Choroid plexus papilloma | |
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Other names | Papilloma of the choroid plexus |
Specialty | Neuro-oncology, Neurosurgery |
Choroid plexus papilloma, also known as papilloma of the choroid plexus, is a rare benign neuroepithelial intraventricular WHO grade I lesion found in the choroid plexus. [1] It leads to increased cerebrospinal fluid production, thus causing increased intracranial pressure and hydrocephalus. [2]
Choroid plexus papilloma occurs in the lateral ventricles of children and in the fourth ventricle of adults. This is unlike most other pediatric tumors and adult tumors, in which the locations of the tumors is reversed. In children, brain tumors are usually found in the infratentorial region and in adults, brain tumors are usually found in the supratentorial space. The relationship is reversed for choroid plexus papillomas.
CPPs are rare tumors of neuroectodermal origin. They make up 0.4 to 0.6 percent of all intracranial neoplasms in children and are the third most prevalent congenital brain tumors after teratomas and gliomas. [3] With a median age upon diagnosis of 3.5 years, this lesion is often a disease of infancy. They often reside supratentorial in the lateral ventricles of infants (most commonly in the atrium). [4] The fourth ventricle in adults is the optimum location. [5] Adults rarely have it at the cerebellopontine angle. [4]
Simian virus (SV) 40 has been linked in studies to the development of choroid plexus tumors (CPTs). [6] The BK and JC viruses have also been linked to the problem. [7] In people with choroid plexus tumors, complexes formed by the big T antigen and the tumor suppressor proteins p53 and pRb have been shown to develop. [7] However, the available evidence do not suggest a causal involvement. There is currently no additional causal component being investigated. One of the most prevalent mutations in CPTs is the TP53 R248W mutation. [8]
Signs of the tumor resulting from increased intracranial pressure are present in 91% of patients, with vomiting, homonymous visual field defects and headache being the most common symptoms. Other symptoms are ear ringing and nausea, loss of balance and mobility, worsening eyesight, memory issues, brain fog, and mood swings.
Choroid plexus tumors are divided into three categories by the World Health Organization (2016): [9] papillomas (grade I), atypical tumors (grade II), and carcinomas (grade III). Less than two mitotic figures per 10 high power fields are present in CPPs, two to five are present in atypical ones, and more than five are present in carcinomas. The tumors are visible as pink, soft, spherical lumps with erratic projections and considerable vascularity.
The tumor is neuroectodermal in origin and similar in structure to a normal choroid plexus. They may be created by epithelial cells of the choroid plexus. Papillary fronds lined by bland columnar epithelium are visible under the microscope. Normal absences include mitotic activity, nuclear pleomorphism, and necrosis. [10] Tumors have positive immunohistochemistry for cytokeratin, vimentin, podoplanin, and S-100. [11] Up to 20% of choroid plexus papilloma patients may test positive for glial fibrillary acidic protein (GFAP). [12] Studies have found that fourth ventricle cancers express more S100 than lateral ventricle tumors, and older patients (over 20 years) express more GFAP and transthyretin than younger patients. [13] Some individuals with choroid plexus papilloma have germline TP53 gene mutations, according to genetic analyses. [14] These cancers rarely exhibit nuclear p53 protein positivity. Aicardi syndrome, hypomelanosis of Ito, and 9p duplication are syndromic correlations of choroid plexus papilloma.
A neurosonogram via the anterior fontanelle will show an echogenic lesion inside the ventricles if the fontanelles are not united. This lesion exhibits bidirectional flow throughout the diastole, demonstrating blood flow via disorganized vascular arrangement. Sometimes ultrasound scans are used to diagnose the lesions before birth. [15] An isodense or slightly hyperdense lesion inside the ventricles, as well as the resulting ventriculomegaly, are visible on computer tomography (CT). [16] The intraventricular lobulated masses are well-defined and resemble fronds; they are hypointense on T1WI and hyperintense on T2WI on magnetic resonance imaging (MRI). [17] Active blood flow is indicated by the presence of flow voids. A rich vascularity gives the lesions a brilliant enhancing quality. Recent researches have shown that choroid plexus papilloma and choroid plexus cancer may be distinguished from one another using arterial spin labeling. [18]
There is disagreement about the best time to do surgery on tumors that were discovered accidentally. [19] An option may be an immediate surgical removal, or surgery might be postponed until follow-up imaging reveals radiographic abnormalities or hydrocephalus, or surgery might be a possibility if the patient starts to experience symptoms. Once hydrocephalus occurs, it is simpler to remove the tumor since there is more room around it and the path to the ventricles is shorter. Such a policy has the drawback that patients may experience focused deficiencies as a result of seizures, subarachnoid hemorrhage, or mass impact. In addition, patients often experience cognitive problems as tumor progress. [20] In symptomatic patients, since these tumors are benign, gross total resection (GTR) is the preferred course of therapy. Because of recent improvements in imaging, surgical techniques, and intensive care quality, the chance of a cure has reached nearly 100% . [4] Significant (12 percent) perioperative mortality occurs in the juvenile population, with catastrophic blood loss accounting for the majority of cases. [21] Preoperative embolization can improve the likelihood of a full tumor resection and reduce this risk. [22] Radiosurgery could be a therapy option. [23] To decrease blood flow and increase tumor resectability, percutaneous stereotactic intratumoral embolization with a sclerosing agent has also been tried. [24] Although it is rarely used, adjuvant chemotherapy can prolong survival and prevent recurrence. [25] Irradiation followed by subtotal resection may be used to treat a developing residual choroid plexus papilloma, making it more likely to success. Malignant tumors and those with leptomeningeal dissemination require adjuvant treatment as well. [26] Bevacizumab is playing a bigger part in disseminated choroid plexus papilloma, according to recent research. [27]
Choroid plexus papilloma differentials include the following: [28] Meningioma, Chordoid glioma, Rosette-forming glioneuronal tumor, Central nervous system lymphoma Metastasis, Central neurocytoma, Intraventricular tumors such as papillary ependymoma, Subependymoma, Subependymal giant cell tumor, Choroid plexus tumors, Medulloblastoma.
The prognosis of patients has been significantly enhanced by advances in surgical and critical care treatments. [29] Maximum tumor removal is associated with improved progression-free and overall survival. [30] Recurrences are uncommon. Although uncommon, reports of suprasellar metastases and craniospinal seeding have been made. [31]
Children who have had their intracranial pressure elevated for a long time may come with symptoms such papilledema, optic atrophy, and vision loss that may not improve after surgery. [32] Some may experience postoperative convulsions, bleeding, and cognitive impairments. [19] [33] Additionally, reports of postoperative CSF rhinorrhea have been reported. [34]
Cerebrospinal fluid (CSF) is a clear, colorless body fluid found within the tissue that surrounds the brain and spinal cord of all vertebrates.
Hydrocephalus is a condition in which an accumulation of cerebrospinal fluid (CSF) occurs within the brain. This typically causes increased pressure inside the skull. Older people may have headaches, double vision, poor balance, urinary incontinence, personality changes, or mental impairment. In babies, it may be seen as a rapid increase in head size. Other symptoms may include vomiting, sleepiness, seizures, and downward pointing of the eyes.
Intracranial pressure (ICP) is the pressure exerted by fluids such as cerebrospinal fluid (CSF) inside the skull and on the brain tissue. ICP is measured in millimeters of mercury (mmHg) and at rest, is normally 7–15 mmHg for a supine adult. The body has various mechanisms by which it keeps the ICP stable, with CSF pressures varying by about 1 mmHg in normal adults through shifts in production and absorption of CSF.
The choroid plexus, or plica choroidea, is a plexus of cells that arises from the tela choroidea in each of the ventricles of the brain. Regions of the choroid plexus produce and secrete most of the cerebrospinal fluid (CSF) of the central nervous system. The choroid plexus consists of modified ependymal cells surrounding a core of capillaries and loose connective tissue. Multiple cilia on the ependymal cells move to circulate the cerebrospinal fluid.
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 fourth ventricle. Rarely, ependymomas can occur in the pelvic cavity.
Choroid plexus cysts (CPCs) are cysts that occur within choroid plexus of the brain. They are the most common type of intraventricular cyst, occurring in 1% of all pregnancies.
The lateral ventricles are the two largest ventricles of the brain and contain cerebrospinal fluid (CSF). Each cerebral hemisphere contains a lateral ventricle, known as the left or right lateral ventricle, respectively.
A colloid cyst is a non-malignant tumor in the brain. It consists of a gelatinous material contained within a membrane of epithelial tissue. It is almost always found just posterior to the foramen of Monro in the anterior aspect of the third ventricle, originating from the roof of the ventricle. Because of its location, it can cause obstructive hydrocephalus and increased intracranial pressure. Colloid cysts represent 0.5–1.0% of intracranial tumors.
A cerebral shunt is a device permanently implanted inside the head and body to drain excess fluid away from the brain. They are commonly used to treat hydrocephalus, the swelling of the brain due to excess buildup of cerebrospinal fluid (CSF). If left unchecked, the excess CSF can lead to an increase in intracranial pressure (ICP), which can cause intracranial hematoma, cerebral edema, crushed brain tissue or herniation. The drainage provided by a shunt can alleviate or prevent these problems in patients with hydrocephalus or related diseases.
Intraventricular hemorrhage (IVH), also known as intraventricular bleeding, is a bleeding into the brain's ventricular system, where the cerebrospinal fluid is produced and circulates through towards the subarachnoid space. It can result from physical trauma or from hemorrhagic stroke.
Endoscopic third ventriculostomy (ETV) is a surgical procedure for treatment of hydrocephalus in which an opening is created in the floor of the third ventricle using an endoscope placed within the ventricular system through a burr hole. This allows the cerebrospinal fluid to flow directly to the basal cisterns, bypassing the obstruction. Specifically, the opening is created in the translucent tuber cinereum on the third ventricular floor.
Choroid plexus tumors are a rare type of cancer that occur from the brain tissue called choroid plexus of the brain. Choroid plexus tumors are uncommon tumors of the central nervous system that account for 0.5–0.6% of intracranial neoplasms in people of all ages. Choroid plexus papilloma, atypical choroid plexus papilloma, and choroid plexus carcinoma are the three World Health Organization types for these neoplasms. Children under the age of five account for 10% of cases of choroid plexus tumors. In children and adults, respectively, the lateral ventricle and the fourth ventricle are common locations, About 5% of all choroid plexus tumors are located in the third ventricle. Along with other unusual places such the cerebellopontine angle, the Luschka foramen, or brain parenchyma, the third ventricle is a rare location for choroid plexus tumors. Together, atypical choroid plexus papilloma, and choroid plexus carcinoma make up around 25% of all choroid plexus tumors. Although there have been reports of third ventricle choroid plexus papillomas in people in their fifth decade of life, only 14% of choroid plexus tumors are reported to arise in infants. Most findings indicate that choroid plexus tumors have no sex predilection.
A choroid plexus carcinoma is a type of choroid plexus tumor that affects the choroid plexus of the brain. It is considered the worst of the three grades of chord plexus tumors, having a much poorer prognosis than choroid atypical plexus papilloma and choroid plexus papilloma. The disease creates lesions in the brain and increases cerebrospinal fluid volume, resulting in hydrocephalus.
Bobble-head doll syndrome is a rare neurological movement disorder in which patients, usually children around age 3, begin to bob their head and shoulders forward and back, or sometimes side-to-side, involuntarily, in a manner reminiscent of a bobblehead doll. The syndrome is related to cystic lesions and swelling of the third ventricle in the brain.
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.
Astroblastoma is a rare glial tumor derived from the astroblast, a type of cell that closely resembles spongioblastoma and astrocytes. Astroblastoma cells are most likely found in the supratentorial region of the brain that houses the cerebrum, an area responsible for all voluntary movements in the body. It also occurs significantly in the frontal lobe, parietal lobe, and temporal lobe, areas where movement, language creation, memory perception, and environmental surroundings are expressed. These tumors can be present in major brain areas not associated with the main cerebral hemispheres, including the cerebellum, optic nerve, cauda equina, hypothalamus, and brain stem.
Papillary tumors of the pineal region were first described by A. Jouvet et al. in 2003 and were introduced in the World Health Organization (WHO) classification of central nervous system 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.
Central neurocytoma (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.
Angiocentric glioma (AG) refers to a rare neuroepithelial tumor when the superficial brain malignant cells enclose the brain vessels, commonly found in children and young adults. Initially identified in 2005 by Wang and his team from the University of Texas, AG was classified as Grade I by 2007 WHO Classification of Tumors of the Central Nervous System due to its benign clinical behavior, low proliferation index, and curative properties. AG primarily affects children and young adults at an average initial diagnosis age of 16 years old. Over 85% AG patients experience intractable seizures since childhood, especially partial epilepsy.
Diffuse leptomeningeal glioneuronal tumor (DLGNT) is a rare, primary CNS tumor, classified as distinct entity in 2016 and described as diffuse oligodendroglial-like leptomeningeal tumor of children in the 2016 classification of CNS neoplasms by the WHO., Typically, it's considered juvenile tumors but can occur in adults, the average age of diagnosis is five years. It's characterised by wide leptomeningeal spread with male predominance, like histopathology of neurocytoma, oligodendrocyte-like cytopathology, bland appearance, and severe clinical behaviour. Children's basal cisterns and inter-hemispheric fissures are typically involved in plaque like subarachnoid tumors. A common related intraparenchymal lesion is a spinal lesion. However, in certain situations, superficial parenchyma or Virchow-Robin gaps were affected.
Molecular and genetic investigations frequently show a combination of KIAA1549 and the serine/threonine protein kinase BRAF gene, and also deletions of the short arm of chromosome number 1 and/or the long arm of chromosome number 19.
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