eMedicine Specialties > Oncology > Carcinomas of the Central and Peripheral Nervous System
Ependymoma: Follow-up
Updated: Jan 26, 2009
Follow-up
Further Inpatient Care
- Patients with ependymomas who undergo surgical resection typically spend the night after surgery in an intensive care unit followed by an inpatient stay of 3-5 days. The final length of stay depends on each patient's neurological condition as well as tumor location and extent of resection.
- Postoperative antibiotics are usually continued for 24 hours, and deep vein thrombosis prophylaxis is continued until patients are ambulatory. Anticonvulsants are maintained at therapeutic levels throughout the inpatient stay for supratentorial ependymoma, while steroid dose is tailored to each patient's clinical status and gradually tapered pending improvement. Many patients benefit from occupational therapy and physical therapy/rehabilitation.
- While patients are still in the hospital, they should undergo postoperative imaging to determine the extent of surgical resection. This is best evaluated within 3 days of surgery by a contrast-enhanced MRI of the brain because contrast enhancement during this period accurately reflects residual tumor.
- In addition, patients should have an MRI of the entire spine with and without gadolinium to rule out seeding.
- If not performed preoperatively, complete evaluations by consulting physicians, including a neurooncologist and radiation oncologist, should be considered.
Further Outpatient Care
- Follow-up care with a rehabilitative medicine team is recommended for patients who sustain neurological deficits after spinal tumor resection.
- Children with posterior fossa tumors must be monitored for signs of hydrocephalus, and all patients with supratentorial tumors should have serum levels of anticonvulsant drugs checked on a regular basis.
Inpatient & Outpatient Medications
- For patients with supratentorial tumors, postoperative anticonvulsant medication is continued upon discharge.
- Steroids are usually tapered in accordance with the patient's clinical status and degree of edema documented on postoperative imaging.
Transfer
- At some institutions, transferring the patient to another facility may be necessary if the proper consultations cannot be obtained. In most cases, surgical resection can be performed on an urgent, but not emergent, basis.
Complications
- In general, brain tumor resection has an overall mortality rate of 1-2%; 40% of patients remain healthy or have minimal deficits after surgery, 30% manifest no postoperative change relative to preoperative deficits, and 25% of patients sustain increased postoperative deficits that most often improve.
- Children who undergo resection of a posterior fossa lesion are at risk for postoperative cerebellar mutism.
- Nonspecific complications that can occur in any location of tumor include hemorrhage, infection, and worsening of neurological deficit.
Prognosis
- Predictors of long-term survival include extent of resection made at surgery and amount of residual tumor on postoperative imaging.34 Although lower WHO tumor grade, infratentorial location in children, absence of tumor invasion within the brainstem, absence of metastases,35 improved performance status, and older age (for childhood ependymoma) have been associated with a survival advantage in isolated, retrospective series,36 these factors are not significantly correlated with long-term survival.25
- Three recent series37,38,35,39,40,41,42,43 support the suggestion that the extent of resection is the most important predictor of outcome, independent of the histologic grade of the tumor. Patients with totally resected tumors, primarily of the posterior fossa, had an overall 5-year, progression-free survival rate of nearly 70% compared with 30-40% for those patients with partially resected tumors.
- As noted in Mortality/Morbidity, intracranial ependymoma has an overall 5-year survival rate of approximately 50%, but the survival rate is significantly less for children with posterior fossa tumors.15
We wish to acknowledge previous contributions to this article by Paul C McCormick, MD, and Allen Waziri, MD.
More on Ependymoma |
| Overview: Ependymoma |
| Differential Diagnoses & Workup: Ependymoma |
| Treatment & Medication: Ependymoma |
Follow-up: Ependymoma |
| Multimedia: Ependymoma |
| References |
| « Previous Page | Next Page » |
References
Applegate GL, Marymont MH. Intracranial ependymomas: a review. Cancer Invest. 1998;16(8):588-93. [Medline].
Schwartz TH, Kim S, Glick RS, et al. Supratentorial ependymomas in adult patients. Neurosurgery. Apr 1999;44(4):721-31. [Medline].
Poppleton H, Gilbertson RJ. Stem cells of ependymoma. Br J Cancer. Jan 15 2007;96(1):6-10. [Medline].
Taylor MD, Poppleton H, Fuller C, Su X, Liu Y, Jensen P, et al. Radial glia cells are candidate stem cells of ependymoma. Cancer Cell. Oct 2005;8(4):323-35. [Medline].
Dal Cin P, Sandberg AA. Cytogenetic findings in a supratentorial ependymoma. Cancer Genet Cytogenet. Feb 1988;30(2):289-93. [Medline].
Metzger AK, Sheffield VC, Duyk G, et al. Identification of a germ-line mutation in the p53 gene in a patient with an intracranial ependymoma. Proc Natl Acad Sci U S A. Sep 1 1991;88(17):7825-9. [Medline].
Sainati L, Montaldi A, Putti MC, et al. Cytogenetic t(11;17)(q13;q21) in a pediatric ependymoma. Is 11q13 a recurring breakpoint in ependymomas?. Cancer Genet Cytogenet. Apr 1992;59(2):213-6. [Medline].
Mazewski C, Soukup S, Ballard E, et al. Karyotype studies in 18 ependymomas with literature review of 107 cases. Cancer Genet Cytogenet. Aug 1999;113(1):1-8. [Medline].
Nijssen PC, Deprez RH, Tijssen CC, et al. Familial anaplastic ependymoma: evidence of loss of chromosome 22 in tumour cells. J Neurol Neurosurg Psychiatry. Oct 1994;57(10):1245-8. [Medline].
Yokota T, Tachizawa T, Fukino K, Teramoto A, Kouno J, Matsumoto K. A family with spinal anaplastic ependymoma: evidence of loss of chromosome 22q in tumor. J Hum Genet. 2003;48(11):598-602. [Medline].
Ebert C, von Haken M, Meyer-Puttlitz B, et al. Molecular genetic analysis of ependymal tumors. NF2 mutations and chromosome 22q loss occur preferentially in intramedullary spinal ependymomas. Am J Pathol. Aug 1999;155(2):627-32. [Medline].
Hulsebos TJ, Oskam NT, Bijleveld EH, Westerveld A, Hermsen MA, van den Ouweland AM. Evidence for an ependymoma tumour suppressor gene in chromosome region 22pter-22q11.2. Br J Cancer. Dec 1999;81(7):1150-4. [Medline].
James CD, He J, Carlbom E, et al. Loss of genetic information in central nervous system tumors common to children and young adults. Genes Chromosomes Cancer. Jul 1990;2(2):94-102. [Medline].
McGuire CS, Sainani KL, Fisher PG. Incidence patterns for ependymoma: a Surveillance, Epidemiology, and End Results study. J Neurosurg. Dec 5 2008;[Medline].
Bouffet E, Perilongo G, Canete A, Massimino M. Intracranial ependymomas in children: a critical review of prognostic factors and a plea for cooperation. Med Pediatr Oncol. Jun 1998;30(6):319-29; discussion 329-31. [Medline].
Polednak AP, Flannery JT. Brain, other central nervous system, and eye cancer. Cancer. Jan 1 1995;75(1 Suppl):330-7. [Medline]. [Full Text].
Macdonald J. Advances in imaging techniques in neuroendocrine tumors: miscellaneous papers of interest. Curr Opin Oncol. Feb 1990;2(1):117-8. [Medline].
Chamberlain MC, Kormanik PA. Practical guidelines for the treatment of malignant gliomas. West J Med. Feb 1998;168(2):114-20. [Medline].
Merchant TE. Current management of childhood ependymoma. Oncology (Williston Park). 2002/05;16(5):629-42, 644; discussion 645-6, 648.
Timmermann B, Kortmann RD, Kühl J, Meisner C, Slavc I, Pietsch T. Combined postoperative irradiation and chemotherapy for anaplastic ependymomas in childhood: results of the German prospective trials HIT 88/89 and HIT 91. Int J Radiat Oncol Biol Phys. Jan 15 2000;46(2):287-95. [Medline].
National Comprehensive Cancer Network. NCCN Practice Guidelines in Oncology: Adult Intracranial Ependymomas. Available at http://www.nccn.org/professionals/physician_gls/PDF/cns.pdf. Accessed Jan 2009.
Rogers L, Pueschel J, Spetzler R, et al. Is gross-total resection sufficient treatment for posterior fossa ependymomas?. J Neurosurg. Apr 2005;102(4):629-36. [Medline].
Merchant TE, Li C, Xiong X, Gaber MW. Cytokine and Growth Factor Responses After Radiotherapy for Localized Ependymoma. Int J Radiat Oncol Biol Phys. Nov 17 2008;[Medline].
Goldwein JW, Glauser TA, Packer RJ, et al. Recurrent intracranial ependymomas in children. Survival, patterns of failure, and prognostic factors. Cancer. Aug 1 1990;66(3):557-63. [Medline].
Chiu JK, Woo SY, Ater J, et al. Intracranial ependymoma in children: analysis of prognostic factors. J Neurooncol. Jul 1992;13(3):283-90. [Medline].
Stafford SL, Pollock BE, Foote RL, Gorman DA, Nelson DF, Schomberg PJ. Stereotactic radiosurgery for recurrent ependymoma. Cancer. Feb 15 2000;88(4):870-5. [Medline].
Partap S, Fisher PG. Update on new treatments and developments in childhood brain tumors. Curr Opin Pediatr. Dec 2007;19(6):670-4. [Medline].
Reni M, Gatta G, Mazza E, Vecht C. Ependymoma. Crit Rev Oncol Hematol. Jul 2007;63(1):81-9. [Medline].
Tabori U, Ma J, Carter M, Zielenska M, Rutka J, Bouffet E, et al. Human telomere reverse transcriptase expression predicts progression and survival in pediatric intracranial ependymoma. J Clin Oncol. Apr 1 2006;24(10):1522-8. [Medline].
Sowar K, Straessle J, Donson AM, Handler M, Foreman NK. Predicting which children are at risk for ependymoma relapse. J Neurooncol. May 2006;78(1):41-6. [Medline].
Modena P, Lualdi E, Facchinetti F, Veltman J, Reid JF, Minardi S, et al. Identification of tumor-specific molecular signatures in intracranial ependymoma and association with clinical characteristics. J Clin Oncol. Nov 20 2006;24(33):5223-33. [Medline].
Catsman-Berrevoets CE, Van Dongen HR, Mulder PG, et al. Tumour type and size are high risk factors for the syndrome of "cerebellar" mutism and subsequent dysarthria. J Neurol Neurosurg Psychiatry. Dec 1999;67(6):755-7. [Medline].
Doxey D, Bruce D, Sklar F, et al. Posterior fossa syndrome: identifiable risk factors and irreversible complications. Pediatr Neurosurg. Sep 1999;31(3):131-6. [Medline].
Healey EA, Barnes PD, Kupsky WJ, Scott RM, Sallan SE, Black PM. The prognostic significance of postoperative residual tumor in ependymoma. Neurosurgery. May 1991;28(5):666-71; discussion 671-2. [Medline].
Carrie C, Mottolese C, Bouffet E, Negrier S, Bachelot TH, Lasset C. Non-metastatic childhood ependymomas. Radiother Oncol. Aug 1995;36(2):101-6. [Medline].
McGuire CS, Sainani KL, Fisher PG. Both location and age predict survival in ependymoma: a SEER study. Pediatr Blood Cancer. Jan 2009;52(1):65-9. [Medline].
Tomita T, McLone DG, Das L, Brand WN. Benign ependymomas of the posterior fossa in childhood. Pediatr Neurosci. 1988;14(6):277-85. [Medline].
Sutton LN, Goldwein J, Perilongo G, Lang B, Schut L, Rorke L. Prognostic factors in childhood ependymomas. Pediatr Neurosurg. 1990-1991;16(2):57-65. [Medline].
Jayawickreme DP, Hayward RD, Harkness WF. Intracranial ependymomas in childhood: a report of 24 cases followed for 5 years. Childs Nerv Syst. Jul 1995;11(7):409-13. [Medline].
Pollack IF, Gerszten PC, Martinez AJ, et al. Intracranial ependymomas of childhood: long-term outcome and prognostic factors. Neurosurgery. Oct 1995;37(4):655-66; discussion 666-7. [Medline].
Robertson PL, Zeltzer PM, Boyett JM, et al. Survival and prognostic factors following radiation therapy and chemotherapy for ependymomas in children: a report of the Children's Cancer Group. J Neurosurg. Apr 1998;88(4):695-703. [Medline].
Horn B, Heideman R, Geyer R, Pollack I, Packer R, Goldwein J. A multi-institutional retrospective study of intracranial ependymoma in children: identification of risk factors. J Pediatr Hematol Oncol. May-Jun 1999;21(3):203-11. [Medline].
Merchant TE, Mulhern RK, Krasin MJ, Kun LE, Williams T, Li C. Preliminary results from a phase II trial of conformal radiation therapy and evaluation of radiation-related CNS effects for pediatric patients with localized ependymoma. J Clin Oncol. Aug 1 2004;22(15):3156-62. [Medline].
Bailey P. Tumors arising from ependymal cells. Arch Neurol Psychiatr. 1924;11:1-27.
Barnholtz-Sloan JS, Severson RK, Stanton B, Hamre M, Sloan AE. Pediatric brain tumors in non-Hispanics, Hispanics, African Americans and Asians: differences in survival after diagnosis. Cancer Causes Control. Jun 2005;16(5):587-92. [Medline]. [Full Text].
Bijlsma EK, Voesten AM, Bijleveld EH, et al. Molecular analysis of genetic changes in ependymomas. Genes Chromosomes Cancer. Aug 1995;13(4):272-7. [Medline].
Birch BD, Johnson JP, Parsa A, et al. Frequent type 2 neurofibromatosis gene transcript mutations in sporadic intramedullary spinal cord ependymomas. Neurosurgery. Jul 1996;39(1):135-40. [Medline].
Duffner PK, Krischer JP, Sanford RA, Horowitz ME, Burger PC, Cohen ME. Prognostic factors in infants and very young children with intracranial ependymomas. Pediatr Neurosurg. Apr 1998;28(4):215-22. [Medline].
Goldwein JW, Leahy JM, Packer RJ, et al. Intracranial ependymomas in children. Int J Radiat Oncol Biol Phys. Dec 1990;19(6):1497-502. [Medline].
Guyotat J, Signorelli F, Desme S, Frappaz D, Madarassy G, Montange MF. Intracranial ependymomas in adult patients: analyses of prognostic factors. J Neurooncol. Dec 2002;60(3):255-68. [Medline].
Kramer DL, Parmiter AH, Rorke LB, et al. Molecular cytogenetic studies of pediatric ependymomas. J Neurooncol. Mar 1998;37(1):25-33. [Medline].
Lyons MK, Kelly PJ. Posterior fossa ependymomas: report of 30 cases and review of the literature. Neurosurgery. May 1991;28(5):659-64; discussion 664-5. [Medline].
Neumann E, Kalousek DK, Norman MG, et al. Cytogenetic analysis of 109 pediatric central nervous system tumors. Cancer Genet Cytogenet. Nov 1993;71(1):40-9. [Medline].
Ransom DT, Ritland SR, Kimmel DW, et al. Cytogenetic and loss of heterozygosity studies in ependymomas, pilocytic astrocytomas, and oligodendrogliomas. Genes Chromosomes Cancer. Nov 1992;5(4):348-56. [Medline].
Rogatto SR, Casartelli C, Rainho CA, Barbieri-Neto J. Chromosomes in the genesis and progression of ependymomas. Cancer Genet Cytogenet. Sep 1993;69(2):146-52. [Medline].
Ross GW, Rubinstein LJ. Lack of histopathological correlation of malignant ependymomas with postoperative survival. J Neurosurg. Jan 1989;70(1):31-6. [Medline].
Rubio MP, Correa KM, Ramesh V, et al. Analysis of the neurofibromatosis 2 gene in human ependymomas and astrocytomas. Cancer Res. Jan 1 1994;54(1):45-7. [Medline].
Slavc I, MacCollin MM, Dunn M, et al. Exon scanning for mutations of the NF2 gene in pediatric ependymomas, rhabdoid tumors and meningiomas. Int J Cancer. Aug 22 1995;64(4):243-7. [Medline].
Stratton MR, Darling J, Lantos PL, et al. Cytogenetic abnormalities in human ependymomas. Int J Cancer. Oct 15 1989;44(4):579-81. [Medline].
Stüben G, Stuschke M, Kroll M, Havers W, Sack H. Postoperative radiotherapy of spinal and intracranial ependymomas: analysis of prognostic factors. Radiother Oncol. Oct 1997;45(1):3-10. [Medline].
Tominaga T, Kayama T, Kumabe T, et al. Anaplastic ependymomas: clinical features and tumour suppressor gene p53 analysis. Acta Neurochir (Wien). 1995;135(3-4):163-70. [Medline].
Vagner-Capodano AM, Gentet JC, Gambarelli D, et al. Cytogenetic studies in 45 pediatric brain tumors. Pediatr Hematol Oncol. Jul-Sep 1992;9(3):223-35. [Medline].
Vanuytsel L, Brada M. The role of prophylactic spinal irradiation in localized intracranial ependymoma. Int J Radiat Oncol Biol Phys. Aug 1991;21(3):825-30. [Medline].
von Haken MS, White EC, Daneshvar-Shyesther L, et al. Molecular genetic analysis of chromosome arm 17p and chromosome arm 22q DNA sequences in sporadic pediatric ependymomas. Genes Chromosomes Cancer. Sep 1996;17(1):37-44. [Medline].
Weremowicz S, Kupsky WJ, Morton CC, Fletcher JA. Cytogenetic evidence for a chromosome 22 tumor suppressor gene in ependymoma. Cancer Genet Cytogenet. Jul 15 1992;61(2):193-6. [Medline].
Wernicke C, Thiel G, Lozanova T, et al. Involvement of chromosome 22 in ependymomas. Cancer Genet Cytogenet. Feb 1995;79(2):173-6. [Medline].
Yates AJ. An overview of principles for classifying brain tumors. Mol Chem Neuropathol. Oct 1992;17(2):103-20. [Medline].
Further Reading
Keywords
ependymoma diagnosis, ependymoma treatment, ependymoma pictures, spinal cord tumors, cellular ependymoma, papillary ependymoma, clear cell ependymoma, anaplastic ependymoma, myxopapillary ependymoma, subependymoma, glial tumor, ependymal cell, CNS tumor, CNS malignancy, central nervous system tumor, central nervous system malignancy, brain cancer, spinal cancer
Follow-up: Ependymoma