts are classified into two categories: cysts that originate from non-central nervous system tissue, migrate to, and form on a portion of the CNS, and cysts that originate within central nervous system tissue itself. Within these two categories, there are many types of CNS cysts that have been identified from previous studies.
Metastatic breast cancer, also referred to as metastases, advanced breast cancer, secondary tumors, secondaries or stage IV breast cancer, is a stage of breast cancer where the breast cancer cells have spread to distant sites beyond the axillary lymph nodes. There is no cure for metastatic breast cancer; there is no stage after IV.
Pleomorphic xanthoastrocytoma (PXA) is a brain tumor that occurs most frequently in children and teenagers. At Boston Children's Hospital, the average age at diagnosis is 12 years.
Fibrillary astrocytomas are a group of primary slow-growing brain tumors that typically occur in adults between the ages of 20 and 50.
Cancer symptoms are changes in the body caused by the presence of cancer. They are usually caused by the effect of a cancer on the part of the body where it is growing, although the disease can cause more general symptoms such as weight loss or tiredness. There are more than 100 different types of cancer with a wide range of signs and symptoms which can manifest in different ways.
A central nervous system primitive neuroectodermal tumor, often abbreviated as PNET, supratentorial PNET, or CNS-PNET, is one of the 3 types of embryonal central nervous system tumors. It is considered an embryonal tumor because it arises from cells partially differentiated or still undifferentiated from birth. Those cells are usually neuroepithelial cells, stem cells destined to turn into glia or neurons. It can occur anywhere within the spinal cord and cerebrum and can have multiple sites of origins, with a high probability of metastasis through cerebrospinal fluid (CSF).
An extracranial germ cell tumor (EGCT) occurs in the abnormal growth of germ cells in the gonads and the areas other than the brain via tissue, lymphatic system, or circulatory system. The tumor can be benign or malignant (cancerous) by its growth rate. According to the National Cancer Institute and St. Jude Children's Research Hospital, the chance of children who are under 15 years old having EGCTs is 3%, in comparison to adolescents, a possibility of 14% with aged 15 to 19 can have EGCTs. There is no obvious cut point in between children and adolescents. However, common cut points in researches are 11 years old and 15 years old.
Paranasal sinus and nasal cavity cancer is a type of cancer that is caused by the appearance and spread of malignant cells into the paranasal sinus and nasal cavity. The cancer most commonly occurs in people between 50 and 70 years old, and occurs twice as often in males as in females. During early phases of the cancer, symptoms may include nasal obstruction and hyposmia, as well as other symptoms. More symptoms may develop as malignant cells further grow and spread into other nearby tissue such as the palate or orbital floor. X-rays of the head and MRI can aid in diagnosis of the cancer while tumor resection surgery, radiation therapy and chemotherapy can be used for treatment of the cancer.
CNS metastasis is the spread and proliferation of cancer cells from their original tumour to form secondary tumours in portions of the central nervous system.