Ependymoma

[6] || Grade I Grade II and III with a peak at 3 years[3] || Treatment: Primary treatment is surgery but should be delayed in infants until after chemotherapy External beam radiation therapy - Stage dependent [11] Proton beam therapy [11] Clinical trials Ages 1-21 include chemotherapy: vincristine, carboplatin, cyclophophamide, etoposide and cisplatin Stem cell [12] Treatment: Initial treatment is maximum surgical resection External beam: 2-4 weeks post-op surgery Typical dose: 54-59.4Gy [13] 3D conformal fields: 64.8 Gy Radiosurgery or Stereotactic radiotherapy Brachytherapy Glucocorticoids used during the beginning of radiation therapy [3,14] Chemotherapy used for recurrence – Alkylanie agents/ platinum compound [3] __<span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Treatment dose guidelines __ __<span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Treatment fields determined by location in brain __ __<span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Treatment field parameters __ <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Spine Superior Match the inferior border of the cranial field edge <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Inferior 2 vertebral bodies below the pre-op tumor extension <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Lateral 1 cm lateral to the lateral edge of each ipsilateral pedicle <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Boost: 2-3 cm margin defined by the pre-op tumor extension and/or involvement <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Limited Cranial field boost: 1-1.5 cm margin around the GTV [13] <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Cranial field Angle collimator on lateral fields to align posterior aspect of skull, external auditory canal and lateral canthus and/or use blocks/MLC <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Gantry can also be angles 2-3 degrees posterior off lateral to eliminate beam divergence into contralateral eye <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Spine field Superior border must match the inferior border of the cranial field <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">S3 must be included in the spine field <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">If 2 separate spine fields are needed to encompass length of spine, gap calculation is necessary for superior/inferior field separation [16,17] [ 18] <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px; line-height: 115%;">D2L, Brain PPT, slide 28 ||
 * Epidemiology: || <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%; font-weight: normal;">The third most common pediatric brain tumor is ependymoma. The average age of diagnosis is between the ages of 4 to 6 with one third being diagnosed before the age of 3. Ependymoma occurs in about 10 percent of pediatric brain tumor. According to Gupta, “The annual incidence of ependymoma is 2.6 per million for 0-14 age group, and 2.2 per million for the 0-20 age group. Ependymoma of the spinal cord also occurs approximately 10 percent in children and young adults. Spinal cord ependymoma rarely occurs in children under 10 years old, but after the age of 10 the percentage increases. Also ependymoma is twice as common in males as in females. [1, 2] ||
 * Etiology: || <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%; font-weight: normal;">The etiology of ependymoma is still uncertain. The reason for this is because ependymoma cases are classified under other brain tumors, which makes it difficult to determine the risk factors. The only known cause of ependymoma is a genetic disorder called neurofibromatosis type 2 (NF2). [1, 2] ||
 * Signs & Symptoms: || [[image:ependymoma[1].jpg width="560" height="254"]] ||
 * Diagnostic Procedures: || * <span style="color: #b71a1a; font-family: 'Times New Roman',Times,serif; font-size: 120%; font-weight: normal;">Complete History and Work up [3,4]
 * <span style="color: #b71a1a; font-family: 'Times New Roman',Times,serif; font-size: 120%; font-weight: normal;">General Physical exam [3,4]
 * <span style="color: #b71a1a; font-family: 'Times New Roman',Times,serif; font-size: 120%; font-weight: normal;">Complete Blood work (CBC & chemistries)
 * <span style="color: #b71a1a; font-family: 'Times New Roman',Times,serif; font-size: 120%; font-weight: normal;">Complete Neurologic examination- Assess mental status, coordination, reflexes, & motor and cranial nerves [3,4]
 * <span style="color: #b71a1a; font-family: 'Times New Roman',Times,serif; font-size: 120%; font-weight: normal;">Opthalmoscopy – check for papilladema associated with ↑ cranial pressure [3]
 * <span style="color: #b71a1a; font-family: 'Times New Roman',Times,serif; font-size: 120%; font-weight: normal;">CT Scan of brain with and without contrast shows bony extension for disease if present [3,4]
 * <span style="color: #b71a1a; font-family: 'Times New Roman',Times,serif; font-size: 120%; font-weight: normal;">MRI – T1 weighted for anatomy delineation and T2 weighted for edema and tumor localization [3,4]
 * <span style="color: #b71a1a; font-family: 'Times New Roman',Times,serif; font-size: 120%; font-weight: normal;">Biopsy – when and where possible depending on location and size. Either stereotactic with needle or open craniotomy.
 * <span style="color: #b71a1a; font-family: 'Times New Roman',Times,serif; font-size: 120%; font-weight: normal;">Post Fossa Tumors – Indicate CSF spinal axis staging, (CSF cytology, preoperative Myelogram / MRI) [3,4]
 * Histology: || <span style="color: #ff0066; font-family: 'Times New Roman','serif'; font-size: 16px; line-height: 115%;">An ependymomas originate from ependymal cells from the central nervous system. The World Health Organization defines ependymomas based off of histological appearances. The following are the WHO grades: grade I myxopapillary ependymoma and subependymoma, grade II ependymoma (cellular, papillary, and clear cell differences), and grade III anaplastic ependymoma. [7] ||
 * Lymph Node Drainage: || ** <span style="color: #4f6228; font-family: 'Times New Roman',Times,serif; font-size: 120%;">There are no lymphatic channels in the brain and therefore do not metastasize through the lymphatic system [1] ||
 * Metastatic Spread: || <span style="color: #ff0066; font-family: 'Times New Roman','serif'; font-size: 16px; line-height: 115%;">Ependymomas may develop within the brain or spinal cord near or far from the ventricular system. The metastatic spread of ependymoma occurs infrequently. [2] ||
 * Grading: || <span style="color: #9900cc; font-family: 'Times New Roman',Times,serif; font-size: 120%; font-weight: normal;">Graded on a scale of I to III (World Health Organization [WHO] Grading system)
 * <span style="color: #9900cc; font-family: 'Times New Roman',Times,serif; font-size: 120%; font-weight: normal;">Subependymomas and myxopapillary ependymomas
 * <span style="color: #9900cc; font-family: 'Times New Roman',Times,serif; font-size: 120%; font-weight: normal;">The myxopapillary ependymoma generally present in the spinal canal of adults
 * <span style="color: #9900cc; font-family: 'Times New Roman',Times,serif; font-size: 120%; font-weight: normal;">Both tumors tend to grow slowly, especially the subependymoma
 * <span style="color: #9900cc; font-family: 'Times New Roman',Times,serif; font-size: 120%; font-weight: normal;">Classic ependymomas (Grade II) and anaplastic ependymoma (Grade III)
 * <span style="color: #9900cc; font-family: 'Times New Roman',Times,serif; font-size: 120%; font-weight: normal;">These occur mainly in the brain
 * <span style="color: #9900cc; font-family: 'Times New Roman',Times,serif; font-size: 120%; font-weight: normal;">These tumors grow more quickly than Grade I tumors [8] ||
 * Staging: || <span style="color: #9900cc; font-family: 'Times New Roman',Times,serif; font-size: 120%; font-weight: normal;">There is no formal staging system for ependymoma; however, it can be classified based on the location of the tumor and if the cancer has spread.
 * <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">// Supratentorial // : The tumor is above the membrane that covers the cerebellum known as the tentorium cerebella.
 * <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">// Infratentoria // l: The tumor is growing below the tentorium cerebella.
 * <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">// Recurrent // : A recurrent tumor is a tumor that comes back after treatment. [9] ||
 * Radiation Side Effects: || * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Slow growth and development
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Alopecia
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Headaches
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Seizures
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Learning disabilities
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Short term memory deficiency
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Difficulty with problem solving
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Loss of balance and/or trouble walking
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Otitis externa
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Serous otitis media
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Dermatitis
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Decreased visual acuity
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Decreased visual field
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Retinopathy
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Cataract
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Blindness
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Hormone insufficiency
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">High tone hearing loss
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Vestibular damage
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Mucositis
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Esophagitis
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Nausea and vomiting
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Fatigue
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Secondary tumor
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Death [10,3]
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Neurologic deterioration Can begin 6-12 months following radiation treatment.
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Radiation necrosis Can appear after 6 months following radiation treatment
 * Prognosis: || ** <span style="color: #4f6228; font-family: 'Times New Roman',Times,serif; font-size: 120%;">The three most important prognostic factors are age, performance status, and tumor type. Tumor grade is a stronger factor than size. The average 5 year survival is about 80% . ||
 * Treatments: || <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Childhood
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Adult **
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Primary: 50-60Gy Field size reduction at 45Gy
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Cranium alone: 50-54Gy
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Anaplastic: 55-60Gy
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Cervical spine:45Gy
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Boost dose to: 55-65Gy Small volumes of KNOWN residual SRT or Fractionated Stereotactic Radiotherapy
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Intraventricular: 45Gy
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Axis: 36-40Gy
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Craniospinal 40-45Gy with Boost to CTV to 50-54 (if positive MRI or CSF cytology)
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">3-D conformal 60-64.8Gy
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Junction shift every 10Gy[3,13,14,15]
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Bilateral or Medial cerebral hemisphere: Parallel opposed laterals
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Frontal lobe: AP and opposed wedged laterals, SAD
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Midcerebral: Parallel opposed AP/PA and laterals, SAD
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">With or without wedges
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Posterior Parietal or Occipital PA and lateral, wedged, SAD
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Temporal tip of lobe: Laterals [14]
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Cranium Superior 1-2 cm flash off cranium
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Inferior C2-C3 interface
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Posterior 1-2 cm flash off cranium
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Anterior 1-2 cm flash off cranium
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Central Axis Pineal region
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Cranium “Helmet” Superior 1-2 cm flash off cranium
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Inferior Lowest border of lowest cervical vertebra without treating ipsilateral shoulder
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Posterior 1-2 cm flash off cranium
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Anterior 1-2 cm flash off cranium
 * <span style="color: #0070c0; font-family: 'Times New Roman','serif'; font-size: 16px;">Central Axis Pineal region
 * TD5/5: || [[image:5_table[1]_(2).jpg width="635" height="208"]] ||

<span style="font-family: 'Times New Roman',Times,serif; font-size: 120%; font-weight: normal;">**Shun** Epidemiolgy and Etiology <span style="color: #cc0000; font-family: 'Times New Roman',Times,serif; font-size: 120%;">**Eric** Signs and Symptoms, Diagnostic procedures <span style="color: #ff0066; font-family: 'Times New Roman',Times,serif; font-size: 120%;">**Lisa** Histology and Metastatic spread <span style="color: #4f6228; font-family: 'Times New Roman',Times,serif; font-size: 120%;">**Curtis** Lymph node drainage, Prognosis and TD 5/5 <span style="color: #9900cc; font-family: 'Times New Roman',Times,serif; font-size: 120%;">**Kim** Grading and Staging <span style="color: #1111e8; font-family: 'Times New Roman',Times,serif; font-size: 120%;">**LaDonna** Side Effects and Treatment

<span style="font-family: 'Times New Roman',Times,serif; font-size: 120%;">**__References__** <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%; font-weight: normal;">[1] Albright AL, Adelson PD, Pollack IF. //Principles and Practice of Pediatric Neurosurgery.// 2nd edition. New York: Thieme Medical Publisher. 2008: 621 <span style="font-family: 'Times New Roman',Times,serif; font-size: 120%; font-weight: normal;">[2] Gupta N, Banerjee A, Haas-Kogan D. //Pediatric CNS Tumors//. 2nd edition. Springer-Verlag Berlin Heidelber, 2010:67-68 <span style="color: #cc0000; font-family: 'Times New Roman',Times,serif; font-size: 120%;">[3] Chaos KS, Perez CA, Brady LW. //Radiation Oncology-Management Decisions//. 2nd edition. Philadelphia:Lippincott, Williams & Wilkins. 2002 <span style="color: #cc0000; font-family: 'Times New Roman',Times,serif; font-size: 120%; font-weight: normal; margin-left: 0in;">[4] National Cancer Institute at the National Institutes of health. Available at: [] Accessed June 1, 2011 <span style="color: #cc0000; font-family: 'Times New Roman',Times,serif; font-size: 120%; font-weight: normal; margin-left: 0in;">[5] American Cancer Society. //Brain and spinal cord tumors in adults.// Available at: [] Accessed June 1, 2011 <span style="color: #cc0000; font-family: 'Times New Roman',Times,serif; font-size: 120%; font-weight: normal; margin-left: 0in;">[6] Nucleus catalog, //Papilledema.// Available at: [] Accessed on June 1, 2011 <span style="color: #ff0066; font-family: 'Times New Roman',Times,serif; font-size: 120%; line-height: 115%;">[7] Medscape Reference. //Ependymoma Background.// Available at : [|http://emedicine.medscape.com/article/277621-overview]Accessed on June 2, 2011 <span style="color: purple; font-family: 'Times New Roman',Times,serif; font-size: 120%; font-weight: normal;">[8] Cernfoundation. //Ependymoma// .Available at [|http://www.cern-foundation.org]. Accessed 5/31/11. <span style="color: purple; font-family: 'Times New Roman',Times,serif; font-size: 120%; font-weight: normal;">[9] Cancer.net. //Ependymoma//. Available at []. Accessed 5/31/11 <span style="color: #0070c0; font-family: 'Times New Roman',Times,serif; font-size: 120%;">[10]Medline Plus. Available at: [] Accessed on June 1, 2011 <span style="color: #0070c0; font-family: 'Times New Roman',Times,serif; font-size: 120%;">[11]The childhood brain tumor foundation. Available at: [] Accessed on June 1, 2011 <span style="color: #0070c0; font-family: 'Times New Roman',Times,serif; font-size: 120%; line-height: normal; margin-bottom: 0pt;">[12] Panchision, David M. Ph.D., (n.d.). //Cancer stem cells: A new paradigm for developing selective brain tumor treatmenst.// Available at: [] Accessed on June 1, 2011 <span style="color: #0070c0; font-family: 'Times New Roman',Times,serif; font-size: 120%;">[13] <span style="background-color: transparent; color: #0070c0; font-family: Times New Roman; font-size: 16px; text-decoration: none; vertical-align: auto;">Halperin E, Perez CA, Brady LW. //<span style="background-color: transparent; color: #0070c0; font-family: Times New Roman; font-size: 16px; text-align: left; text-decoration: none; vertical-align: auto;">Perez and Brady's Principles and Practice of Radiation Oncology //. 5th edition. Philadelphia:Lippincott, Williams & Wilkins. 2008

<span style="color: #0070c0; font-family: 'Times New Roman',Times,serif; font-size: 120%;">[14] University of Wisconsin-LaCross. DOS 431; D2L Brain PPT, slide 5 <span style="color: #0070c0; font-family: 'Times New Roman',Times,serif; font-size: 120%;">[15]BC Cancer Agency. //Ependymomas in adults.// Available at: [] Accessed on June 1, 2011 <span style="color: #0070c0; font-family: 'Times New Roman',Times,serif; font-size: 120%;">[16]Bentel, GC. Radiation therapy planning. 2nd edition. //Chapter 10, Treatment planning-central nervous system and pituitary gland.// <span style="background-color: transparent; color: #0070c0; font-family: 'Times New Roman',Times,serif; font-size: 120%; line-height: 115%; text-decoration: none; vertical-align: auto;">Macmillan Publishing Company; 1992

<span style="color: #0070c0; font-family: 'Times New Roman',Times,serif; font-size: 120%;">[17] Kahn, F. Treatment planning in radiation oncology. //Chapter 25, Cancers of the central nervous system//. <span style="background-color: transparent; color: #0070c0; font-family: 'Times New Roman',Times,serif; font-size: 120%; line-height: 115%; text-decoration: none; vertical-align: auto;">2nd ed. Philadelphia, PA; Lippincott Williams & Wilkins; 2007. <span style="background-color: transparent; color: #0070c0; font-family: 'Times New Roman',Times,serif; font-size: 120%; line-height: 115%; text-decoration: none; vertical-align: auto;">[18] University of Wisconsin-LaCross. DOS 431; D2L Brain powerpoint, slide5.