Epidemiology:
"About 10% of all childhood brain tumors are Ependymomas." "60% of ependymomas that occur in lumbosacral region are not truly intramedullary."⁶
Etiology:
Tuberous clerosis is associated with Ependymomas.⁷
Signs & Symptoms:
Morning headaches, nausea, vomiting, coordination, increased intracranial pressure, abducens and oculomotor nerve defects.⁷
Diagnostic Procedures:
Workup as detailed by Chao, et. al. includes: complete history and physical, complete neurologic evaluation, CT or MRI head scan (or both) with contrast, positron emission tomography (PET) scan, complete blood count, cerebral spinal fluid chemistry tests, cytology and microbiology studies. Neurologic exam includes mental condition, coordination, sensation, reflexes and motor + cranial nerves. Ophthalmoscopy to check for papilledema, which indicates intracranial pressure. MRI contrast scan of the complete neuraxis for staging. Cerebral spinal fluid (CSF) cytology should be performed for any tumor that typically spreads through the CSF.This is particularly true of germ call tumor, primary neuroectodermal tumors (PNET), medulloblastoma and central nervous system (CNS) lymphoma.³
Histology:
These types of lesions have a histology related to the ependymal or subepedymal tissue that lines the ventricles and the canal of the spinal cord. The histological types are as follows: cellular, papillary, clear cell and tanycytic.⁴
Lymph Node Drainage:
There is no lymphatic vasculature in the CNS and tumors of the CNS do not metastsize through lymphatics.⁵
Metastatic Spread:
Ependymomas may occasionallly spread from the brain to the spinal cord in the cerebrospinal fluid (CSF).¹¹
Grading:
Grade 1- myxo-papillary ependymoma and subependymoma. Grade 2- ependymoma (low grade) Grade 3- anaplaxtic (malignant) ependymoma. The grade of a tumor refers to how abnormal the cancer cells look under a microscope and how quickly the tumor is likely to grow and spread. the pathologist determines the grade of the tumor using tissue removed for biopsy. The following grading system may be used for adult brain tumors. Grade l, The tumor grows slowly, has cells that liil similar to normal cells, and rarely spreads into nearby tissues. it may be possible to remove the entire tumor by surgery. Grade ll, the tumor grows slowly, but may spread into nearby tissue and may become a higher grade tumor. Grade lll, the tumor grows quickly, is likely to spread into nearby tissue, and the tumor cells look very different from normal cells. Grade lV, the tumor grows very aggressively, has cells that look very different from normal cells, and is difficult to treat successfully.¹²
Staging:

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.¹²
Supratentorial: The tumor is above the membrane that covers the cerebellum, known as the tentorium cerebella.
Infratentorial: The tumor is growing below the tentorium cerebella.
Recurrent: A recurrent tumor is a tumor that comes back after treatment
.
Radiation Side Effects:
Transient, reversible myelopathy within 2-6 months. A classic symptom of this is Lhermitte's sign, a shock like sensation, radiating to the hands and feet when the neck is flexed.
Chronic, progressive, or delayed myelopathy can occur months or years after radiation.
Progressive myelopathy occurs depending dose. Tolerances are 45Gy if using 1.8-2Gy fractions. Actual incidence of myelopathy is 0.2%-0.5% after 50 Gy, 1%-5% after 60 Gy, and 50% after 68-73 Gy.

Prognosis:
Most important indicators are age, performance status, and tumor grade¹⁰
Treatments:
Sugery is the preferred treatment with complete excision as the goal. For complete excision, no further treatment is needed.
Radiation is done post surgically, and only if there was incomplete or piecemeal excision.
Radiation dose: 50 Gy in 1.5 to 2.0 Gy fractions. If more than half of spinal cord is irradiated, total dose should not exceed 45 Gy
The spinal field can be treated with one PA field, or a wedged pair, whichever has better normal tissue sparing.
Tumors involving only the cervical spine can be treated with opposed lateral fields to avoid exposure to hypopharyx and oral cavity
.⁹

TD 5/5
TD 50/5
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Copy_of_CranioSpinal.JPG
Figure1. CranioSpinal

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Figure2. APandLaterals

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Figure3. CranioSpinal w/ Posterior Fossa Boost

References:
1. Philip Rubin, Clinical Oncology - A Multidisciplinary Approach for Physicians and Students. 7th edition. Philadelphia, PA: W.B. Saunders Company. 1993.
2. Washington CM, Leaver D, eds. Principles and Practice of Radiation Therapy. 2nd ed. St. Louis, MO: Mosby; 2004.

3. Chao KS, Perez CA., Brady LW. Radiation Oncology - Management Decisions. 2nd ed. Philadelphia, PA: Lippincott, Williams & Wilkins; 2002:132-133.
4. Nelson DF, Jenkins RB, Scheithauer BW, et. al. Central nervous system tumors. In: Rubin P, ed. Clinical Oncology: A Multidisciplinary Approach for Physicians and Students. 8th ed.Philadelphia, PA: W.B. Saunders Company; 2001:797.
5. Adams RD, Langlin L, Leaver D. Central nervous system. In: Washington CM, Leaver D, eds. Principles and Practice of Radiation Therapy. 2nd ed. St. Louis, MO: Mosby; 2004:726-727.
6. Chao KS, Perez CA., Brady LW. Radiation Oncology - Management Decisions. 2nd ed. Philadelphia, PA: Lippincott, Williams & Wilkins; 2002: Figure 1, 2 and 3

7. Massachusetts General Hospital www2.massgeneral.org Accessed January 13, 2010: Figure 3
8. Wikibooks en.wikibooks.org TD5/5 adapted from Emami 1991

9. Chao KS, Perez CA. Brady LW. Radiation Oncology - Management Decisions. 2nd ed. Philadelphia, PA: Lippincott, Williams & Wilkins. 2002
10. Washington CM, Leaver D. Principles and Practice of Radiation Therapy. 2nd ed. St. Louis, MO: Mosby. 2004

11. Chao KS, Perez CA. Brady LW. Radiation Oncology- Management Decisions. 2nd ed. Philadelphia, PA: Lippincott, Williams & Wilkins. 2002
12.www.cancer.net/ependymoma.com. Acessed January 14, 2010