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Choroid plexus tumors have an annual incidence of about 0.3 per 1 million cases.
It is seen mainly in children under the age of 5, representing 5% of all pediatric tumors and 20% of tumors in children less than 1 year old. There has been no link between sex and occurrence.
Although choroid plexus carcinomas are significantly more aggressive and have half the survival rate as choroid plexus papillomas, they are outnumbered in incidence by 5:1 in all age groups. Clinical studies have shown that patients who receive a total resection of a tumor have a 86% survival rate, while patients who only receive a partial resection have a 26% 5-year survival rate. Many incomplete resections result in recurrence within 2 years of primary surgery.
Choroid plexus papillomas are benign tumors that are usually cured by surgery; malignant progression has been rarely reported.
Internationally tumors of the choroid plexus are rare, accounting for 0.4-0.6% of all intracranial neoplasms. It most commonly affects young children under the age of 5 with a mean patient age of 5.2 years.
The cause of choroid plexus carcinomas are relatively unknown, although hereditary factors are suspected. The sometimes occur in conjunction with other hereditary cancers, including Li–Fraumeni syndrome and malignant rhabdoid tumors. A mutation in the tumor suppressor gene TP53 is usually characterized in this disease.
Choroid plexus tumors are a rare type of cancer that occur from the brain tissue called choroid plexus of the brain. These tumors usually occur in children younger than 2 years and are classified according to the WHO classification of the tumors of the central nervous system:
- Choroid plexus carcinoma (WHO grade III)
- Choroid atypical plexus papilloma (WHO grade II)
- Choroid plexus papilloma (WHO grade I)
Symptoms vary depending on the size and location of the tumor and typically include headaches, nausea and vomiting, irritability, and decreased energy.
In order to remove it completely, surgery may be an option.It relieves the hydrocephalus (excess water in the brain) about half of the time.
Another treatment is chemotherapy, recommended for patients with severe problem.
This is a very rare tumor, since only about 1 in 35,000 to 40,000 people have VHL, of whom about 10% have endolymphatic sac tumors. Patients usually present in the 4th to 5th decades without an gender predilection. The tumor involves the endolymphatic sac, a portion of the intraosseous inner ear of the posterior petrous bone.
The majority of patients can be expected to be cured of their disease and become long-term survivors of central neurocytoma. As with any other type of tumor, there is a chance for recurrence. The chance of recurrence is approximately 20%. Some factors that predict tumor recurrence and death due to progressive states of disease are high proliferative indices, early disease recurrence, and disseminated disease with or without the spread of disease through the cerebral spinal fluid. Long-term follow up examinations are essential for the evaluation of the outcomes that each treatment brings about. It is also essential to identify possible recurrence of CN. It is recommended that a cranial MRI is performed between every 6–12 months.
Papillary tumors of pineal region are extremely rare, constituting 0.4-1% of all central nervous system tumors. These tumors most commonly occur in adults with the mean age being 31.5. There have been cases reported for people between the ages 5 to 66 years. There is a slight predominance of females who have these tumors.
An inverted papilloma is a type of tumor in which surface epithelial cells grow downward into the underlying supportive tissue. It may occur in the nose and/or sinuses or in the urinary tract (bladder, renal pelvis, ureter, urethra). When it occurs in the nose or sinuses, it may cause symptoms similar to those caused by sinusitis, such as nasal congestion. When it occurs in the urinary tract, it may cause blood in the urine.
An endolymphatic sac tumor is a very uncommon papillary epithelial neoplasm arising within the endolymphatic sac or endolymphatic duct. This tumor shows a very high association with von Hippel-Lindau syndrome (VHL).
Genetic counseling is often recommended to provide more information about fetal CPCs, to answer questions and concerns, and to outline available options such as amniocentesis or a blood test from the mother. There is a possible association between ultrasound-detected fetal CPCs and Trisomy 18. It is not correlated to the presence of Trisomy 21 (Down syndrome).
Generally the risks are very low if there are no other risk factors. If no additional abnormalities are detected by a thorough "level II" ultrasound, the likelihood the fetus has trisomy 18 is very low.
A meta-analysis of 8 studies between 1990 and 2000 with choroid plexus cysts that were identified in second-trimester (an incidence of 1.2%). The incidence of the cysts in women younger than 35 was 1% (n=1017). The study found no cases of trisomy 18 in fetuses with cysts whose mother was younger than 35. The study concluded that "there is no evidence that detection of isolated choroid plexus cyst in women who are <35 years of age increases the risk of trisomy 18".
Other factors which may have a bearing on the baby's chances of developing chromosome problems include:
- mother's age at the expected date of delivery
- the results of serum screening; XAFP triple testing or quad screening
- evidence of other "fetal findings" seen at the time of the ultrasound that may suggest a chromosome problem
Immunoperoxidase stains have identified antigens of the human papillomavirus (HPV) types 6 and 11 in approximately 50% of cases of squamous cell papilloma.
A papilloma (plural papillomas or papillomata) ("" + "") is a benign epithelial tumor growing exophytically (outwardly projecting) in nipple-like and often finger-like fronds. In this context refers to the projection created by the tumor, not a tumor on an already existing papilla (such as the nipple).
When used without context, it frequently refers to infections (squamous cell papilloma) caused by human papillomavirus (HPV), such as warts. Human papillomavirus infection is a major cause of cervical cancer, although most HPV infections do not cause cancer. There are, however, a number of other conditions that cause papilloma, as well as many cases in which there is no known cause.
Central neurocytoma, abbreviated CNC, is an extremely rare, ordinarily benign intraventricular brain tumour that typically forms from the neuronal cells of the septum pellucidum. The majority of central neurocytomas grow inwards into the ventricular system forming interventricular neurocytomas. This leads to two primary symptoms of CNCs, blurred vision and increased intracranial pressure. Treatment for a central neurocytoma typically involves surgical removal, with an approximate 1 in 5 chance of recurrence. Central neurocytomas are classified as a grade II tumor under the World Health Organization's classification of tumors of the nervous system.
Inverted papillomas are definitively diagnosed by histologic examination. However, Magnetic Resonanace Imaging (MRI) may show a characteristic feature described as a Convoluted Cerebriform Pattern (CCP). A retrospective study published in the American Journal of Neuroradiology concluded that identification of CCP by MRI in a patient with a nasal tumor made the diagnosis of Inverted papilloma quite likely. The study reported the sensitivity and specificity to be 100% and 87% respectively. CCP can be associated with other malignant tumors as well.
An ependymal tumor is a type of brain tumor that begins in cells lining the spinal cord central canal (fluid-filled space down the center) or the ventricles (fluid-filled spaces of the brain). Ependymal tumors may also form in the choroid plexus (tissue in the ventricles that makes cerebrospinal fluid). Also called ependymoma.
Treatment may include the following:
- Surgery with or without radiation
- Radiotherapy
Fast neutron therapy has been used successfully to treat salivary gland tumors, and has shown to be significantly more effective than photons in studies treating unresectable salivary gland tumors.
- Chemotherapy
Because of the rarity of these tumors, there is still a lot of unknown information. There are many case studies that have been reported on patients who have been diagnosed with this specific type of tumor. Most of the above information comes from the findings resulting from case studies.
Since Papillary Tumors of the Pineal Region were first described in 2003, there have been seventy cases published in the English literature. Since there is such a small number of cases that have been reported, the treatment guidelines have not been established. A larger number of cases that contain a longer clinical follow-up are needed to optimize the management of patients with this rare disease.
Even though there is a general consensus on the morphology and the immunohistochemical characteristics that is required for the diagnosis, the histological grading criteria have yet to be fully defined and its biological behavior appears to be variable. This specific type of tumor appears to have a high potential for local recurrence with a high tumor bed recurrence rate during the five years after the initial surgery. This suggests the need for a tumor bed boost radiotherapy after surgical resection.
As stated above, the specific treatment guidelines have not yet been established, however, gross total resection of the tumor has been the only clinical factor associated overall and progression-free survival. The value of radiotherapy as well as chemotherapy on disease progression will need to be investigated in future trials. With this information, it will provide important insight into long-term management and may further our understanding of the histologic features of this tumor.
Brain, other CNS or intracranial tumors are the ninth most common cancer in the UK (around 10,600 people were diagnosed in 2013), and it is the eighth most common cause of cancer death (around 5,200 people died in 2012).
Normally found in children or young adults, a common cause of conjunctival squamous cell papilloma is during childbirth, when the mother passes the virus to her child.
Little is known about the total incidence of salivary gland tumours as most benign tumours go unrecorded in national cancer registries. The majority of salivary tumours are benign (65-70%). Within the parotid gland 75 - 80% of tumours are benign. Around 50% of the tumours found in the submandibular glands are benign. Sublingual gland tumours are very rare but if present, they are most likely to be malignant.
In the United States, salivary gland cancers are uncommon with an incidence rate of 1.7 in 100000 between 2009 and 2013.
A squamous cell papilloma is a generally benign papilloma that arises from the stratified squamous epithelium of the skin, lip, oral cavity, tongue, pharynx, larynx, esophagus, cervix, vagina or anal canal. Squamous cell papillomas are a result of infection with human papillomavirus (HPV).
An estimated 3% of pediatric brain tumors are AT/RTs, although this percentage may increase with better differentiation between PNET/medulloblastoma tumors and AT/RTs.
As with other CNS tumors, more males are affected than females (ratio 1.6:1). The ASCO study showed a 1.4:1 male to female ratio.
Epidemiological studies are required to determine risk factors. Aside from exposure to vinyl chloride or ionizing radiation, there are no known environmental factors associated with brain tumors. Mutations and deletions of so-called tumor suppressor genes, such as P53, are thought to be the cause of some forms of brain tumor. Inherited conditions, such as Von Hippel–Lindau disease, multiple endocrine neoplasia, and neurofibromatosis type 2 carry a high risk for the development of brain tumors. People with celiac disease have a slightly increased risk of developing brain tumors.
Although studies have not shown any link between cell phone or mobile phone radiation and the occurrence of brain tumors, the World Health Organization has classified mobile phone radiation on the IARC scale into Group 2B – possibly carcinogenic. Discounting claims that current cell phone usage may cause brain cancer, modern, third-generation (3G) phones emit, on average, about 1% of the energy emitted by the GSM (2G) phones that were in use when epidemiological studies that observed a slight increase in the risk for glioma – a malignant type of brain cancer – among heavy users of wireless and cordless telephones were conducted.