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Rhabdoid tumoursRRhabdoid tumoursRhabdoid tumoursEnglishNeurology;OncologyChild (0-12 years);Teen (13-18 years)BrainNervous systemConditions and diseasesAdult (19+)NA2009-07-10T04:00:00ZEric Bouffet, MD, FRCPCAnnie Huang, MD, PhD, FRCPC000Flat ContentHealth A-Z<p>This page describes a type of brain tumour called a rhabdoid tumour, what causes this kind of tumour, and some possible symptoms.<br></p><p>Rhabdoid tumours are rare but highly malignant tumours that occur mostly in young children. Rhabdoid tumours can occur in various parts of the central nervous system as well as in other parts of the body. In the brain, rhabdoid tumours can arise in the cerebral hemispheres, ventricles, suprasellar region, pineal gland, cerebellar hemispheres, cerebellopontine angle, and brain stem. Rarely, rhabdoid tumours can occur in the spinal cord.</p><h2>Key points</h2> <ul><li>In the brain, rhabdoid tumours can arise in the cerebral hemispheres, ventricles, suprasellar region, pineal gland, cerebellar hemispheres, cerebellopontine angle, and brain stem.</li> <li>Rhabdoid tumours can not be diagnosed with CT or MRI scans alone, and require a biopsy and a BAF47 staining to confirm diagnosis.</li> <li>Rhabdoid tumours can sometimes arise as part of a rhabdoid tumour predisposition syndrome.</li> <li>Symptoms can vary depending on the age of the child, and the location and size of the tumour.</li></ul>
Tumeurs rhabdoïdesTTumeurs rhabdoïdesRhabdoid tumoursFrenchNeurology;OncologyChild (0-12 years);Teen (13-18 years)BrainNervous systemConditions and diseasesAdult (19+)NA2009-07-10T04:00:00ZEric Bouffet, MD, FRCPCAnnie Huang, MD, PhD, FRCPC000Flat ContentHealth A-Z<p>Cette page décrit un type de tumeur cérébrale nommée tumeur rhabdoïde, les causes et les symptômes.<br></p><p>Les tumeurs rhabdoïdes sont rares, mais malignes. Elles atteignent surtout chez les jeunes enfants. Les tumeurs rhabdoïdes peuvent se manifester dans diverses parties du système nerveux central et dans d’autres parties du corps. Dans l’encéphale, les tumeurs rhabdoïdes peuvent se retrouver dans les hémisphères cérébraux, les ventricules, la région suprasellaire, le corps pinéal, les hémisphères cérébelleux, l’angle ponto-cérébelleux et le tronc cérébral. Veuillez consulter la page intitulée « Aperçu du cerveau » afin d’en apprendre davantage sur les diverses parties du cerveau. Rarement, les tumeurs rhabdoïdes peut atteindre la moelle épinière.</p><h2>À retenir</h2> <ul><li>Dans l’encéphale, les tumeurs rhabdoïdes peuvent se retrouver dans les hémisphères cérébraux, les ventricules, la région suprasellaire, la glande pinéale, les hémisphères cérébelleux, l’angle ponto-cérébelleux et le tronc cérébral.</li> <li>On ne peut diagnostiquer les tumeurs rhabdoïdes à l’aide d’examens de tomodensitométrie ou d’imagerie par résonance magnétique uniquement. Une biopsie et une coloration BAF47 doivent être effectuées afin de confirmer le diagnostic.</li> <li>Les tumeurs rhabdoïdes peuvent parfois survenir dans le cadre d’un syndrome prédisposant à une tumeur rhabdoïde.</li> <li>Les symptômes peuvent varier en fonction de l’âge de l’enfant et de l’emplacement et la taille de la tumeur.</li></ul>

 

 

Rhabdoid tumours1315.00000000000Rhabdoid tumoursRhabdoid tumoursREnglishNeurology;OncologyChild (0-12 years);Teen (13-18 years)BrainNervous systemConditions and diseasesAdult (19+)NA2009-07-10T04:00:00ZEric Bouffet, MD, FRCPCAnnie Huang, MD, PhD, FRCPC000Flat ContentHealth A-Z<p>This page describes a type of brain tumour called a rhabdoid tumour, what causes this kind of tumour, and some possible symptoms.<br></p><p>Rhabdoid tumours are rare but highly malignant tumours that occur mostly in young children. Rhabdoid tumours can occur in various parts of the central nervous system as well as in other parts of the body. In the brain, rhabdoid tumours can arise in the cerebral hemispheres, ventricles, suprasellar region, pineal gland, cerebellar hemispheres, cerebellopontine angle, and brain stem. Rarely, rhabdoid tumours can occur in the spinal cord.</p><h2>Key points</h2> <ul><li>In the brain, rhabdoid tumours can arise in the cerebral hemispheres, ventricles, suprasellar region, pineal gland, cerebellar hemispheres, cerebellopontine angle, and brain stem.</li> <li>Rhabdoid tumours can not be diagnosed with CT or MRI scans alone, and require a biopsy and a BAF47 staining to confirm diagnosis.</li> <li>Rhabdoid tumours can sometimes arise as part of a rhabdoid tumour predisposition syndrome.</li> <li>Symptoms can vary depending on the age of the child, and the location and size of the tumour.</li></ul><h2>What is a rhabdoid tumour?</h2><p>Rhabdoid tumours look a lot like other types of tumours on CT and MRI. It is not possible to tell the difference between these types of tumours using these imaging tests. The only way to determine that a tumour is really a rhabdoid tumour is to do a biopsy and a specific test called BAF47 staining.</p><h2>What causes rhabdoid tumours?</h2><p>Rhabdoid tumours can sometimes arise as part of a rhabdoid tumour predisposition syndrome. This means that some children are predisposed to developing this type of tumour. Most children with this type of tumour have a mutation or loss of a gene called INI1/hSNF5. The specific function of INI1 and its role in malignancy are not entirely clear. However, loss of INI1 expression is seen in almost all rhabdoid tumours. In some children with rhabdoid tumours, mutation or loss of INI1 is also detectable by a staining test on a biopsy sample.</p><p>Because rhabdoid tumours occur in infants and very young children, some researchers have suggested that these tumours could arise early in development, from the stem cell or germ cell stage.</p><h2>How many other children have rhabdoid tumours?</h2><p>Rhabdoid tumours occur in one to two out of every 100 children who have brain cancer. However, this type of tumour is most common in children under the age of three years. Rhabdoid tumours occur in 10 out of every 100 children under age three who have a tumour of the central nervous system.</p><p>Rhabdoid tumours usually present around age two. It is rare for this type of tumour to present in children older than age six years, although some have been reported in teenagers and even in adults.</p><h2>What are some medical symptoms of rhabdoid tumours?</h2><p>Symptoms can vary depending on the age of the child, and the location and size of the tumour. Infants may present with nonspecific signs of fatigue, vomiting, or failure to thrive. The child may tilt their head to the side. One non-specific symptom of increased cranial pressure is called cranial nerve 6 palsy, which is a paralysis of the sixth cranial nerve which leads to problems with moving the eye. In young children, a rapidly growing head size may be a sign of a fast growing tumour.</p><p>Children with rhabdoid tumour in the posterior fossa area of the brain may present with symptoms related to hydrocephalus: early morning headaches, vomiting, and lethargy. They may have problems with motor coordination.</p><p>Children older than three years may present with headache or hemiplegia, which is paralysis of one side of the body.</p><p>Because rhabdoid tumours are highly malignant, children usually only have progressive symptoms for days or weeks before diagnosis. It is unusual for symptoms to have been present for more than a few months.</p><p>For more Information please see:</p><ul><li> <a href="/Article?contentid=1329&language=English">Diagnosis of Rhabdoid Tumours</a></li><li> <a href="/Article?contentid=1368&language=English">Treatment of Rhabdoid Tumours</a></li></ul>Rhabdoid tumours

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