|Tumeurs des cellules germinales||T||Tumeurs des cellules germinales||Germ cell tumours||French||Neurology;Oncology||Child (0-12 years);Teen (13-18 years)||Brain||Nervous system||Conditions and diseases||Adult (19+)||NA||2009-07-10T04:00:00Z||0||0||0||Flat Content||Health A-Z||<p>Cette page offre une brève description d’un type de tumeur cérébrale qui s’appelle la tumeur des cellules germinales.</p>||<p>Les tumeurs des cellules germinales se produisent surtout dans la région au-dessus de l’hypophyse (région suprasellaire) ou la région pinéale de l’encéphale. Il existe deux principaux types de tumeurs des cellules germinales : les germinomes et les tumeurs des cellules germinales non germinomateuses. Les germinomes sont le type le plus fréquent. Environ 60 à 70 % des tumeurs des cellules germinales sont des germinomes purs.</p>||<h2>À retenir</h2>
<ul><li>Il existe deux principaux types de tumeurs des cellules germinales : les germinomes et les tumeurs des cellules germinales non germinomateuses.</li>
<li>Pendant le développement normal d'un bébé, une cellule germinale se développe en un ovule (chez les filles) ou un spermatozoïde (chez les garçons), mais ces cellules migrent vers la mauvaise partie du corps du bébé, comme l'encéphale, où elles évoluent en tumeur.</li>
<li>Les symptômes des tumeurs des cellules germinales dépendent de l’emplacement dans l’encéphale où elles vont se loger.</li></ul>|
|Central nervous system germ cell tumours (CNS-GCT)||C||Central nervous system germ cell tumours (CNS-GCT)||Central nervous system germ cell tumours (CNS-GCT)||English||Neurology;Oncology||Child (0-12 years);Teen (13-18 years)||Brain||Nervous system||Conditions and diseases||Adult (19+)||NA||2022-01-10T05:00:00Z||9.80000000000000||53.9000000000000||1682.00000000000||Health (A-Z) - Conditions||Health A-Z||<p>This page gives an overview of the different types of germ cell tumours, what the symptoms of germ cell tumours are and how they are treated.</p>||<p>Germ cell tumours occur mostly in the area of the pituitary gland (suprasellar region) and/or in the pineal region of <a href="/article?contentid=1307&language=english">the brain</a>. The most common type of central nervous system germ cell tumour (CNS-GCT) is the germinoma. About 60% to 70% of germ cell tumours are germinomas.</p><h3>Non-germinomatous germ cell tumours (NGGCT)</h3><p>Non-germinomatous germ cell tumours (NGGCT) are sometimes called “mixed malignant germ cell tumours”. They are also called “secreting tumours” because they may secrete, or produce, a substance called alpha-fetoprotein (AFP) and/or human chorionic gonadotropin (hCG). </p><p>The terminology surrounding NGGCTs can be very complex and confusing. If your child is diagnosed with a NGGCT, their doctor may mention that the tumour consists of one or more of the following components:</p><ul><li>Embryonal carcinoma</li><li>Choriocarcinoma</li><li>Yolk sac tumour</li><li>Immature teratoma</li><li>Mature teratoma</li></ul><p>Each of these components must be addressed during treatment. </p><h3>Germinomas</h3><p>Germinomas do not secrete AFP and they may secrete hCG in lower levels with different threshold used in Europe (lower than 50 international units per litre, or IU/L) in America (lower than 100 IU/L) and Japan (lower than 200 IU/L). Germinomas may have sometimes mature teratoma components.</p><p>If germinomas contain anything other than mature teratoma germ cell tumour components, then it is not a germinoma. Instead, it is classified as a NGGCT, even if it contains some germinoma elements.</p>||<h2>Key points</h2><ul><li>In Europe and North America, two main types of germ cell tumours are distinguished: germinomas and non-germinomatous germ-cell tumours.</li><li>Symptoms of germ cell tumours depend on the part of the brain where the tumour is located.</li><li>Diagnosis of a germ cell tumour does not always require a biopsy.</li><li>Both germinoma and non-germinomatous germ cell tumours have relatively high treatment success rates.
</li></ul>||<h2>What are symptoms of germ cell tumours?</h2><p>Symptoms of germ cell tumours depend on the part of the brain where the tumour is located.</p><p>Germ cell tumours located in the pineal region generally have symptoms of increased intracranial pressure:</p><ul><li>Headaches increasing in frequency and severity </li><li>Nausea and vomiting increasing in frequency and severity </li><li>Blurred vision</li><li>Unusual movements of the eyes</li></ul><p>Germ cell tumours located in the basal ganglia can cause weakness on one side of the body.</p><p>Germ cell tumours in the suprasellar region cause problems with vision and often diabetes insipidus. Diabetes insipidus is not related to diabetes (mellitus) and a condition where the person pees and drinks a lot. There may be other endocrine problems such as delayed or precocious (early) puberty, which is the onset of puberty in girls and boys younger than eight or nine years of age respectively. Most children treated for a germ cell tumour in the suprasellar region of the brain will need multiple hormone replacement for the rest of their lives.</p>||<h2>What causes germ cell tumours?</h2><p>There are different scientific theories about what may cause a germ cell tumour but none of them has been proven with certainty.</p><h2>How many other children have a germ cell tumour?</h2><p>Germ cell tumours make up about 3% of paediatric tumours of the central nervous system in Europe and the United States. However, they are much more prevalent in Asian countries. In Japan, germ cell tumours make up 18% of brain tumours in people younger than 20 years old.</p><p>Germ cell tumours affect mostly teenagers and young adults. They are most often diagnosed around 13 to 15 years of age. They are much more common in boys than in girls.</p><p>In about half of children with a germ cell tumour, the tumour is located in a part of the brain called the pineal region. The second most common location is the suprasellar region (30%). About 10-20% will affect both locations; occurrence in the basal ganglia is very rare. </p>||<h2>How are germ cell tumours diagnosed?</h2><p>Unlike many other types of brain tumours, the diagnosis of a germ cell tumour does not always require testing of tumour tissue from a <a href="/article?contentid=1335&language=english">biopsy</a> (taking a small sample of tissue from the tumour). If a <a href="/article?contentid=1270&language=english">magnetic resonance imaging (MRI) </a>or <a href="/article?contentid=1272&language=english">computerized tomography (CT) scan</a> shows a tumour growing in the pineal or suprasellar region of the brain, this may already suggest that it is a germ cell tumour.</p><p>The most important type of testing is to determine whether tumour markers are increased. The presence of increased tumour markers is enough to confirm a diagnosis of germ cell tumour. Most brain tumour scientists agree that if a child has both evidence of a midline brain tumour on MRI and positive tumour markers as described above, there is no need for a biopsy to confirm the diagnosis.</p><p>When tumour markers are increased, the doctor can decide to start treatment without further testing.</p><p>If a child shows evidence of brain tumour without the elevated tumour markers, most doctors will do a biopsy to determine what type of tumour the child has.</p><h3>What is staging?</h3><p>Staging helps to determine the prognosis, or expected effect the tumour will have on your child, as well as the type of treatment plan that is most appropriate for your child.</p><p>Germ cell tumours can spread, and comprehensive staging investigations, including MRI of the brain and spine, analysis for tumour markers and investigation of CSF for malignant cells, are needed to determine the extent of disease.</p>||<h2>How are germ cell tumours treated?</h2><p>Once the health-care team has a clear understanding of what is causing your child’s symptoms, a meeting with the team will be set up to talk about results and the treatment plan. Remember that it is helpful to bring something to take notes with, such as a paper and a pen or laptop, at each meeting with this team. It is important to have the child’s primary caregivers in this meeting, for example both parents. </p><p>The treatment team may include a neurosurgeon, a neuro-oncologist, radiation oncologist, a nurse practitioner or nurse, and a social worker. During the meeting, they will explain which doctor is responsible for your child’s treatment, and the roles of everyone who is there. Other team members may be involved such as a dietician, pharmacist, occupational therapist, and physiotherapist, depending on your child’s needs. Every team member has their role in your child’s care, and everyone works together to make your child feel better. </p><p>The doctor will explain the type of tumour that your child has, based on what the team has learned through diagnostic testing. You will learn the expected effect this tumour will have on your child in the upcoming months and years, based on what is known about the tumour. This is called the prognosis. </p><p>The team may talk about placing your child on a protocol, which is a treatment plan for germ cell tumours. You will need to consent (agree) to the plan for the treatment to begin. Teenaged patients may be asked for their consent as well.</p><p>Your team will also talk to you about placing an IV line called a <a href="/article?contentid=52&language=english">central line</a>, this will help doctors give the treatments in a safer way and avoid multiple pokes for blood tests. You will get more information about the line insertion in details during the meeting. </p><p>The treatment of germ cell tumours depends on the type of germ cell tumour. <a href="/article?contentid=1351&language=english">Surgery</a> is required for a type of germ cell tumour called teratoma. For all other types of germ cell tumours, aggressive surgery may not be necessary. </p><p>Germinomas are very sensitive to <a href="/article?contentid=1357&language=english">chemotherapy</a> and <a href="/article?contentid=1353&language=english">radiation</a>. Non-germinomatous GCT require both chemotherapy and radiation therapy. Chemotherapy is generally given before radiation.</p><p>In North America, the treatment of a
<strong>germinoma</strong> may involve one of the following:</p><ul><li>A combination of chemotherapy and whole ventricular irradiation</li><li>Craniospinal radiation to the brain and spine</li></ul><p>The treatment of a <strong>NGGCT</strong> may involve one of the following:</p><ul><li>Chemotherapy and craniospinal radiation</li><li>Chemotherapy and focal radiation</li></ul><p>Surgery may be recommended for NGGCTs if they do not respond completely to chemotherapy.</p><p>The use of chemotherapy is sometimes complicated in children with diabetes insipidus, particularly when the chemotherapy given requires hydration. This can trigger an imbalance in the fluid electrolytes in the child’s body.</p><p>The testing of tumour markers continues regularly throughout treatment and follow-up.</p>|