Ependymoma+(child)

Headaches Seizures Nausea and vomiting Changes in vision, such as blurriness Difficulty with walking or balance Swelling of the nerve at the back of the eye Jerky eye movements Neck pain ||
 * **Epidemiolgy:** || Ependymomas are uncommon brain tumors accounting for less than 10 percent of all childhood brain tumors.[3] Ependyomas and arise in the ventricular system or CNS and few originate in the spinal cord. There is not a higher incidence in males verse females and occurs in children 0 to 5 years of age.
 * Derived form the ependymal cell in the ventricles systems. [1]
 * 90% of are intracranial neoplasms.[1]
 * 60% to 70% arise in posterior fossa.[1]
 * 50% of cases grow though foramen magnum, extending into the C1/C2 level. [1] ||
 * **Etiology:** || Ependymomas have no clear biological or environmental cause. A number of genetic mutations have been associated with ependymomas. Intramedullary ependymomas have been associated with neurofibromatosis type I. [1] Although little is known it is thought that ependymomas arise from a cytogennitc alteration involving chromosome 22. [3] ||
 * **Signs & Symptoms:** || Children with ependymoma can experience the following symptoms or signs[10]. Sometimes, children with ependymoma do not show any of these symptoms. Or, these symptoms may be cause by a medical condition that is not a tumor[10].
 * **Diagnostic Procedures:** || Doctors use many tests to diagnose a tumor and find out if it has metastasized (spread)[10]. Some tests may also determine which treatments may be the most effective[10]. For most tumors, a biopsy is the only way to make a definitive diagnosis of cancer[10]. If a biopsy is not possible, the doctor may suggest other tests that will help make a diagnosis[10]. Imaging tests may be used to find out whether the tumor has metastasized. The child’s doctor may consider these factors when choosing a diagnostic test[10]:

Age and medical condition

Type of tumor suspected

Severity of symptoms

Previous test results

In addition to a physical examination, the following tests may be used to diagnose ependymoma[10]:

Computed tomography (CT or CAT) scan. A CT scan creates a three-dimensional picture of the inside of the body with an x-ray machine. A computer then combines these images into a detailed, cross-sectional view that shows any abnormalities or tumors. Sometimes, a contrast medium (a special dye) is injected into a vein to provide better detail[10].

Magnetic resonance imaging (MRI). An MRI uses magnetic fields, not x-rays, to produce detailed images of the body. A contrast medium may be injected into a patient’s vein to create a clearer picture[10]. A spinal MRI may be used to determine if the tumor has spread to the spine[10].

Biopsy. A biopsy is the removal of a small amount of tissue for examination under a microscope. Other tests can suggest that cancer is present, but only a biopsy can make a definite diagnosis[10. The sample removed from the biopsy is analyzed by a pathologist (a doctor who specializes in interpreting laboratory tests and evaluating cells, tissues, and organs to diagnose disease)[10]. For ependymoma, surgery (see Treatment) is needed to get a sample of tissue[10].

Lumbar puncture (spinal tap). A lumbar puncture is a procedure in which a doctor takes a sample of cerebral spinal fluid (CSF) to look for cancer cells, blood, or tumor markers (substances found in higher than normal amounts in the blood, urine, or body tissues of people with certain kinds of cancer). CSF is the fluid that flows around the brain and the spinal cord[10]. Doctors generally give an anesthetic to numb the lower back before the procedure.

Learn more about what to expect when having common tests, procedures, and scans[10].

|| Ependymoblastoma is close in spelling but is more closely related to medulloblastoma and considers a Primitive Neuoectodermal Tumor (PNET).[1][2] [] || Primary brain tumors very rarely spread outside of the cranial cavity and are more likely to invade other areas of the brain. Brain metastases are spread from other areas of the body to the brain and are not associated with a primary brain lesion. [9] || __** Grade I: **__ __** Grade II and III: **__ __**Supratentorial:**__ The tumor is positioned above the membrane that covers the cerebellum (tentorium cerebella). __**Infratentorial:**__ The tumor is positioned below the tentorium cerebella. __**Recurrent:**__ The tumor has come back after treatment. || __** Neurocognitive problems: **__ __** Depression: **__ __** Hearing loss: **__ __** Pituitary and hypothalamic dysfunction: (there will be substantial scatter to the pituitary and a high risk of endocrinopathy) **__ __** Increased risk for cerebrovascular events (strokes): **__ __** Increased risk of second neoplasms: **__ __** Sparse growth of hair: **__ __** Hypoplasia of occipital region and reduced growth of upper cervical spine: **__ __** Ataxia: **__ __** Thyroid damage (due to scatter or exit dose RT): **__ __** Cataracts: **__ __** Spinal damage from RT (effect on spinal growth): **__ __** Infertility: **__ __** Damage to bone marrow reserve: **__ Poorer prognosis is related to loss of differentiating structures or a combination of necrosis, endothelial proliferation, and mitotic index >5. [1] The prognosis is better for brain ependymomas than for most other glial tumors. Five-year survival rates are approximately 80%, and 10-year survival rates are nearly 50%. Prognosis is worse for anaplastic ependymomas since they are more invasive and more likely to spread to by CSF pathways. [2] || Supratentorial and low-grade tumors can be treated with a 2cm margin using 3D conformal techniques. Doses of 54-59.4Gy are recommended. [3] Craniospinal treatment can be used if CSF seeding is noted. Craniospinal technique uses laterally opposed fields for the brain and CSF spaces from the retro-orbital space down through the mid-cervical cord. The couch is angled to compensate for divergence over the spinal cord. The divergence of the spinal radiation field determines the collimator angle for the brain fields. Depending on height, two posterior fields may be used to encompass the spine down to the level of the 2nd or 3rd sacral segment where the CSF space ends. The patient is usually treated in a prone position. 150 to 180cGy/day is given. [3] Coronal dose distribution [4] Sagittal dose distribution [4] || Brain: Whole 4500 cGy, 2/3 5000 cGy, 1/3 6000 cGy Ear (acute serous otitis: Whole 3000 cGy, 2/3 3000 cGy, 1/3 3000 cGy Ear (chronic serous otitis: Whole 5500 cGy, 2/3 5800 cGy, 1/3 6000 cGy Lens: Whole 1000 cGy Optic chiasm: Whole 5000 cGy Optic nerve: Whole 5000 cGy Retina: Whole 4500 cGy [8] || 2. Lenhard RE, Osteen R, Gansler T. The American Cancer Society’s Clinical Oncology. Williston, VT: Blackwell Publishing, Inc; 2001. 3. Washington CM, Leaver D. Principles and Practice of Radiation Therapy. St. Louis, MO: Mosby; 2010. 4. Ependymoma. Pediatric Oncology Education Materials. Available at: []. Accessed on June 25, 2012. 5. Ependymoma fast facts. CERN Foundation. Available at: []. Accessed June 25, 2012. 6. Ependymoma- Childhood. Cancer.net. Available at: []. Accessed June 25, 2012. 7. Ependymoma. Pediatric Oncology Education Materials. Available at: []. Accessed June 25, 2012. 8. RadiationOncology/Toxicity/Emami.Available at: []. Accessed June 25, 2012. 9. Brain Cancer Overview. Available at: []. Accessed June 28 2012 10.Ependymoma-Children. July 11, 2012. Available at: []. Accessed on: June 28, 2012. ||
 * **Histology:** || Found in the ventricles of the CNS system, ependymal cells are the cellular origin of ependymomas. While 90% of ependymomas are intracranial they can be anywhere in the Central Nervous System(CNS). [1] There are four classifications of ependymomas:
 * Ependymoma: can be cellular, papillary, epithelial, clear-cell, or a mix of the four
 * Anaplastic ependymoma
 * Myxopapillary ependymoma: usually occur at the distal end of the spinal cord
 * Subependymoma [2]
 * **Lymph node drainage:** || There are no true lymphatic pathways from the brain.[3] ||
 * **Metastatic spread:** || ====== Since ependymomas arise from the lining of the CNS, seeding through the Cerebral Spinal Fluid (CNS) can be a concern and should be evaluated. [4] ======
 * **Grading:** || Ependymoma tumors are graded on a scale of I to III by the World Health Organization (WHO) grading system. [5]
 * Subependymomas and Myxopapillary Ependymomas: both tumors tend to grow slowly, especially the Subependymoma.
 * Classic Ependymomas (Grade II), and Anaplastic Ependymoma (Grade III): these occur mainly in the brain, and grow quicker than the Grade I tumors. ||
 * **Staging:** || There is no formal staging system for ependymoma. We can, however, classify where in the brain the tumor is positioned and whether it has spread. [6]
 * **Radiation side effects:** || Some of the late effects of Radiation Therapy (RT) for an Ependymoma (depending on the area of RT) are: [7]
 * Poor short term memory
 * Difficulty with executive function
 * Usually related to multiple long-term health problems and neurocognitive dysfunction
 * Direct RT related damage to the Cochlea
 * Compounded sometimes by previous Cisplatin
 * Metabolic syndrome (with hypertension)
 * GH deficiency (common with failure to grow)
 * TSH deficiency
 * LH/FSH deficiency
 * ACTH deficiency
 * RT effect on cerebral vasculature
 * RT induced Meningioma is common
 * RT induced malignancy is less common
 * Affects posterior fossa where high dose RT is given
 * Due to posterior fossa boost
 * due to posterior fossa boost
 * Chronic ataxia can occur as a result of Cerebellar atrophy
 * Related to posterior fossa boost
 * Hypothyroidism
 * Thyroid cancer and multinodular goiter
 * Lens of the eye is very sensitive to RT
 * Shorter height
 * Scoliosis and kyphosis of spine (pain)
 * Increased risk of early spinal arthritis and osteoporosis
 * Exit dose from lower end of spinal field (damages ovaries or testicles)
 * __Cardiac damage__: **
 * Valvular stenosis form exit RT dose (rare)
 * After craniospinal RT, it’s difficult to tolerate further intensive chemotherapy
 * Bone marrow reserve is permanently damaged ||
 * **Prognosis:** || Better outcomes are correlated with total surgical resection and median infratentorial location. Adjuvant chemotherapy or radiation therapy significantly enhances progression-free survival in patients who received complete tumor resection. [1]
 * **Treatments:** || Surgery is the treatment of choice. A gross total resection is desirable, but is usually impossible due to the location of the tumor, especially when it involves structures at the floor of the 4th ventricle. Surgery is followed by radiation therapy. If surgery and radiation therapy fail, chemotherapy, particularly carboplatin can be tried. [2]
 * **TD 5/5:** || Brainstem: Whole 5000 cGy, 2/3 5300 cGy, 1/3 6000 cGy
 * **References:** || 1. Chao KS, Perez CA, Brady LW. Radiation Oncology: Management Decisions. Philadelphia, PA: Lippincott Williams & Wilkins; 2002.

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