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Small tumors (e.g., < 2.0 cm) usually are incidental findings at autopsy without having caused symptoms. Larger tumors may cause symptoms, depending on the size and location.
- Focal seizures may be caused by meningiomas that overlie the cerebrum.
- Progressive spastic weakness in legs and incontinence may be caused by tumors that overlie the parasagittal frontoparietal region.
- Tumors of the Sylvian aqueduct may cause myriad motor, sensory, aphasic, and seizure symptoms, depending on the location.
- Increased intracranial pressure eventually occurs, but is less frequent than in gliomas.
- Diplopia (Double vision) or uneven pupil size may be symptoms if related pressure causes a third and/or sixth nerve palsy.
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.
Risk factors include exposure to ionizing radiation such as during radiation therapy, a family history of the condition, and neurofibromatosis type 2. As of 2014 they do not appear to be related to cell phone use. They appear to be able to form from a number of different types of cells including arachnoid cells. Diagnosis is typically by medical imaging.
If there are no symptoms, periodic observation may be all that is required. Most cases that result in symptoms can be cured by surgery. Following complete removal less than 20% recur. If surgery is not possible or all the tumor cannot be removed radiosurgery may be helpful. Chemotherapy has not been found to be useful. A small percentage grow rapidly and are associated with worse outcomes.
About one per thousand people in the United States are currently affected. Onset is usually in adults. In this group they represent about 30% of brain tumors. Women are affected about twice as often as men. Meningiomas were reported as early as 1614 by Felix Plater.
Headaches as a result of raised intracranial pressure can be an early symptom of brain cancer. However, isolated headache without other symptoms is rarer, and other symptoms often occur before headaches become common. Certain warning signs for headache exist which make it more likely to be associated with brain cancer. These are as defined by the American Academy of Neurology: "abnormal neurological examination, headache worsened by Valsalva maneuver, headache causing awakening from sleep, new headache in the older population, progressively worsening headache, atypical headache features, or patients who do not fulfill the strict definition of migraine".
The brain is divided into 4 lobes and each lobe or area has its own function. A tumor in any of these lobes may affect the area's performance. The location of the tumor is often linked to the symptoms experienced but each person may experience something different.
- Frontal lobe tumors may contribute to poor reasoning, inappropriate social behavior, personality changes, poor planning, lower inhibition, and decreased production of speech (Broca's area).
- Temporal lobe: Tumors in this lobe may contribute to poor memory, loss of hearing, difficulty in language comprehension (Wernicke's area).
- Parietal lobe: Tumors here may result in poor interpretation of languages, decreased sense of touch and pain, and poor spatial and visual perception.
- Occipital lobe: Damage to this lobe may result in poor or loss of vision.
- Cerebellum: Tumors in this area may cause poor balance, muscle movement, and posture.
- Brain stem: Tumors on this can affect blood pressure, swallowing, and heartbeat.
Chordomas can arise from bone in the skull base and anywhere along the spine. The two most common locations are cranially at the clivus and in the sacrum at the bottom of the spine.
There are three histological variants of chordoma: classical (or "conventional"), chondroid and dedifferentiated.
- The histological appearance of classical chordoma is of a lobulated tumor composed of groups of cells separated by fibrous septa. The cells have small round nuclei and abundant vacuolated cytoplasm, sometimes described as physaliferous (having bubbles or vacuoles).
- Chondroid chordomas histologically show features of both chordoma and chondrosarcoma.
Typically patients present with progressive pain, often long standing and/or bony swelling and restricted range of movement in affected limb 3,12. The latter is most often the case in bones with little overlying soft tissues (e.g. short tubular bones of the hands and feet).
Most chondromyxoid fibromas are located in the metaphyseal region of long bones (60%), and may extend to the epiphyseal line and even rarely abut the articular surface 3,12. They are almost never just epiphyseal 3. The classical site is the upper 1/3rd of tibia ( which accounts for 25% of all cases) with the small tubular bones of the foot, the distal femur and pelvis being other relatively common locations 12.
Rarely occur in the skull or skull base.
Malignant meningioma is a rare, fast-growing tumor that forms in one of the inner layers of the meninges (thin layers of tissue that cover and protect the brain and spinal cord). Malignant meningioma often spreads to other areas of the body.
The World Health Organization classification system defines both grade II and grade III meningiomas as malignant. Historically, histological subtypes have also been used in classification including:
- clear cell (WHO grade II),
- chordoid (WHO grade II),
- rhabdoid (WHO grade III), and
- papillary (WHO grade III)
Benign or low grade meningiomas (WHO grade I) include meningothelial, fibrous, transitional, psammomatous, angiomatous, microcystic, secretory, lymphoplasmacyte-rich, and metaplastic.
The cerebellopontine angle is the anatomic space between the cerebellum and the pons filled with cerebrospinal fluid. This is a common site for the growth of acoustic neuromas or schwannomas. A distinct neurologic syndrome of deficits occurs due to the anatomic proximity of the cerebellopontine angle to specific cranial nerves. Indications include unilateral hearing loss (85%), speech impediments, disequilibrium, tremors or other loss of motor control.
Tumors within the nerve canaliculi initially present with unilateral sensorineural hearing loss, unilateral tinnitus, or disequilibrium (vertigo is rare, on account of the slow growth of neuromas). Speech discrimination out of proportion to hearing loss, difficulty talking on the telephone are frequent accompaniments. Tumors extending into the CPA will likely present with disequilibrium or ataxia depending on the amount of extension on the brainstem. With brainstem extension, midfacial and corneal hypesthesia, hydrocephalus, and other cranial neuropathies become more prevalent.
For example, involvement of CN V from a cerebellopontine mass lesion often results in loss of the ipsilateral (same side of the body) corneal reflex (involuntary blink).
Patients with larger tumours can develop Bruns nystagmus ('dancing eyes') due to compression of the flocculi.
The majority of cases occur in the second and third decades, with approximately 75% of cases occurring before the age of 30 years 1,12-15. There is no recognised gender predilection. Examples have however been seen in patients up to the age of 75 years. In some series there is a male predilection 12 whilst in others no such distribution is found 2
Hemangiopericytoma located in the cerebral cavity is an aggressive tumor of the Mesenchyme with oval nuclei with scant cytoplasm. "There is dense intercellular reticulin staining. Tumor cells can be fibroblastic, myxoid, or pericytic. These tumors, in contrast to meningiomas, do not stain with epithelial membrane antigen. They have a grade 2 or 3 biological behavior, and need to be distinguished from benign meningiomas because of their high rate of recurrence (68.2%) and metastases (Maier et al. 1992; Kleihues et al. 1993 )."
A hemangiopericytoma (HPC) is a type of soft tissue sarcoma that originates in the pericytes in the walls of capillaries. When inside the nervous system, although not strictly a meningioma tumor, it is a meningeal tumor with a special aggressive behavior. It was first characterized in 1942.
Neurofibromatosis type II (also known as MISME syndrome - multiple inherited schwannomas, meningiomas, and ependymomas) is a genetic condition which may be inherited or may arise spontaneously. The main manifestation of the condition is the development of symmetric, benign brain tumors in the region of the cranial nerve VIII, which is the "auditory-vestibular nerve" that transmits sensory information from the inner ear to the brain. Many people with this condition also experience visual problems. NF 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. However, new drug research and some clinical trials have shown some promise in having beneficial effects. Collaborative research to find better treatments is ongoing, such as the work of the Synodos NF-2 Consortium of scientists.
The clinical spectrum of the condition is broad. In other words, people with NF II may develop a wide range of distinct problems.
1. Acoustic nerve: 90% of the patients show bilateral acoustic schwannomas on magnetic resonance imaging (MRI).
2. Other cranial nerves and meninges: About 50% of patients develop tumours in other cranial nerves or meningiomas.
3. Spinal cord: About 50% of the patients develop spinal lesions. Only 40% of the spinal lesions are symptomatic. The spinal tumours in NF II are separated in two groups. Intramedullary lesions are located within the spinal tissue and usually belong to the so-called spinal astrocytomas or ependymomas. The extramedullary lesions are located within the small space between the surface of the spinal cord and the bony wall of the spinal canal. These tumours belong to the schwannomas and meningiomas.
4. Skin: If children show neurofibromas, a diagnostic procedure should be performed to decide which form of neurofibromatosis causes the alterations.
5. Eyes: Studies on patients with NF II show that more than 90% of the affected persons suffer eye lesions. The most common alteration in NF II is the juvenile subcapsular cataract (opacity of the lens) in young people.
"Presenting symptoms" (initial concern that brings a patient to a doctor) of a lesion of the nervus vestibulocochlearis due to a tumour in the region of the cerebello-pontine angle are the following: hearing loss (98%), tinnitus (70%), dysequilibrium (67%), headache (32%), facial numbness and weakness (29% and 10% respectively).
"Clinical signs" (alterations that are not regarded by the patient and that can be detected by the doctor in a clinical examination) of the lesion in discussion are: abnormal corneal reflex (33%), nystagmus (26%), facial hypesthesia (26%).
Evaluation (study of the patient with technical methods) shows the enlargement of the porus acousticus internus in the CT scan, enhancing tumours in the region of the cerebello-pontine angle in gadolinium-enhanced MRI scans, hearing loss in audiometric studies and perhaps pathological findings in electronystagmography. Some times there are elevated levels of protein in liquor study.
In NF II, acoustic neuromas usually affect young people, whereas in sporadic forms of acoustic neuromas, the appearance of the tumour is limited to the elderly.
There are two forms of the NF II:
- The "Wishart-Phenotype" is characterized by multiple cerebral and spinal lesions in patients younger than 20 years and with rapid progression of the tumours.
- Patients that develop single central tumours with slow progression after age of 20 are thought to have the "Feiling-Gardner-Phenotype".
Neuroendocrine adenoma of the middle ear (NAME) is a tumor which arises from a specific anatomic site: middle ear. NAME is a benign glandular neoplasm of middle ear showing histologic and immunohistochemical neuroendocrine and mucin-secreting differentiation (biphasic or dual differentiation).
This uncommon tumor accounts for less than 2% of all ear tumors. While patients present with symptoms related to the middle ear cavity location of the tumor, the tumor may expand into the adjacent structures (external auditory canal, mastoid bone, and eustachian tube). Patients come to clinical attention with unilateral (one sided) hearing loss, usually associated with decreased auditory acuity, and particularly conductive hearing loss if the ossicular bone chain (middle ear bones) is involved. Tinnitus (ringing), otitis media, pressure or occasionally ear discharge are seen. At the time of otoscopic exam, the tympanic membrane is usually intact, with a fluid level or mass noted behind the ear drum. Even though this is a "neuroendocrine" type tumor, there is almost never evidence of neuroendocrine function clinically or by laboratory examination.
Chondrosarcoma is a cancer composed of cells derived from transformed cells that produce cartilage. Chondrosarcoma is a member of a category of tumors of bone and soft tissue known as sarcomas. About 30% of skeletal system cancers are chondrosarcomas. It is resistant to chemotherapy and radiotherapy. Unlike other primary bone cancers that mainly affect children and adolescents, chondrosarcoma can present at any age. It more often affects the axial skeleton than the appendicular skeleton.
Physicians grade chondrosarcoma using several criteria, but particularly on how abnormal the cancerous cells appear under the microscope, and the growth rate of the tumors themselves, both of which are directly linked to the propensity of the cancer to invade locally, and to spread widely to distant organs and sites in the body (called metastasis).
Grade 1 chondrosarcoma grows relatively slowly, has cells whose histological appearance is quite similar to cells of normal cartilage, and have much less aggressive invasive and metastatic properties. Grades 2 and 3 are increasingly faster-growing cancers, with more varied and abnormal-looking cells, and are much more likely to infiltrate surrounding tissues, lymph nodes, and organs. Some, but not all, authorities and medical facilities assign a "Grade 4" to the most anaplastic, undifferentiated cartilage-derived tumors.
The most common sites for chondrosarcoma to grow are the pelvis and shoulder, along with the superior metaphyseal and diaphyseal regions of the arms and legs. However, chondrosarcoma may occur in any bone, and are sometimes found in the skull, particularly at its base.
ICD-O codes provide a more precise classification of chondrosarcoma. These "subtypes" are derived from, and reflect, both (a) the topographical location of the tumor, (b) the histological characteristics of the cancerous cartilage cells, and (c) the makeup of the surrounding matrix material associated with the tumor:
The most obvious clinical sign of syringomyelia is pain. Dogs with CM alone do not seem to have signs, but some appear to have facial pain. Common symptoms in human patients include, severe headache and neck pain, dizziness, vertigo, disequilibrium, visual disturbances, ringing in the ears, difficulty swallowing, palpitations, sleep apnea, muscle weakness, impaired fine motor skills, chronic fatigue and painful tingling of the hands and feet, pruritus.
The blockage of cerebrospinal fluid (CSF) flow may also cause a syrinx to form, eventually leading to syringomyelia. Central cord symptoms such as hand weakness, dissociated sensory loss, and, in severe cases, paralysis may occur.
Chiari malformations (CMs) are structural defects in the cerebellum. They consist of a downward displacement of the cerebellar tonsils through the foramen magnum (the opening at the base of the skull), sometimes causing non-communicating hydrocephalus as a result of obstruction of cerebrospinal fluid (CSF) outflow. The cerebrospinal fluid outflow is caused by phase difference in outflow and influx of blood in the vasculature of the brain. The malformation is named for Austrian pathologist Hans Chiari. A type II CM is also known as an Arnold–Chiari malformation in honor of Chiari and German pathologist Julius Arnold.
CMs can cause headaches, difficulty swallowing (sometimes accompanied by gagging), choking and vomiting, dizziness, nausea, neck pain, unsteady gait (problems with balance), poor hand coordination (fine motor skills), numbness and tingling of the hands and feet, and speech problems (such as hoarseness).
Less often, people with Chiari malformation may experience ringing or buzzing in the ears (tinnitus), weakness, slow heart rhythm, or fast heart rhythm, curvature of the spine (scoliosis) related to spinal cord impairment, abnormal breathing, such as central sleep apnea, characterized by periods of breathing cessation during sleep, and, in severe cases, paralysis.
An exostosis (plural: exostoses) is the formation of new bone on the surface of a bone. Exostoses can cause chronic pain ranging from mild to debilitatingly severe, depending on the shape, size, and location of the lesion. It is most commonly found in places like the ribs, where small bone growths form, but sometimes larger growths can grow on places like the ankles, knees, shoulders, elbows and hips. Very rarely are they on the skull.
They normally form on the bones of joints, and can grow upwards. For example, if an extra bone formed on the ankle, it might grow up to the shin.
Osteophytes are bone spurs that develop on the margins of joints secondary to external stimuli such as osteoarthritis. However, these are not always distinguished from exostoses in any definite way.
When used in the phrases "cartilaginous exostosis" or "osteocartilaginous exostosis", the term is considered synonymous with osteochondroma. Some sources consider the two terms to mean the same thing even without qualifiers, but this interpretation is not universal.
"Fibrous dysplasia" causes bone thinning and growths or lesions in one or more bones of the human body.
These lesions are tumor-like growths that consist of replacement of the medullary bone with fibrous tissue, causing the expansion and weakening of the areas of bone involved. Especially when involving the skull or facial bones, the lesions can cause externally visible deformities. The skull is often, but not necessarily, affected, and any other bone(s) can be involved.
Chiari-like malformation (CM) is the most common cause of foramen magnum obstruction and syringomyelia in dogs. Syringomyelia (SM) is a disease of the spinal cord typified by fluid filled cavities, or syrinxes, within the spinal cord substance. The disease is caused by the obstruction of cerebrospinal fluid (CSF), in the nervous system. A situation of high pressure in the spinal cord compared to low pressure outside, leads to fluid accumulation, which eventually forms cavities. CM is a condition characterized by the mismatch of size between the brain and the skull. The skull is too small causing part of the brain to descend out of the skull through the opening at its base, crowding the spinal cord. The cause of CM is not yet fully understood. CM is rare in most breeds but reportedly has become very widespread in the Cavalier King Charles Spaniel and the Griffon Bruxellois (Brussels Griffon). As many as 95% of Cavalier King Charles Spaniels may have CM. It is worldwide in scope and not limited to any country, breeding line, or kennel, and experts report that it is believed to be inherited in the Cavalier King Charles Spaniel. CM is so widespread in the Cavalier that it may be an inherent part of the CKCS's breed standard. This disease not only affects thousands of dogs, but a similar condition affects over three hundred thousand children yearly. Therefore, canines are an appropriate model for the treatment of the human condition.