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Although brain tumours are rare in children, they are the second most common type of tumour (after leukaemia) and the most common form of solid tumour in children. The majority of childhood brain tumours occur in the posterior fossa (back of the skull). The most common types of tumour are pilocytic astrocytoma, that Ependymoma and the Medulloblastoma.
Pilocytic astrocytoma is the most common brain tumour in children (17 percent of all children's brain tumours). It occurs in around 1 in 100,000 children per year, usually between the ages of five and eight, and is classified by the World Health Organisation (WHO) as a ’low-grade« and therefore benign tumour (WHO grade I).
In principle, astrocytomas can occur in all areas of the brain. In children, however, it classically occurs in the brain stem or cerebellum, and in rare cases in the spinal cord. Pilocytic astrocytoma can have different genetic subgroups. In 70 per cent of cases, a mutation is found in the BRAF and KIAA1549 genes. These mutations lead to various signalling pathways in the tumour cells that promote the growth or «immortality» of the cell, for example. Pilocytic astrocytoma occurs more frequently (in 15 per cent of cases) with neurofibromatosis type I (Von Recklinghausen disease).
Ependymomas affect around 0.3 per 100,000 people per year. They mainly occur in the area of the posterior fossa in the immediate vicinity of the cerebral ventricles. In principle, however, they can also occur in all other areas of the brain and spinal cord. In children, however, 80 per cent of ependymomas are found in the posterior fossa, whereas in adults they tend to occur in the spinal cord.
Ependymomas are classified as «classic ependymomas» (WHO grade II) and «anaplastic ependymomas» (WHO grade III). These are therefore «higher grade» tumours. They usually occur in early childhood between the ages of two and five, but can theoretically occur up to the age of 40.
In the posterior fossa, a rough distinction is made between group A ependymomas (now also PF-EPN-A («posterior fossa ependymoma A»), mainly in younger children) and group B ependymomas (now PF-EPN-B («posterior fossa ependymoma B»), mainly in older children), whereby group A often has a worse prognosis and group B a better prognosis. Ependymoma does not occur more frequently in the context of familial syndromes.
The Medulloblastoma is the most common high-grade brain tumour in children and accounts for around 10 percent of all brain tumours. It usually occurs in Medulloblastoma occur between the ages of five and ten in the area of the cerebellum or the fourth ventricle of the brain. There are four subgroups that are categorised according to the presence of changes in genes for cell signalling pathways (so-called molecular subtypes). These are the wingless type (WNT type), the sonic hedge hog type (SHH, further divided into p53 wild type and p53 mutated (rare)) and then subgroups that are neither SHH nor WNT altered, namely non-WNT/non-SHH group 3 and non-WNT/non-SHH group 4.
These subgroups of the Medulloblastoma have different courses and different prognoses, with the WNT type having a better prognosis, the SHH type with p53 mutations a poor prognosis, the SHH type with wild-type p53 a good prognosis and groups 3 and 4 an intermediate to poor prognosis. Medulloblastomas can occur as part of familial syndromes (e.g. Gorlin syndrome, familial adenomatous polyposis or Li-Fraumeni syndrome), but usually develop spontaneously.
Brain tumours can manifest themselves in different ways. What they all have in common are signs that indicate increased intracranial pressure such as headaches, nausea and vomiting. Other signs include neurological deficits, which are particularly noticeable in the form of gait disorders, as well as disorders of fine motor skills (increasing «clumsiness»), double vision, squinting, eye movement disorders and speech and/or language comprehension disorders.
In infants, a brain tumour can present itself through rapid or unnatural head growth, a tense and bulging fontanel due to hydrocephalus, developmental delays and slight irritability. In rare cases, brain tumours can cause epileptic seizures.
Unfortunately, most symptoms of a brain tumour are rather unspecific. For example, vomiting due to a brain tumour can easily be misinterpreted as a sign of gastrointestinal flu. It usually takes six to twelve months before the symptoms can be correctly linked to an existing brain tumour.
The suspicion of a brain tumour is confirmed with imaging of the head (and spine) in the form of magnetic resonance imaging (MRI). The pilocytic astrocytoma is usually sharply demarcated, i.e. there is no invasion of other brain tissue. The same applies to the Ependymoma, This often grows around the so-called cranial nerves, which makes surgical removal difficult. The Medulloblastoma typically grows within or near the fourth ventricle of the brain. However, an exact diagnosis requires a tissue analysis of the tumour.
The tissue for tissue analysis is obtained during the operation. Our neuropathology department, which is the reference centre for childhood brain tumours in Switzerland, then analyses the tissue in detail. In the case of an ependymoma or medulloblastoma, it may be necessary to obtain and examine cerebrospinal fluid using a so-called lumbar puncture in order to rule out seeding of the tumour.
Brain tumours can primarily be treated by surgical removal (resection). However, treatment can also consist of a combination of surgery, radiotherapy and chemotherapy.
At the pilocytic astrocytoma surgical resection is the treatment of choice. Chemotherapy or radiotherapy is only necessary if the resection was incomplete and the tumour continues to grow - or if the tumour cannot be surgically removed due to its proximity to vital brain structures, for example. Nowadays, such tumours can also be treated with targeted therapy as part of chemotherapy, provided the tumour has certain genetic characteristics.
In the case of the Ependymomas complete removal of the tumour is also necessary. After the operation (and if no or only limited resection is possible), radiotherapy is initiated. These tumours react very poorly to chemotherapy, which is why it is rarely used.
The primary therapy of a Medulloblastoma is the complete surgical removal of the tumour. If this is not possible or only possible to a limited extent due to the close proximity to the cerebellum, the brain stem and the cerebrospinal fluid system, radiotherapy of the tumour area, any residual tumour and the entire spinal cord is carried out after the operation. In addition, the treatment programme is supplemented with chemotherapy. Follow-up treatment with chemotherapy or radiotherapy is not necessary in the case of Medulloblastoma strongly dependent on the molecular subtype. The use of targeted therapies for the subtypes of the Medulloblastoma is an area that is currently being researched.
The surgical technique is very similar for all these tumours. The children are positioned in the prone position under anaesthetic. The classic approach to the tumour in the posterior fossa is the so-called suboccipital approach. A skin incision is made in the centre of the back of the head and neck, the muscles are carefully detached, two small holes are drilled into the bone, which are then widened to create a so-called craniotomy (opening of the skull calvaria). The meninges are then opened and the tumour is exposed. The tumour is then carefully removed under the surgical microscope using an ultrasound scrambler and other micro-instruments. The skull plate is then reinserted, fixed with self-dissolving plates and the skin is closed using self-dissolving sutures.
As hydrocephalus is sometimes associated with a tumour in the posterior fossa, this must also be treated. During the operation in these cases, a tube is inserted into the ventricles of the brain to drain the cerebrospinal fluid outwards into a canister (known as extraventricular drainage). The operation takes three to five hours on average.
Possible complications following these operations include a pseudomeningocele (accumulation of cerebrospinal fluid under the skin), cerebrospinal fluid fistula (leakage of cerebrospinal fluid from the wound), secondary haemorrhage, infections and damage to the surrounding brain structures. In around 30 per cent of cases, despite successful tumour removal and insertion of an external ventricular drain, cerebrospinal fluid circulation may still be impaired. The children may require additional surgery so that cerebrospinal fluid that has accumulated can be drained into the abdominal cavity via a drainage tube (ventriculo-peritoneal shunt). Further information on this can be found in our section on the treatment of hydrocephalus.
Our experienced paediatric neurosurgery team will advise you in collaboration with our paediatric oncology colleagues to determine the best treatment for your child.
During the entire hospital stay, your child will be cared for by our paediatric neurosurgery team at UKBB. If your child was not admitted as an emergency via the emergency ward, he or she will be admitted one day before the operation and examined again by our paediatric neurosurgery team and the anaesthetists. If you wish, your child can sleep at home again afterwards. The next day, your child will be operated on and then transferred to the paediatric intensive care unit (1st floor) for monitoring.
If your child is admitted as an emergency and is awake, they will be closely monitored overnight in our paediatric intensive care unit and the operation will be performed the following day. Immediate emergency surgery is very rarely necessary.
On the day of the operation, a companion from the Pro UKBB Foundation's parent support service (BELOP) will be at your disposal. She will accompany you until your child has fallen asleep and show you where he or she will wake up. In the meantime, she will help you to find your way around the hospital. As soon as your child no longer needs close monitoring, he or she will be transferred to the paediatric surgery ward (2nd floor, ward B).
As a rule, your child will recover from the operation after five to seven days and will be allowed to go home within this period. In certain cases, your child will need neurorehabilitation. This will be evaluated by our team and organised if necessary. Further treatment will then be continued as determined by a paediatric tumour board with the various specialist disciplines. You can be with your child during the entire hospitalisation and contact the paediatric neurosurgery team with any questions, who will visit you and your child every day.
The prognosis of brain tumours depends on the type of tumour, the location, the extent of spread at the time of diagnosis and the extent of resection of the tumour.
The pilocytic astrocytoma has an excellent prognosis if completely removed. The tumour itself is low grade, i.e. it grows slowly and without infiltrating the tissue. It can therefore usually be completely removed and thus cured. The tumour also very rarely spreads. However, depending on the location of the tumour, brain structures may have been impaired or destroyed, either by the tumour itself or by its removal. This can lead to cognitive impairment and/or permanent neurological damage.
The forecast of the Ependymomas varies depending on the type of tumour. The location of the tumour and how much of it could be removed play a major role. With complete removal, long-term survival after ten years is around 50-70 per cent. The age of the children when the tumour occurs, the subgroup of the tumour and the grading of the tumour are also important. Due to the tumour's location in the immediate vicinity of the cerebellum and many important cranial nerves, there is an increased risk of damage to the cerebellum or cranial nerves. Furthermore, side effects such as growth problems and memory disorders can occur as a result of chemotherapy and radiotherapy.
The forecast of the Medulloblastoma with today's therapeutic approaches is good, with around 70 per cent of children surviving into adulthood. The prognosis is highly dependent on the degree of removal, the age at onset, any spread of the tumour and the molecular subgroup. The WNT type has an excellent prognosis, the SHH p53 non-mutated type has a good prognosis (up to 75 per cent survival), the SHH p53-mutated type has a rather poor prognosis, and groups 3 and 4 also have a rather poor prognosis. Group 3 tumours in particular have already formed offshoots in 30 percent of cases when they are discovered. Medulloblastoma has a recurrence rate of around 20 to 30 per cent, which is often associated with a poor prognosis. Unfortunately, as with ependymoma, the combined therapy of a medulloblastoma can result in further side effects (e.g. growth disorders, cranial nerve disorders, hearing loss, cognitive impairment).
Franceschi E, Hofer S, Brandes AA, Guzman R et al. EANO-EURACAN clinical practice guideline for diagnosis, treatment, and follow-up of post-pubertal and adult patients with medulloblastoma. Lancet Oncol. 2019;20(12):e715-e728. doi:10.1016/S1470-2045(19)30669-2
Hayden Gephart MG, Colglazier E, Paulk KL, Vogel H, Guzman R, Edwards MSB. Primary pediatric skull tumours. Pediatr Neurosurg. 2011;47(3):198-203. doi:10.1159/000330544
Gephart MGH, Taft BP, Giese A-K, Guzman R, Edwards MSB. Perioperative posterior reversible encephalopathy syndrome in 2 paediatric neurosurgery patients with brainstem ependymoma. J Neurosurg Pediatr. 2011;7(3):235-237. doi:10.3171/2010.12.PEDS10299
Greuter L, Guzman R, Soleman J. Typical Pediatric Brain Tumours Occurring in Adults-Differences in Management and Outcome. Biomedicines. 2021;9(4). doi:10.3390/biomedicines9040356
Soleman J, Kozyrev DA, Ben-Sira L, Constantini S, Roth J. Management of incidental brain tumours in children: a systematic review. Child's Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg. 2020;36(8):1607-1619. doi:10.1007/s00381-020-04658-8
Soleman J, Dvir R, Ben-Sira L, et al. MRI-based diagnosis and treatment of pediatric brain tumours: is tissue sample always needed? Child's Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg. 2021;37(5):1449-1459. doi:10.1007/s00381-021-05148-1
Soleman J, Roth J, Ram Z, Yalon M, Constantini S. Malignant transformation of a conservatively managed incidental childhood cerebral mass lesion: controversy regarding management paradigm. Child's Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg. 2017;33(12):2169-2175. doi:10.1007/s00381-017-3566-z
Roth J, Soleman J, Paraskevopoulos D, Keating RF, Constantini S. Incidental brain tumours in children: an international neurosurgical, oncological survey. Child's Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg. 2018;34(7):1325-1333. doi:10.1007/s00381-018-3836-4
Greuter L, Guzman R, Soleman J. Pediatric and Adult Low-Grade Gliomas: Where Do the Differences Lie? Child (Basel, Switzerland). 2021;8(11). doi:10.3390/children8111075
Ebel F, Greuter L, Guzman R, Soleman J. Transitional Care in Pediatric Brain Tumour Patients: A Systematic Literature Review. Child (Basel, Switzerland). 2022;9(4). doi:10.3390/children9040501
Ebel F, Greuter L, Licci M, Guzman R, Soleman J. Endoscopic and Endoscopically-Assisted Resection of Intraventricular Lesions Using a Neuroendoscopic Ultrasonic Aspirator. J Clin Med. 2021;10(17). doi:10.3390/jcm10173889
https://www.mdpi.com/journal/children/special_issues/Brain_Tumors_Children
Plexus tumours arise from and on the so-called choroid plexus. The choroid plexus is a structure in the four cerebral ventricles that produces cerebrospinal fluid. Plexus tumours are extremely rare; they account for less than one percent of all brain tumours, but about 10-15% in children. Most plexus tumours occur in the lateral ventricles, i.e. the two largest cerebrospinal fluid chambers, while around 40% of the remaining plexus tumours occur in the fourth ventricle and around 5% in the third ventricle.
Plexus tumours are divided into two categories: plexus papillomas and plexus carcinomas. Plexus papillomas are generally low-grade tumours and account for the majority of plexus tumours (approx. 60%). It is not known exactly how plexus papillomas develop; it is currently assumed that it is due to excessive growth of the choroid plexus. Plexus carcinomas are high-grade tumours of the choroid plexus.
Mutations in cell growth inhibitors play an important role in their development. Plexus tumours frequently lead to an overproduction of cerebrospinal fluid and are therefore often associated with hydrocephalus (see «Hydrocephalus») is associated.
Plexus tumours can manifest themselves with various signs. As these tumours produce an increased amount of cerebrospinal fluid, they often lead to a so-called hydrocephalus (see «Hydrocephalus»), which leads to symptoms of increased intracranial pressure (headaches, nausea and vomiting). In infants, hydrocephalus can lead to rapid or abnormal head growth, a stretched and bulging fontanel, developmental delays or mild irritability.
Neurological deficits may also occur, in particular gait disorders, disorders of fine motor skills (increasing «clumsiness»), double vision, strabismus, disorders of eye movements and disorders of speech and/or speech comprehension. In rare cases, brain tumours can cause epileptic seizures.
Unfortunately, most symptoms of a brain tumour are rather unspecific (e.g. vomiting, which can often be misinterpreted as a stomach flu), which often delays the correct identification of a tumour. It usually takes six to twelve months before the symptoms are correctly associated with the tumour.
The suspicion of a plexus tumour is usually confirmed with imaging of the head in the form of magnetic resonance imaging (MRI). However, the type of tumour cannot be determined with certainty by MRI, which is why surgery (either removal or taking a tissue sample) is usually required. All children with brain tumours also have an MRI of the entire spinal axis (MRI of the back) to rule out so-called drip metastases in the area of the spinal cord. The tissue analysis of the tumour is analysed in detail by our neuropathology department, Switzerland's reference centre for childhood brain tumours.
The treatment of brain tumours is primarily surgical removal of the tumour, but can also consist of a combination of surgery, radiotherapy and chemotherapy or only chemotherapy and/or radiotherapy. Plexus tumours are usually treated surgically, with plexus papillomas generally requiring no further treatment afterwards, while treatment for plexus carcinomas can be supplemented with radiotherapy and chemotherapy.
The operation is minimally invasive and can be performed using either a microscope or an endoscope. Endoscopic surgery is possible for smaller tumours that are not heavily supplied with blood. A small hole is drilled into the skull bone using a drill. A camera (known as an endoscope) is used to view the ventricular system through this hole. The plexus tumour is then identified and removed in the ventricular system.
The neurosurgeon sees the operation through the camera on the endoscope. The hole in the skull bone is then closed again and the muscles and skin are sutured shut. The procedure for microscopic surgery is very similar, except that instead of a drill hole, a small opening (approx. 5 cm in diameter, known as a craniotomy) is made in the skull and the tumour in the ventricle is then removed with the help of the microscope. The bone is then closed using absorbable plates and screws and the skin is sutured closed with self-dissolving stitches.
Our experienced paediatric neurosurgery team will advise you on the best treatment for your child in cooperation with our paediatric oncology colleagues. We perform all tumour operations using navigation, which means that we use computer images of the skull to obtain a millimetre-precise image of the skull and brain, which we use to guide us during the operation.
All children will have an MRI immediately after the operation, under the same anaesthetic, to assess the surgical removal of the tumour. If, unexpectedly, there is still a residual tumour, your child will be taken straight back to the operating theatre to have this removed. After the operation and MRI, children are monitored in the paediatric intensive care unit.
During the entire hospital stay, your child will be cared for by our paediatric neurosurgery team at UKBB. If your child is not admitted as an emergency via our emergency ward at UKBB, your child will be admitted one day before the operation and will be examined again by our paediatric neurosurgery team and the anaesthetists and can then sleep at home if you wish. Your child will be operated on the next day and will then be monitored in the paediatric intensive care unit (1st floor).
Children who are admitted as emergencies and are in an alert state are closely monitored overnight in our paediatric intensive care unit, after which the operation is often scheduled for the following day. Very rarely, the operation has to be performed immediately as an emergency.
On the day of the operation, you will be accompanied by parents from the Parental support service (BELOP) is available at the UKBB. They accompany the parents until the child has fallen asleep and show the parents where the child will wake up. In the meantime, the parent carer will help you to find your way around the hospital.
As soon as your child no longer requires close monitoring (usually the day after the operation), he or she will be admitted to the paediatric surgery ward (3rd floor, ward B). As a rule, your child will recover from the operation after five to seven days and will also be allowed to go home during this period. In certain cases, your child will need neurorehabilitation; this will be evaluated by our team and organised if necessary. Further treatment will then be continued as determined by a paediatric tumour board with the various specialist disciplines.
You can be with your child during the entire hospitalisation and ask questions to the paediatric neurosurgery team, who will visit you and your child every day.
Plexus papillomas are benign tumours and therefore have a very good prognosis. After surgery, most patients are cured and have a good long-term survival. Unfortunately, patients with a plexus carcinoma have a poorer prognosis, as the tumour can grow again despite treatment (so-called recurrence).
Although complications can occur during surgery, these are rare and occur in approximately 3% of cases. As plexus tumours often produce cerebrospinal fluid, around 30-40% of patients require a so-called ventriculo-peritoneal shunt even after complete tumour removal (see «Hydrocephalus»).
In rare cases, infections, an accumulation of cerebrospinal fluid under the skin (known as a cerebrospinal fluid cushion) or leakage of cerebrospinal fluid through the skin (known as a cerebrospinal fluid leak), haemorrhage in the area of the operation, a stroke and neurological deficits can occur. However, children have a great potential to recover from such complications.
Ebel F, Greuter L, Guzman R, Soleman J. Transitional Care in Pediatric Brain Tumour Patients: A Systematic Literature Review. Child (Basel, Switzerland). 2022;9(4). doi:10.3390/children9040501
Ebel F, Greuter L, Licci M, Guzman R, Soleman J. Endoscopic and Endoscopically-Assisted Resection of Intraventricular Lesions Using a Neuroendoscopic Ultrasonic Aspirator. J Clin Med. 2021;10(17). doi:10.3390/jcm10173889
Ebel F, Greuter L, Guzman R, Soleman J. Resection of brain lesions with a neuroendoscopic ultrasonic aspirator - a systematic literature review. Neurosurg Review. 2022 doi: 10.1007/s10143-022-01837-w
https://www.mdpi.com/journal/children/special_issues/Brain_Tumors_Children
Gliomas are the most common group of brain tumours and include all tumours that can arise from so-called glial cells (i.e. astrocytomas from astrocytes, oligodendrogliomas from oligodendrocytes, etc.). Gliomas can be categorised into low-grade and high-grade gliomas, whereby the molecular/genetic composition of the glioma often plays a role in the classification. Most gliomas show a mutation of the so-called isocitrate dehydrogenase (IDH). Other common mutations are 1p/19q code deletions, in which information on the genes has been deleted, TP53 mutations, in which an important cell growth inhibitor is missing, EGFR and TERT mutations, in which receptors that transmit cell signals are mutated, as well as BRAF mutations, in which a receptor for cell signals is also mutated.
Low grade gliomas can be divided into five entities:
For pilocytic astrocytoma, also a low grade glioma, please refer to the section «Tumours of the posterior fossa». Diffuse astrocytoma (with a so-called MYB mutation) is usually characterised by childhood epilepsies that cannot be treated with medication. Angiocentric glioma also typically presents with epilepsy and occurs mainly in children and teenagers. In the polymorphic low-grade neuroepithelial tumour of boys and the diffuse low-grade glioma, the so-called MAPK pathway, a receptor for cell signals, is mutated.
Low grade gliomas of childhood differ from low grade gliomas of adulthood in that they have different molecular-genetic markers and rarely mutate into high grade gliomas. It is not uncommon for low grade gliomas to be discovered incidentally (by chance) during an imaging scan, in which case they are referred to as incidentalomas. The treatment of incidentalomas in children is also different from that of adult patients.
Brain tumours can manifest themselves with various signs. What they have in common, however, are the signs of increased intracranial pressure (headaches, nausea and vomiting), neurological deficits with, in particular, gait disorders, disorders of fine motor skills (increasing «clumsiness»), double vision, squinting, disorders of eye movements and disorders of speech and/or speech comprehension. In infants, a brain tumour can manifest itself through rapid or unnatural head growth, a tense and bulging fontanel due to hydrocephalus (see «Hydrocephalus»), developmental delays or mild irritability. Low grade gliomas often also cause epileptic seizures.
Unfortunately, most symptoms of a brain tumour are rather unspecific (e.g. vomiting, which can often be misinterpreted as a stomach flu), which often delays the correct identification of a tumour. It usually takes six to twelve months before the symptoms are correctly associated with the tumour.
The suspicion of a brain tumour is usually confirmed with imaging of the head in the form of magnetic resonance imaging (MRI).
However, the type of tumour cannot be determined with certainty by MRI, which is why surgery (either removal or taking a tissue sample) is usually necessary.
A few childhood brain tumours can be diagnosed and treated using certain MRI criteria together with clinical signs without having to perform surgery first. All children with brain tumours also have an MRI of the entire spinal axis (MRI of the back) to rule out so-called drip metastases in the area of the spinal cord.
The tissue analysis of the tumour is analysed in detail by our neuropathology department, Switzerland's reference centre for childhood brain tumours.
The treatment of brain tumours consists primarily of surgical removal of the tumour, but can also include a combination of surgery, radiotherapy and chemotherapy or only chemotherapy and/or radiotherapy.
Low grade gliomas are usually removed surgically and, if completely removed, often require no further treatment. An exception are so-called incidentalomas, i.e. lesions/tumours that were discovered by chance and do not cause any neurological symptoms. In these cases, it is usual for children to undergo conservative treatment by means of clinical and MRI follow-up checks. If the tumour grows in size, fulfils certain criteria in the MRI findings or leads to symptoms, surgery to remove or biopsy the tumour is recommended.
The child is placed on its back and an incision is made to access the skull bone. Depending on the size and location of the tumour, the calvaria is then opened (the size of the opening depends on the size of the tumour), through which the tumour can then be removed. The skull cap, the muscles and the skin are then closed again. The bone is fixed using absorbable plates and screws. The skin is sutured closed with absorbable suture material.
Low grade gliomas that cannot be completely removed, usually because they are located in so-called eloquent brain areas (i.e. brain areas with an important neurological function) and a complete resection can result in serious neurological deficits, can be treated with chemotherapy and/or radiotherapy. As a rule, this is only done if growth of the residual tumour is detected during follow-up checks. Sometimes, in the event of recurrence or growth of a residual tumour, low-grade gliomas are operated on several times. For a few years now, our colleagues in pathology, who are the reference centre for the diagnosis of childhood brain tumours in Switzerland, have been able to analyse the genetics (so-called molecular genetics) of the tumour. In the case of certain genetic changes within the tumour, a so-called «targeted therapy» can then be initiated.
All children with brain tumours at the UKBB are discussed at our paediatric neuro-oncology board, consisting of specialists in paediatric neuro-oncology, paediatric neurosurgery, paediatric neurology, radiotherapy, pathology and paediatric oncology nursing staff, and are then seen and advised by our highly specialised team.
During the entire hospital stay, your child will be cared for by our paediatric neurosurgery team at UKBB. If your child has not been admitted as an emergency via our emergency ward at UKBB, he or she will be admitted the day before the operation and will be examined again by our paediatric neurosurgery team and the anaesthetists and can then sleep at home if you wish.
Your child will be operated on the next day and will then be transferred to the paediatric intensive care unit (1st floor) for monitoring. Children who are admitted as emergencies and are in an alert state are closely monitored overnight in our paediatric intensive care unit, after which the operation is often scheduled for the following day. Very rarely, the operation has to be performed immediately as an emergency.
On the day of the operation, one of our parent carers from the parent support service (BELOP) at UKBB will be at your disposal. They accompany the parents until the child has fallen asleep and show the parents where the child will wake up. In the meantime, they will help you to find your way around the hospital. As soon as your child no longer needs close monitoring (usually the day after the operation), he or she will be transferred to the paediatric surgery ward (3rd floor, ward B).
As a rule, your child will recover from the operation after five to seven days and will be allowed to go home within this period. In certain cases, your child will need neurorehabilitation, which will be evaluated and, if necessary, organised by our team. Further treatment will then be continued as determined by a paediatric tumour board with the various specialist disciplines.
You can be with your child during the entire hospitalisation and ask questions to the paediatric neurosurgery team, who will visit you and your child every day.
The prognosis of brain tumours depends on the type of tumour, the location, the extent of spread at the time of diagnosis and the extent of resection of the tumour.
For diffuse astrocytoma, angiocentric glioma and the polymorphic low-grade neuroepithelial tumour of boys, complete surgical removal is curative and they show very good long-term survival. The prognosis for diffuse low-grade glioma varies depending on the location, age and molecular (genetic) composition of the tumour.
Although complications can occur during surgery, these are rare and depend on where the tumour is located in the brain. The overall complication rate is approx. 3%; in the case of operations near important brain structures (eloquent areas), complications can also occur in 5-10% of cases. In rare cases, infections, an accumulation of cerebrospinal fluid under the skin (so-called cerebrospinal fluid cushion) or leakage of cerebrospinal fluid from the skin (so-called cerebrospinal fluid leak), hydrocephalus (see factsheet «Hydrocephalus»), haemorrhage in the area of the operation, stroke and neurological deficits may occur. Sometimes these complications can lead to further interventions. However, children have a much greater potential to recover from such complications.
Hayden Gephart MG, Colglazier E, Paulk KL, Vogel H, Guzman R, Edwards MSB. Primary pediatric skull tumours. Pediatr Neurosurg. 2011;47(3):198-203. doi:10.1159/000330544
Gephart MGH, Taft BP, Giese A-K, Guzman R, Edwards MSB. Perioperative posterior reversible encephalopathy syndrome in 2 paediatric neurosurgery patients with brainstem ependymoma. J Neurosurg Pediatr. 2011;7(3):235-237. doi:10.3171/2010.12.PEDS10299
Greuter L, Guzman R, Soleman J. Typical Pediatric Brain Tumours Occurring in Adults-Differences in Management and Outcome. Biomedicines. 2021;9(4). doi:10.3390/biomedicines9040356
Soleman J, Kozyrev DA, Ben-Sira L, Constantini S, Roth J. Management of incidental brain tumours in children: a systematic review. Child's Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg. 2020;36(8):1607-1619. doi:10.1007/s00381-020-04658-8
Soleman J, Dvir R, Ben-Sira L, et al. MRI-based diagnosis and treatment of pediatric brain tumours: is tissue sample always needed? Child's Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg. 2021;37(5):1449-1459. doi:10.1007/s00381-021-05148-1
Soleman J, Roth J, Ram Z, Yalon M, Constantini S. Malignant transformation of a conservatively managed incidental childhood cerebral mass lesion: controversy regarding management paradigm. Child's Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg. 2017;33(12):2169-2175. doi:10.1007/s00381-017-3566-z
Roth J, Soleman J, Paraskevopoulos D, Keating RF, Constantini S. Incidental brain tumours in children: an international neurosurgical, oncological survey. Child's Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg. 2018;34(7):1325-1333. doi:10.1007/s00381-018-3836-4
Greuter L, Guzman R, Soleman J. Pediatric and Adult Low-Grade Gliomas: Where Do the Differences Lie? Child (Basel, Switzerland). 2021;8(11). doi:10.3390/children8111075
Ebel F, Greuter L, Guzman R, Soleman J. Transitional Care in Pediatric Brain Tumour Patients: A Systematic Literature Review. Child (Basel, Switzerland). 2022;9(4). doi:10.3390/children9040501
Ebel F, Greuter L, Licci M, Guzman R, Soleman J. Endoscopic and Endoscopically-Assisted Resection of Intraventricular Lesions Using a Neuroendoscopic Ultrasonic Aspirator. J Clin Med. 2021;10(17). doi:10.3390/jcm10173889
https://www.mdpi.com/journal/children/special_issues/Brain_Tumors_Children
Gliomas are the most common group of brain tumours and include all tumours that can arise from so-called glial cells (i.e. astrocytomas from astrocytes, oligodendrogliomas from oligodendrocytes, etc.). Gliomas can be categorised into low-grade and high-grade gliomas, whereby the molecular/genetic composition of the glioma often plays a role in the classification. Most gliomas show a mutation of the so-called isocitrate dehydrogenase (IDH). Other common mutations are 1p/19q code deletions, in which information on the genes has been deleted, TP53 mutations, in which an important cell growth inhibitor is missing, EGFR and TERT mutations, in which receptors that transmit cell signals are mutated, as well as BRAF mutations, in which a receptor for cell signals is also mutated.
With high-grade gliomas A distinction is made between diffuse midline glioma, diffuse paediatric high-grade glioma (H3 and IDH wild type), also known as diffuse intrinsic pontine glioma (DIPG), and hemispheric glioma, which is typical in children.
Diffuse midline glioma is a high-grade (aggressive) tumour that migrates into the surrounding tissue and can also be found in the spinal cord. They are probably caused by a mutation of the so-called H3 gene (a gene responsible for the winding of genetic information, the DNA). Midline glioma accounts for around 20% of childhood tumours and around a third of tumours are high-grade. About 80% of midline gliomas grow in the bridge (also called the pons), which is part of the brain stem. However, they can also occur in the spinal cord. Affected children are usually between five and nine years old.
DIPG can occur throughout childhood. It usually develops and is located in the bridge. Similar to diffuse midline glioma, it affects children between the ages of five and nine and affects more girls than boys. Most DIPGs are histologically high-grade astrocytomas. DIPG is characterised by mutations in the H3 gene, TP53 or by an IDH wild type.
Diffuse hemispheric glioma is an aggressive tumour that often occurs in teenage years. It is characterised by a TP53 mutation. Hemispheric glioma is often associated with other genetic diseases, e.g. a TP53 mutation or so-called X-linked α-thalassaemia.
Compared to adult patients, it is very rare for high-grade gliomas to develop from low-grade gliomas (so-called malignant transformation). In rare cases, however, this is also possible in children.
Brain tumours can manifest themselves with various signs. What they have in common, however, are signs of increased intracranial pressure (headaches, nausea and vomiting), neurological deficits with, in particular, gait disorders, disorders of fine motor skills (increasing «clumsiness»), double vision, squinting, disorders of eye movements and disorders of speech and/or speech comprehension. In infants, a brain tumour can manifest itself through rapid or unnatural head growth, a tense and bulging fontanel due to hydrocephalus (see «Hydrocephalus»), developmental delays or mild irritability. In rare cases, brain tumours can also cause other symptoms.
Unfortunately, most symptoms of a brain tumour are rather unspecific (e.g. vomiting, which can often be misinterpreted as a stomach flu), which often delays the correct identification of a tumour. It often takes six to twelve months before the symptoms are correctly associated with the tumour, especially in the case of slow-growing brain tumours.
The suspicion of a brain tumour is usually confirmed with imaging of the head in the form of magnetic resonance imaging (MRI).
However, the type of tumour cannot be determined with certainty by MRI, which is why surgery (either removal or taking a tissue sample) is usually necessary.
A few childhood brain tumours (e.g. DIPG) can be diagnosed and treated using certain MRI criteria together with clinical signs without having to perform surgery first. All children with brain tumours also have an MRI of the entire spinal axis (MRI of the back) to rule out so-called drip metastases in the spinal cord area.
The tissue analysis of the tumour is analysed in detail by our neuropathology department, Switzerland's reference centre for childhood brain tumours.
The treatment of brain tumours consists primarily of surgical removal of the tumour, but can also include a combination of surgery, radiotherapy and chemotherapy or only chemotherapy and/or radiotherapy.
In the case of high-grade gliomas, the decision as to whether surgical removal or tissue removal (biopsy) should be performed depends on the location of the tumour. As a rule, the aim is to remove the tumour surgically as completely as possible, as tumour removal is the factor that most strongly influences the prognosis. The exact surgical technique depends on the location, but in most cases the child is placed on its back and an incision is made to access the skull bone. Depending on the size and location of the tumour, the calvaria is then opened (the size of the opening depends on the size of the tumour), through which the tumour can then be removed. The skull cap, the muscles and the skin are then closed again. The bone is fixed using absorbable plates and screws. The skin is sutured shut with absorbable suture material. In all children, an MRI is performed immediately after the operation, under the same anaesthetic, to assess the surgical removal of the tumour. If, unexpectedly, there is any residual tumour, your child will be taken straight back to the operating theatre to have it removed. After the operation and MRI, children are monitored in the paediatric intensive care unit.
The majority of high-grade gliomas require follow-up treatment with chemotherapy and/or radiotherapy in addition to surgery. Surgery is not an option for certain high-grade gliomas such as DIPG, as the tumours are located in hard-to-reach areas of the brain that are in the immediate vicinity of vital brain structures. Radiotherapy of the tumour with or without chemotherapy is the treatment of choice in these cases.
All children with brain tumours at the UKBB are discussed at our paediatric neuro-oncology board, consisting of specialists in paediatric neuro-oncology, paediatric neurosurgery, paediatric neurology, radiotherapy, pathology and paediatric oncology nursing staff, and are then seen and advised by our highly specialised team.
During the entire hospital stay, your child will be cared for by our paediatric neurosurgery team at UKBB. If your child has not been admitted as an emergency via our emergency ward at UKBB, he or she will be admitted the day before the operation and will be examined again by our paediatric neurosurgery team and the anaesthetists and can then sleep at home if you wish.
Your child will be operated on the next day and will then be transferred to the paediatric intensive care unit (1st floor) for monitoring. Children who are admitted as emergencies and are in an alert state are closely monitored overnight in our paediatric intensive care unit, after which the operation is often scheduled for the following day. Very rarely, the operation has to be performed immediately as an emergency.
On the day of the operation, one of our parent carers from the parent support service (BELOP) at UKBB will be at your disposal. They accompany the parents until the child has fallen asleep and show the parents where the child will wake up. In the meantime, they will help you to find your way around the hospital. As soon as your child no longer needs close monitoring (usually the day after the operation), he or she will be transferred to the paediatric surgery ward (3rd floor, ward B).
As a rule, your child will recover from the operation after five to seven days and will be allowed to go home within this period. In certain cases, your child will need neurorehabilitation, which will be evaluated and, if necessary, organised by our team. Further treatment will then be continued as determined by a paediatric tumour board with the various specialist disciplines.
You can be with your child during the entire hospitalisation and ask questions to the paediatric neurosurgery team, who will visit you and your child every day.
The prognosis of brain tumours depends on the type of tumour, the location, the extent of spread at the time of diagnosis and the extent of resection of the tumour.
In general, the prognosis for high-grade gliomas is limited, as the therapies available to us today are generally unable to cure the tumour. However, the prognosis often depends on the tumour location, the tumour genetics (molecular genetics) and also the extent of the resection that has been achieved.
Although complications can occur during surgery, these are rare and depend on where the tumour is located in the brain. The overall complication rate is around 3%. In operations near important brain structures (eloquent areas), complications can also occur in 5-10% of cases. In rare cases, infections, an accumulation of cerebrospinal fluid under the skin (so-called cerebrospinal fluid cushion) or leakage of cerebrospinal fluid (CSF) from the skin (so-called CSF leak), hydrocephalus (see «Hydrocephalus»), haemorrhage in the area of the operation, stroke and neurological deficits. Sometimes these complications can lead to further surgery. However, children have a much greater potential to recover from such complications.
Franceschi E, Hofer S, Brandes AA, Guzman R et al. EANO-EURACAN clinical practice guideline for diagnosis, treatment, and follow-up of post-pubertal and adult patients with medulloblastoma. Lancet Oncol. 2019;20(12):e715-e728. doi:10.1016/S1470-2045(19)30669-2
Hayden Gephart MG, Colglazier E, Paulk KL, Vogel H, Guzman R, Edwards MSB. Primary pediatric skull tumours. Pediatr Neurosurg. 2011;47(3):198-203. doi:10.1159/000330544
Gephart MGH, Taft BP, Giese A-K, Guzman R, Edwards MSB. Perioperative posterior reversible encephalopathy syndrome in 2 paediatric neurosurgery patients with brainstem ependymoma. J Neurosurg Pediatr. 2011;7(3):235-237. doi:10.3171/2010.12.PEDS10299
Greuter L, Guzman R, Soleman J. Typical Pediatric Brain Tumours Occurring in Adults-Differences in Management and Outcome. Biomedicines. 2021;9(4). doi:10.3390/biomedicines9040356
Soleman J, Dvir R, Ben-Sira L, et al. MRI-based diagnosis and treatment of pediatric brain tumours: is tissue sample always needed? Child's Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg. 2021;37(5):1449-1459. doi:10.1007/s00381-021-05148-1
Soleman J, Roth J, Ram Z, Yalon M, Constantini S. Malignant transformation of a conservatively managed incidental childhood cerebral mass lesion: controversy regarding management paradigm. Child's Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg. 2017;33(12):2169-2175. doi:10.1007/s00381-017-3566-z
Roth J, Soleman J, Paraskevopoulos D, Keating RF, Constantini S. Incidental brain tumours in children: an international neurosurgical, oncological survey. Child's Nerv Syst ChNS Off J Int Soc Pediatr Neurosurg. 2018;34(7):1325-1333. doi:10.1007/s00381-018-3836-4
Greuter L, Guzman R, Soleman J. Pediatric and Adult Low-Grade Gliomas: Where Do the Differences Lie? Child (Basel, Switzerland). 2021;8(11). doi:10.3390/children8111075
Ebel F, Greuter L, Guzman R, Soleman J. Transitional Care in Pediatric Brain Tumour Patients: A Systematic Literature Review. Child (Basel, Switzerland). 2022;9(4). doi:10.3390/children9040501
Ebel F, Greuter L, Licci M, Guzman R, Soleman J. Endoscopic and Endoscopically-Assisted Resection of Intraventricular Lesions Using a Neuroendoscopic Ultrasonic Aspirator. J Clin Med. 2021;10(17). doi:10.3390/jcm10173889
https://www.mdpi.com/journal/children/special_issues/Brain_Tumors_Children
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