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- severe headache
- visual loss (due to papilledema)
- vomiting
- bilateral Babinski sign
- drowsiness (after several hours of the above symptoms)
- gait change (rotation of feet when walking)
- impaction/constipation
- back flexibility
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 ependymoma is the fourth ventricle. Rarely, ependymoma can occur in the pelvic cavity.
Syringomyelia can be caused by an ependymoma.
Ependymomas are also seen with neurofibromatosis type II.
There is a wide range of symptoms that patients show. Symptoms can lie dormant, but come about due to Obstructive hydrocephalus. These symptoms include:
- Intracranial pressure
- Headache
- Papilledema
- Vomiting
- Light headedness
- Impaired mental activity
- Gait instability
In rare and extreme cases, more severe symptoms can be observed:
- Memory disturbance
- Dementia
- Hemiparesis
- Seizures
- Hemorrhage
- Psychosis
The desire to eat normally becomes worse over time, leading to weight loss from vomiting. Nausea is seen in almost all cases of astroblastoma, especially in low-grade tumors.
Along with cranial pressure, patients exhibit noticeable lethargy, increasing in severity as the tumor progresses. In the first few months, morning activities are usually unaffected; over time, these effects become more pronounced, especially late at night. Lethargy can disrupt vital signs, depleting energy and desire to perform simple cognitive tasks.
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.
In anywhere from fifty to eighty percent of cases, the first symptom of an oligodendroglioma is the onset of seizure activity. They occur mainly in the frontal lobe.
Headaches combined with increased intracranial pressure are also a common symptom of oligodendroglioma. Depending on the location of the tumor, any neurological deficit can be induced, from visual loss, motor weakness and cognitive decline. A computed tomography (CT) or magnetic resonance imaging (MRI) scan is necessary to characterize the anatomy of this tumor (size, location, heter/homogeneity). However, final diagnosis of this tumor, like most tumors, relies on histopathologic examination (biopsy examination).
Pediatric ependymomas are similar in nature to the adult form of ependymoma in that they are thought to arise from radial glial cells lining the ventricular system. However, they differ from adult ependymomas in which genes and chromosomes are most often affected, the region of the brain they are most frequently found in, and the prognosis of the patients. Children with certain hereditary diseases, such as neurofibromatosis type II (NF2), have been found to be more frequently afflicted with this class of tumors, but a firm genetic link remains to be established. Symptoms associated with the development of pediatric ependymomas are varied, much like symptoms for a number of other pediatric brain tumors including vomiting, headache, irritability, lethargy, and changes in gait. Although younger children and children with invasive tumor types generally experience less favorable outcomes, total removal of the tumors is the most conspicuous prognostic factor for both survival and relapse.
Oligodendrogliomas are a type of glioma that are believed to originate from the oligodendrocytes of the brain or from a glial precursor cell. They occur primarily in adults (9.4% of all primary brain and central nervous system tumors) but are also found in children (4% of all primary brain tumors). The average age at diagnosis is 35 years.
Gangliogliomas are generally benign WHO grade I tumors; the presence of anaplastic changes in the glial component is considered to represent WHO grade III (anaplastic ganglioglioma). Criteria for WHO grade II have been suggested, but are not established. Malignant transformation of spinal ganglioglioma has been seen in only a select few cases. Poor prognostic factors for adults with gangliogliomas include older age at diagnosis, male sex, and malignant histologic features.
Ganglioglioma is a rare, slow-growing primary central nervous system (CNS) tumor which most frequently occurs in the temporal lobes of children and young adults.
The most common symptom of the papillary tumor is a headache. Because headaches are so common, most people think nothing of it. This is why brain tumors are so dangerous. There are not a lot of symptoms that go along with them so people tend to wait a long time before seeking medical help. Most of the time people will go see a doctor when their headaches become consistent and start to never go away. This symptom however occurs secondary to hydrocephalus, which is a result from compression of the cerebral aqueduct. The cerebral aqueduct is a narrow channel in the midbrain, which connects the third and fourth ventricles. When a tumor blocks the pathway of the cerebrospinal fluid, this will cause headaches in the patient. Often when hydrocephalus occurs, a shunt is put in place in order to alleviate the pressure. In one case study, an endoscopic third ventriculostomy was performed as a first line procedure to treat the hydrocephalus and also for diagnostic purposes.
In some cases, patients have had progressive diplopia, or double vision. Also, although not in all cases, patients sometimes suffer from nausea and vomiting.
The first symptoms of neuroblastoma are often vague making diagnosis difficult. Fatigue, loss of appetite, fever, and joint pain are common. Symptoms depend on primary tumor locations and metastases if present:
- In the abdomen, a tumor may cause a swollen belly and constipation.
- A tumor in the chest may cause breathing problems.
- A tumor pressing on the spinal cord may cause weakness and thus an inability to stand, crawl, or walk.
- Bone lesions in the legs and hips may cause pain and limping.
- A tumor in the bones around the eyes or orbits may cause distinct bruising and swelling.
- Infiltration of the bone marrow may cause pallor from anemia.
Neuroblastoma often spreads to other parts of the body before any symptoms are apparent and 50 to 60% of all neuroblastoma cases present with metastases.
The most common location for neuroblastoma to originate (i.e., the primary tumor) is in the adrenal glands. This occurs in 40% of localized tumors and in 60% of cases of widespread disease. Neuroblastoma can also develop anywhere along the sympathetic nervous system chain from the neck to the pelvis. Frequencies in different locations include: neck (1%), chest (19%), abdomen (30% non-adrenal), or pelvis (1%). In rare cases, no primary tumor can be discerned.
Rare but characteristic presentations include transverse myelopathy (tumor spinal cord compression, 5% of cases), treatment-resistant diarrhea (tumor vasoactive intestinal peptide secretion, 4% of cases), Horner's syndrome (cervical tumor, 2.4% of cases), opsoclonus myoclonus syndrome and ataxia (suspected paraneoplastic cause, 1.3% of cases), and hypertension (catecholamine secretion or renal artery compression, 1.3% of cases).
The signs and symptoms of brain tumors are broad. People with brain tumors will experience them no matter if the tumor is benign (not cancerous) or cancerous. Primary and secondary brain tumors present with similar symptoms, depending on the location, size, and rate of growth of the tumor. For example, larger tumors in the frontal lobe can cause changes in the ability to think. However, a smaller tumor in an area such as Wernicke's area (small area responsible for language comprehension) can result in a greater loss of function.
Symptoms present 1–36 months before diagnosis, and can vary depending on age, tumor grade, and location. Increased intracranial pressure can induce vomiting, headache, irritability, lethargy, changes in gait, and in children less than 2, feeding problems, involuntary eye movements, and hydrocephalus are often noticeable. Seizures occur in about 20% of pediatric patients. Loss of cognitive function and even sudden death could occur if the tumor is located at a crucial location for CSF flow. Pediatric ependymomas most often occur in the posterior cranial fossa, in contrast with adult ependymomas which usually occur along the spine. Ependymomas present as low-density masses on CT scans, and are hyperintense on T2-weighted MRI images.
Headaches as a result of raised intracranial pressure can be an early symptom of brain cancer. However, isolated headache without other symptoms is rarer, and other symptoms often occur before headaches become common. Certain warning signs for headache exist which make it more likely to be associated with brain cancer. These are as defined by the American Academy of Neurology: "abnormal neurological examination, headache worsened by Valsalva maneuver, headache causing awakening from sleep, new headache in the older population, progressively worsening headache, atypical headache features, or patients who do not fulfill the strict definition of migraine".
Papillary tumors of the pineal region (PTPR) were first described by A. Jouvet et al. in 2003 and were introduced in the World Health Organization (WHO) classification of Central Nervous System (CNS) 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.
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.
Neuroblastoma (NB) is a type of cancer that forms in certain types of nerve tissue. It most frequently starts from one of the adrenal glands, but can also develop in the neck, chest, abdomen, or spine. Symptoms may include bone pain, a lump in the abdomen, neck, or chest, or a painless bluish lump under the skin.
Occasionally neuroblastoma may be due to a mutation inherited from a person's parents. Environmental factors have not been found to be involved. Diagnosis is based on a tissue biopsy. Occasionally it may be found in a baby by ultrasound during pregnancy. At diagnosis the cancer has usually already spread. The cancer is divided into low, intermediate, and high risk groups based on a child's age, cancer stage, and what the cancer looks like.
Treatment and outcomes depends on the risk group a person is in. Treatments may include observation, surgery, radiation, chemotherapy, or stem cell transplantation. Low-risk disease in babies typically has a good outcome with surgery or simply observation. In high-risk disease chances of long term survival, however, are less than 40% despite aggressive treatment.
Neuroblastoma is the most common cancer in babies and the third most common cancer in children after leukemia and brain cancer. About 1 in every 7,000 children is affected at some point in time. About 90% of cases occur in children less than 5 years old and it is rare in adults. Of cancer deaths in children about 15% are due to neuroblastoma. The disease was first described in the 1800s.
Teratomas maybe found in babies, children, and adults. Teratomas of embryonal origin are most often found in babies at birth, in young children, and, since the advent of ultrasound imaging, in fetuses.
The most commonly diagnosed fetal teratomas are sacrococcygeal teratoma (Altman types I, II, and III) and cervical (neck) teratoma. Because these teratomas project from the fetal body into the surrounding amniotic fluid, they can be seen during routine prenatal ultrasound exams. Teratomas within the fetal body are less easily seen with ultrasound; for these, MRI of the pregnant uterus is more informative.
A mature teratoma is a grade 0 teratoma. Mature teratomas are highly variable in form and histology, and may be solid, cystic, or a combination of solid and cystic. A mature teratoma often contains several different types of tissue such as skin, muscle, and bone. Skin may surround a cyst and grow abundant hair (see dermoid cyst). Mature teratomas generally are benign; malignant mature teratomas are of several distinct types.
SCT is seen in 1 in every 35,000 live births, and is the most common tumor presenting in newborn humans. Most SCTs are found in babies and children, but SCTs have been reported in adults and the increasingly routine use of prenatal ultrasound exams has dramatically increased the number of diagnosed SCTs presenting in fetuses. Like other teratomas, an SCT can grow very large. Unlike other teratomas, an SCT sometimes grows larger than the rest of the fetus.
Sacrococcygeal teratomas are the most common type of germ cell tumors (both benign and malignant) diagnosed in neonates, infants, and children younger than 4 years. SCTs occur more often in girls than in boys; ratios of 3:1 to 4:1 have been reported.
Historically, sacrococcygeal teratomas present in 2 clinical patterns related to the child’s age, tumor location, and likelihood of tumor malignancy. With the advent of routine prenatal ultrasound examinations, a third clinical pattern is emerging.
- Fetal tumors present during prenatal ultrasound exams, with or without maternal symptoms. SCTs found during routine exams tend to be small and partly or entirely external. The internal SCTs are not easily seen via ultrasound, unless they are large enough to reveal their presence by the abnormal position of the fetal urinary bladder and other organs, but large fetal SCTs frequently produce maternal complications which necessitate non-routine, investigative ultrasounds.
- Neonatal tumors present at birth protruding from the sacral site and are usually mature or immature teratomas.
- Among infants and young children, the tumor presents as a palpable mass in the sacropelvic region compressing the bladder or rectum. These pelvic tumors have a greater likelihood of being malignant. An early survey found that the rate of tumor malignancy was 48% for girls and 67% for boys older than 2 months at the time of sacrococcygeal tumor diagnosis, compared with a malignant tumor incidence of 7% for girls and 10% for boys younger than 2 months at the time of diagnosis. The pelvic site of the primary tumor has been reported to be an adverse prognostic factor, most likely caused by a higher rate of incomplete resection.
- In older children and adults, the tumor may be mistaken for a pilonidal sinus, or it may be found during a rectal exam or other evaluation.
During prenatal ultrasound, an SCT having an external component may appear as a fluid-filled cyst or a solid mass sticking out from the fetus' body. Fetal SCTs that are entirely internal may be undetected if they are small; detection (or at least suspicion) is possible when the fetal bladder is seen in an abnormal position, due to the SCT pushing other organs out of place.
At birth, the usual presentation is a visible lump or mass under the skin at the top of the buttocks crease. If not visible, it can sometimes be felt; gently prodded, it feels somewhat like a hardboiled egg. A small SCT, if it is entirely inside the body, may not present for years, until it grows large enough to cause pain, constipation and other symptoms of a large mass inside the pelvis, or until it begins to extend out of the pelvis. Even a relatively large SCT may be missed, if it is internal, because the bony pelvis conceals and protects it. Mediastinal tumors, including teratomas, are similarly concealed and protected by the rib cage.
Some SCTs are discovered when a child begins to talk at about age 2 years and complains of their bottom hurting or feeling "poopy" when they ride in a car seat.
Other tumors can occur in the sacrococcygeal and/or presacral regions and hence must be ruled out to obtain a differential diagnosis. These include extraspinal ependymoma, ependymoblastoma, neuroblastoma and rhabdomyosarcoma.
Smaller SCTs with an external component, seen in prenatal ultrasounds or at birth, often are mistaken for spina bifida. Cystic SCT and terminal myelocystocele are especially difficult to distinguish; for more accurate diagnosis, MRI has been recommended.
The most common cancers in children are (childhood) leukemia (32%), brain tumors (18%), and lymphomas (11%). In 2005, 4.1 of every 100,000 young people under 20 years of age in the U.S. were diagnosed with leukemia, and 0.8 per 100,000 died from it. The number of new cases was highest among the 1–4 age group, but the number of deaths was highest among the 10–14 age group.
In 2005, 2.9 of every 100,000 people 0–19 years of age were found to have cancer of the brain or central nervous system, and 0.7 per 100,000 died from it. These cancers were found most often in children between 1 and 4 years of age, but the most deaths occurred among those aged 5–9. The main subtypes of brain and central nervous system tumors in children are: astrocytoma, brain stem glioma, craniopharyngioma, desmoplastic infantile ganglioglioma, ependymoma, high-grade glioma, medulloblastoma and atypical teratoid rhabdoid tumor.
Other, less common childhood cancer types are:
- Neuroblastoma (6%, nervous system)
- Wilms tumor (5%, kidney)
- Non-Hodgkin lymphoma (4%, blood)
- Childhood rhabdomyosarcoma (3%, many sites)
- Retinoblastoma (3%, eye)
- Osteosarcoma (3%, bone cancer)
- Ewing sarcoma (1%, many sites)
- Germ cell tumors (5%, many sites)
- Pleuropulmonary blastoma (lung or pleural cavity)
- Hepatoblastoma and hepatocellular carcinoma (liver cancer)