Ependymoma+(child)

Ependymomas signs and symptoms can vary from child to child depending on the size and location of the tumor.8 These include: 3 The diagnostic procedures for ependymomas are the same as many other Central Nervous System (CNS) abnormailites. These tests include: 3 Ependymoma tumors are tumors that arise directly from the ependymal cells located in the ventricular system. 4 Most of these tumors in children are indicated as intracranial neoplasms. 4 The image below demonstrates ependymal cells in a 32 week old fetus. 5 To help further delineate, these cells are located in a horizontal row with cilia extending into the ventricle.
 * **Epidemiolgy:** || Rachel
 * Ependymomas are derived from the ependymal cells lining the ventricular system; they occur throughout the CNS. They are seen infrequently in children. 1
 * In children, 90% of ependymomas are intracranial neoplasms; 60% to 70% arise in the posterior fossa, primarily within the fourth ventricle. ||
 * **Etiology:** || Rachel
 * The cause of ependmomas is unknown. Research is being carried out into possible causes. 2 ||
 * **Signs & Symptoms:** || Brandon
 * Headaches (especially upon wakening)
 * Nausea and vomiting (especially upon wakening)
 * Lethargy and irritability
 * Problems eating or walking
 * Enlarged head size or fontanelles (the soft spot in the skull bones before the head is fused). This usually only applies to infants. ||
 * **Diagnostic Procedures:** || Brandon
 * Physical Examination
 * Magnetic Resonance Imaging (MRI) scan
 * Computed Tomography (CT) scan
 * Biopsy- if a tumor is located to determine type and grade
 * Lumbar puncture (spinal tap)- this is useful in determining if any tumor cells have spread distally to the cerebrospinal fluid (CSF). ||
 * **Histology:** || Ashley

This image indicates a sample with ependymoma cells. The markers indicate the perivascular rosettes that characterize this cancer. 5

|| Because the brain doesn't contain lymph node channels, metastasis do not occur through lymphatics. 4 || Ependymomas rarely metastasis outside central nervous system. Most recurrences occur locally near the original tumor location. Extraneural metastasis mainly occurs in distant bone, lung, liver, and lymph nodes. 6 || World health organization's (WHO) classification for staging ependymal tumors is as follows: 7 There is no standard staging system for childhood ependymoma. 8 Instead, the plan for cancer treatment after surgery depends on the following: -Whether any cancer cells remain after surgery. -Whether the cancer has spread to other parts of the brain or spinal cord. -The age of the child || -Growth disturbances are common; growth hormone deficiency in most long term survivors 9 -adverse effects may be directly related to the biologically effective dose -Gradual onset of endocrine deficits (ex: growth hormone, thyroid-stimulating hormone, adrenocorticotropic hormone) -Late optic neuropathy or brain necrosis in < 10% of cases of patients treated to doses > 60Gy -Secondary malignant neoplasms -Hyperfractionated treatments are associated with moderate acute epithelial toxicity such as otitis and radioepidermitis -Late toxicities with high dose hyperfractionated treatments: neurocognitive deficits, hearing loss, leukoencephalopathy, diffuse microhemorrhages, dystrophic calcifications on MRI || Overall survival is approximately 55%. Age and extent of tumor resection are major prognostic factors.
 * **Lymph node drainage:** || Ashley
 * **Metastatic spread:** || Amanuel
 * **Grading:** || Amanuel
 * Grade I - Subepyndemoma
 * Grade I - Myxopapillary Ependymoma
 * Grade II - Ependymoma [variants include cellular, papillary, tanycytic, clear cell, and mixed]
 * Grade III - Anaplastic (malignant) Ependymoma ||
 * **Staging:** || Lindsey
 * **Radiation side effects:** || Lindsey
 * **Prognosis:** || Kevin

5 year survival rate: Infants < 1 year: 25% Children 1-4 yrs: 46% Children > 5 yrs: > 70% For patients with a complete tumor resection, survival rates are 60-80% following radiation treatment have been reported. For patient's with a partial tumor resection, survival rates drop to less than 30%. 10 || General Management
 * **Treatments:** || Kevin
 * Maximal surgical resection is the optimal intial therapy, although it possibly should be delayed in infants in whom response to initial chemotherapy may permit more complete "secondary" resection.
 * For supratentorial tumors, size and location may limit resectability.
 * Radiation therapy adds to disease control and survival. Two retrospective studies found the survival to be 0% and 13% with surgery alone compared with 45% and 59% with radiation.
 * Ependymomas are relatively sensitive to chemotherapy, especially alkylating agents and platinum compounds.

Radiation Therapy
 * Debate continues regarding the appropriate irradiation volume for intracranial ependymomas.
 * Disease contral rates in contemporary series show no advantage to full cranial or craniospinal volumes compared with wide local fields, based on modern imaging.
 * The standard local volume for posterior fossa ependymomas encompasses the entire posterior fossa, the inferior border is typically at the C2-3 interface.
 * For tumors extending into the upper cervical spine, the inferior margin should be two vertebral levels below the preoperative tumor extent until field reduction at 45 Gy.
 * Based on the recognized failure pattern at sites of identified invasion or residual disease, there now is greater acceptance of more localized treatment volumes.
 * For supratentorial ependymomas, wide local fields are defined by preoperative tumor extent, accounting for shifts in the normal brain postoperatively; margins of 2 to 3 cm are recommended.
 * For intraventricular extension, full ventricular irradiation (45 Gy) is appropriate.
 * Current guidelines call for 50 to 55 Gy to the primary tumor site, including field reduction at 45 Gy to more narrowly encompass the tumor bed.
 * Boost doses to 55-65 Gy have been recommended and are directed to small volumes of known residual disease, preferably using stereotactic radiosurgery or fractionated stereotactic irradiation. 11

Treatment planning images showing the isodose distribution of 6 field IMRT plan to posterior fossa. 12



||
 * **TD 5/5:** || Jenn
 * Tissue dose associated with 5% injury rate within 5 years ** . **(TD 5/5)** 13


 * Ear (Middle): Serous otitis 5000cGy
 * Ear (Vestibular): Meniere’s syndrome 6000cGy
 * Retina: Blindness 5500cGy
 * Cornea: Blindness 5000cGy
 * Lens: Blindness 500cGy
 * Salivary Glands: Xerostomia 5000cGy
 * Oral cavity and pharynx: Ulceration 6000cGy
 * Pituitary: Reduced hormone production 4500cGy
 * Brainstem: Necrosis, infarction 5000cGy ||
 * **References:** || # Chao KS, Perez CA, Brady LW. Radiation Oncology Management Decisions. 3rd ed. Philadelphia, PA: Lippincott, Williams, and Wilkins; 2011: 724.
 * 1) Ependymoma.Macmillan Cancer Support. []. Accessed July 5,2013.
 * 2) Ependymoma. Boston Children's Hopsital. .[]. Accessed July 3, 2013.
 * 3) Chao KS, Perez CA, Brady LW. //Radiation Oncology Management Decisions//. 3rd ed. Philadelphia, PA: Lippincott, Williams, and Wilkins; 2011: 724.
 * 4) Ependymal Cells. Introduction to Pathobiology of the Nervous System Web site. []. Accessed June 25, 2013.
 * 5) Kumar P, Rastogi N, Jain M, et al. Extraneural metastases in anaplastic ependymoma. http://www.cancerjournal.net/article.asp?issn=0973-1482;year=2007;volume=3;issue=2;spage=102;epage=104;aulast=Kumar. Accessed July, 2013 .
 * 6) <span style="color: #ff7100; font-family: Arial,Helvetica,sans-serif;">Histopathologic Classification of Childhood Ependymal Tumors. http://www.cancer.gov/cancertopics/pdq/treatment/childependymoma/HealthProfessional/page2. Accessed July 2, 2013.
 * 7) Childhood Ependymoma Treatment. http://www.cancer.gov/cancertopics/pdq/treatment/childependymoma/Patient/page2. Accessed July 3, 2013.
 * 8) Chao KS, Perez CA, Brady LW. //Radiation Oncology Management Decisions//. 3rd ed. Philadelphia, PA: Lippincott, Williams, and Wilkins; 2011: 733.
 * 9) eMedicine. Ependymoma. [] . Accessed June 27, 2013.
 * 10) Chao KS, Perez CA, Brady LW. //Radiation Oncology - Management Decisions//. 3rd ed. Philadelphia, PA: Lippincott, Williams and Wilkins; 2011: 724-725.
 * 11) Digitally Reconstructed Radiographs courtesy of Ginnie Dea, RT(T), Alta Bates Summit Comprehensive Cancer Center.
 * 12) Washington C, Leaver D. Principles and Practice of Radiation Oncology. 3rd ed. St. Louis, MO. Mosby Elsevier; 2010: 80-82. ||

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