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A nervous system neoplasm is a tumor affecting the nervous system. Types include:
- Nerve sheath tumor
- Brain tumor
- Arachnoid cyst
- Optic nerve glioma
The most common symptom of the papillary tumor is a headache. Because headaches are so common, most people think nothing of it. This is why brain tumors are so dangerous. There are not a lot of symptoms that go along with them so people tend to wait a long time before seeking medical help. Most of the time people will go see a doctor when their headaches become consistent and start to never go away. This symptom however occurs secondary to hydrocephalus, which is a result from compression of the cerebral aqueduct. The cerebral aqueduct is a narrow channel in the midbrain, which connects the third and fourth ventricles. When a tumor blocks the pathway of the cerebrospinal fluid, this will cause headaches in the patient. Often when hydrocephalus occurs, a shunt is put in place in order to alleviate the pressure. In one case study, an endoscopic third ventriculostomy was performed as a first line procedure to treat the hydrocephalus and also for diagnostic purposes.
In some cases, patients have had progressive diplopia, or double vision. Also, although not in all cases, patients sometimes suffer from nausea and vomiting.
Source:
- severe headache
- visual loss (due to papilledema)
- vomiting
- bilateral Babinski sign
- drowsiness (after several hours of the above symptoms)
- gait change (rotation of feet when walking)
- impaction/constipation
- back flexibility
Papillary tumors of the pineal region (PTPR) were first described by A. Jouvet et al. in 2003 and were introduced in the World Health Organization (WHO) classification of Central Nervous System (CNS) in 2007. Papillary Tumors of the Pineal Region are located on the pineal gland which is located in the center of the brain. The pineal gland is located on roof of the diencephalon. It is a cone shaped structure dorsal to the midbrain tectum. The tumor appears to be derived from the specialized ependymal cells of the subcommissural organ. Papillary tumors of the central nervous system and particularly of the pineal region are very rare and so diagnosing them is extremely difficult.
Seizures, frequent mood changes, and headaches are among the earliest symptoms of the tumor. Hemiparesis, or physical weakness on one side of the body, is also common. A continuous EEG recording of the brain's electrical activity may help to identify and localize seizure activity, especially in children. CT scans and MRI scans of the brain may show the presence of a diffuse mass that fails to light up when a contrast dye is given. In some cases, a biopsy may be required to confirm the nature of the tumour.
A neuroectodermal tumor is a tumor of the central or peripheral nervous system.
Fibrillary astrocytomas also called low grade or diffuse astrocytomas, are a group of primary slow growing brain tumors. They typically occur in adults between the ages of twenty and fifty.
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 ependymoma is the fourth ventricle. Rarely, ependymoma can occur in the pelvic cavity.
Syringomyelia can be caused by an ependymoma.
Ependymomas are also seen with neurofibromatosis type II.
Most paragangliomas are either asymptomatic or present as a painless mass. While all contain neurosecretory granules, only in 1–3% of cases is secretion of hormones such as catecholamines abundant enough to be clinically significant; in that case manifestations often resemble those of pheochromocytomas (intra-medullary paraganglioma).
Most patients present clinically with progressive, one sided hearing loss, much more often of the sensorineural rather than conductive type. Patients may also experience tinnitus, vertigo, and loss of vestibular function (ataxia). Symptoms are usually present for a long time, which supports the slow growth of these tumors. Patients may also present with other symptoms related to von Hippel-Lindau syndrome in other anatomic sites, which will result in imaging evaluation of the head.
About 85% of paragangliomas develop in the abdomen; only 12% develop in the chest and 3% in the head and neck region (the latter are the most likely to be symptomatic). While most are single, rare multiple cases occur (usually in a hereditary syndrome). Paragangliomas are described by their site of origin and are often given special names:
- Carotid paraganglioma (carotid body tumor): Is the most common of the head and neck paragangliomas. It usually presents as a painless neck mass, but larger tumors may cause cranial nerve palsies, usually of the vagus nerve and hypoglossal nerve.
- Organ of Zuckerkandl: A collection of paraganglia near the bifurcation of the aorta, comprising a small mass of neural crest-derived chromaffin cells. Serves as a common origin of abdominal paragangliomas.
- Glomus tympanicum and Glomus jugulare: Both commonly present as a middle ear mass resulting in tinnitus (in 80%) and hearing loss (in 60%). The cranial nerves of the jugular foramen may be compressed, resulting swallowing difficulty, or ipsilateral weakness of the upper trapezius and sternocleiodomastoid muscles (from compression of the spinal accessory nerve). These patients present with a reddish bulge behind an intact ear drum. This condition is also known as the "Red drum". On application of pressure to the external ear canal with the help of a pneumatic ear speculum the mass could be seen to blanch. This sign is known as "Brown's sign". A deficient bony plate along the tympanic portion of the internal carotid artery (aberrant ICA) is a normal variant and can be mistaken with glomus jugulare.
- Vagal paraganglioma: These are the least common of the head and neck paragangliomas. They usually present as a painless neck mass, but may result in dysphagia and hoarseness.
- Pulmonary paraganglioma: These occur in the lung and may be either single or multiple.
- Other sites: Rare sites of involvement are the larynx, nasal cavity, paranasal sinuses, thyroid gland, and the thoracic inlet, as well as the bladder in extremely rare cases.
Children affected by pilocytic astrocytoma can present with different symptoms that might include failure to thrive (lack of appropriate weight gain/ weight loss), headache, nausea, vomiting, irritability, torticollis (tilt neck or wry neck) difficulty to coordinate movements and visual complaints (including nystagmus). The complaints may vary depending on the location and size of the neoplasm. The most common symptoms are associated with increased intracranial pressure due to the size of the neoplasm.
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.
A highly malignant undifferentiated primitive neuroepithelial tumour of children, medulloepithelioma may contain bone, cartilage, skeletal muscle, and tends to metastasize extracranially.
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".
Malignant transformation to squamous cell carcinoma may occur, but is unusual.
The signs and symptoms of brain tumors are broad. People with brain tumors will experience them no matter if the tumor is benign (not cancerous) or cancerous. Primary and secondary brain tumors present with similar symptoms, depending on the location, size, and rate of growth of the tumor. For example, larger tumors in the frontal lobe can cause changes in the ability to think. However, a smaller tumor in an area such as Wernicke's area (small area responsible for language comprehension) can result in a greater loss of function.
Hemangioblastomas (or haemangioblastomas, see spelling differences) (capilliary hemangioblastomas) are tumors of the central nervous system that originate from the vascular system usually during middle-age. Sometimes these tumors occur in other sites such as the spinal cord and retina. They may be associated with other diseases such as polycythemia (increased blood cell count), pancreatic cysts and Von Hippel-Lindau syndrome (VHL syndrome). Hemangioblastomas are most commonly composed of stromal cells in small blood vessels and usually occur in the cerebellum, brain stem or spinal cord. They are classed as grade one tumors under the World Health Organization's classification system.
Medulloepithelioma most commonly affect children between 6 months and 5 years; rarely, this tumour may occur congenitally or beyond this age range. Incidence is equal in males and females.
A nerve sheath tumor is a type of tumor of the nervous system (nervous system neoplasm) which is made up primarily of the myelin surrounding nerves.
A peripheral nerve sheath tumor (PNST) is a nerve sheath tumor in the peripheral nervous system. Benign peripheral nerve sheath tumors include schwannomas and neurofibromas.
A malignant peripheral nerve sheath tumor (MPNST) is a cancerous peripheral nerve sheath tumor.
Neurocutaneous melanosis is associated with the presence of either giant congenital melanocytic nevi or non-giant nevi of the skin. It is estimated that neurocutaneous melanosis is present in 2% to 45% of patients with giant congenital melanocytic nevi. Patients with non-giant congenital melanocytic nevi seem to have a much lower, but undefined risk. Of these patients, only a small number are symptomatic, usually displaying symptoms before the age of 2.
These symptoms are the result of melanocytic lesions being present in the leptomeninges of the central nervous system.
Symptoms can include:
- Papilledema
- Cranial palsies
- Headache
- Vomiting
- Seizures
Others symptoms may also exist that are related to an increase in intracranial pressure. These symptoms seem to be present regardless of the malignancy of the melanin deposits within the central nervous system.
Approximately 10% of patient with neurocutaneous melanosis also present the Dandy–Walker syndrome and associated Dandy-Walker malformation. This malformation involves an enlargement of the posterior fossae and fourth ventricle along with agenesis of the cerebellar vermis. The abnormalities of the leptomeninges during fetal development due to neurocutaneous melanosis may be the cause of this increased incidence of the Dandy-Walker malformation. The development of hydrocephalus is the most common symptom associated with a combination of neurocutaneous melanosis and a Dandy-Walker malformation, occurring in about two out of three patients.
Swelling is the most common presenting complaint; however, OKCs may be asymptomatic and found incidentally on dental X-rays.
Imaging studies help to identify the tumor and the specific anatomic site of involvement. Magnetic resonance images show a hyperintensity (hypervascularity) of a heterogeneous mass by T1 weighted images. Computed tomography shows a multilocular, lytic destructive temporal bone mass, centered within the endolymphatic sac (between internal auditory canal and sigmoid sinus).
Pilocytic astrocytoma or juvenile pilocytic astrocytoma or cystic cerebellar astrocytoma (and its variant juvenile pilomyxoid astrocytoma) is a brain tumor that occurs more often in children and young adults (in the first 20 years of life). 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. The neoplasms are associated with the formation of a single (or multiple) cyst(s), and can become very large.
Pilocytic astrocytomas are often cystic, and, if solid, tend to be well-circumscribed. It is characteristically easily seen on Computed tomography (CT scans) and Magnetic Resonance Imaging (MRI).
Juvenile pilocytic astrocytoma is associated with neurofibromatosis type 1 (NF1), and optic gliomas are among the most frequently encountered tumors in patients with this disorder. The majority of pilocytic astrocytomas have a unique KIAA1549-BRAF fusion gene.
Mammary analogue secretory carcinoma (MASC) (also termed MASC; the "SG" subscript indicates salivary gland)) is a salivary gland neoplasm that shares a genetic mutation with certain types of breast cancer. MASC was first described by Skálová et al. in 2010. The authors of this report found a chromosome translocation in certain salivary gland tumors that was identical to the (12;15)(p13;q25) fusion gene mutation found previously in secretory carcinoma, a subtype of invasive ductal carcinoma of the breast.
Astrocytomas are a type of cancer of the brain. They originate in a particular kind of glial cells, star-shaped brain cells in the cerebrum called astrocytes. This type of tumor does not usually spread outside the brain and spinal cord and it does not usually affect other organs. Astrocytomas are the most common glioma and can occur in most parts of the brain and occasionally in the spinal cord. Within the astrocytomas, there are two broad classes recognized in literature, those with:
- Narrow zones of infiltration (mostly noninvasive tumors; e.g., pilocytic astrocytoma, subependymal giant cell astrocytoma, pleomorphic xanthoastrocytoma), that often are clearly outlined on diagnostic images
- Diffuse zones of infiltration (e.g., high-grade astrocytoma, anaplastic astrocytoma, glioblastoma), that share various features, including the ability to arise at any location in the CNS (Central Nervous System), but with a preference for the cerebral hemispheres; they occur usually in adults; and an intrinsic tendency to progress to more advanced grades.
People can develop astrocytomas at any age. The low-grade type is more often found in children or young adults, while the high-grade type are more prevalent in adults. Astrocytomas in the base of the brain are more common in young people and account for roughly 75% of neuroepithelial tumors.