Ependymoma, childhood: Treatment - Health Professional Information [NCI PDQ]

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Childhood Ependymoma Treatment (PDQ®)

General Information about Pediatric Brain Tumors

This cancer treatment information summary provides an overview of the prognosis, diagnosis, classification, and treatment of childhood ependymoma.

The National Cancer Institute provides the PDQ pediatric cancer treatment information summaries as a public service to increase the availability of evidence-based cancer information to health professionals, patients, and the public. These summaries are updated regularly according to the latest published research findings by an Editorial Board of pediatric oncology specialists.

In recent decades, dramatic improvements in survival have been achieved for children and adolescents with cancer. Childhood and adolescent cancer survivors require close follow-up because cancer therapy side effects may persist or develop months or years after treatment. (Refer to the PDQ Late Effects of Treatment for Childhood Cancer summary for specific information about the incidence, type, and monitoring of late effects in childhood and adolescent cancer survivors.)

Primary brain tumors are a diverse group of diseases that together constitute the most common solid tumor of childhood. Brain tumors are classified according to histology, but tumor location and extent of spread are important factors that affect treatment and prognosis. Immunohistochemical analysis, cytogenetic and molecular genetic findings, and measures of mitotic activity are increasingly used in tumor diagnosis and classification.

The classification of childhood brain tumors is based on both histology and location. Tumors are classically categorized as infratentorial, sellar or suprasellar, or cortical based.

Common infratentorial (posterior fossa) tumors include the following:

1. Cerebellar astrocytomas (usually pilocytic but also fibrillary and, less frequently, high grade).
2. Medulloblastomas (primitive neuroectodermal tumors [PNETs]).
3. Ependymomas (cellular, papillary, clear cell, tanycytic, or anaplastic).
4. Brain stem gliomas (typically diffuse intrinsic high-grade tumors that are diagnosed neuroradiographically without biopsy). Focal, tectal, and exophytic cervicomedullary tumors are generally low-grade tumors.
5. Atypical teratoid/rhabdoid tumors.

Tumors that occur supratentorially include the following:

1. Low-grade cerebral hemispheric astrocytomas (grade 1 [pilocytic] or grade 2).
2. High-grade or malignant astrocytomas (anaplastic astrocytomas, glioblastomas multiforme [grade 3 or grade 4]).
3. Mixed gliomas (low grade or high grade).
4. Oligodendrogliomas (low grade or high grade).
5. PNETs (including cerebral neuroblastomas, pineoblastomas, ependymoblastomas).
6. Atypical teratoid/rhabdoid tumors.
7. Ependymomas (cellular or anaplastic).
8. Meningiomas.
9. Choroid plexus tumors (papillomas and carcinomas).
10. Pineal parenchymal tumors (pineocytomas or mixed pineal parenchymal tumors).
11. Neuronal and mixed neuronal glial tumors (gangliogliomas, desmoplastic infantile gangliogliomas, dysembryoplastic neuroepithelial tumors).
12. Metastasis (rare) from extraneural malignancies.

In addition to those tumors that occur supratentorially, other tumors that most commonly occur in the sellar or suprasellar region are:

1. Craniopharyngiomas.
2. Diencephalic astrocytomas (central tumors involving the chiasm, hypothalamus, and/or thalamus) that are generally low grade (including astrocytomas, grade 1 [pilocytic] or grade 2).
3. Germ cell tumors (germinomas or nongerminomatous).

Important general concepts that should be understood by those caring for a child with brain tumor include the following:

1. Selection of an appropriate therapy can only occur if the correct diagnosis is made and the stage of the disease is accurately determined.
2. Children with primary brain tumors represent a major therapy challenge that, for optimal results, requires the coordinated efforts of pediatric specialists in fields such as neurosurgery, neuropathology, radiation oncology, pediatric oncology, neuro-oncology, neurology, rehabilitation, neuroradiology, endocrinology, and psychology, who have special expertise in the care of patients with these diseases.[1,2,3]
3. More than one half of children diagnosed with brain tumors will survive 5 years from diagnosis. In some subgroups of patients, an even higher rate of survival and cure is possible. Each child’s treatment should be approached with curative intent, and the possible long-term sequela of the disease and its treatment should be considered before therapy is begun.
4. For most children with brain tumors, the optimal treatment regimen has not been determined. Children who have brain tumors should be considered for enrollment in clinical trials when an appropriate study is available. Such clinical trials are being carried out by institutions and/or national cooperative groups.
5. Guidelines for pediatric cancer centers and their role in the treatment of pediatric patients with cancer have been outlined by the American Academy of Pediatrics.[4]
6. The cause of most childhood brain tumors remains unknown.[5,6]

Information about ongoing clinical trials is available from the NCI Web site.

References:

1. Strother DR, Poplack IF, Fisher PG, et al.: Tumors of the central nervous system. In: Pizzo PA, Poplack DG, eds.: Principles and Practice of Pediatric Oncology. 4th ed. Philadelphia, Pa: Lippincott, Williams and Wilkins, 2002, pp 751-824.
2. Pollack IF: Brain tumors in children. N Engl J Med 331 (22): 1500-7, 1994.
3. Cohen ME, Duffner PK, eds.: Brain Tumors in Children: Principles of Diagnosis and Treatment. 2nd ed. New York: Raven Press, 1994.
4. Guidelines for the pediatric cancer center and role of such centers in diagnosis and treatment. American Academy of Pediatrics Section Statement Section on Hematology/Oncology. Pediatrics 99 (1): 139-41, 1997.
5. Kuijten RR, Bunin GR: Risk factors for childhood brain tumors. Cancer Epidemiol Biomarkers Prev 2 (3): 277-88, 1993 May-Jun.
6. Kuijten RR, Strom SS, Rorke LB, et al.: Family history of cancer and seizures in young children with brain tumors: a report from the Childrens Cancer Group (United States and Canada). Cancer Causes Control 4 (5): 455-64, 1993.

General Information and Cellular Classification of Childhood Ependymoma

Childhood ependymoma comprises approximately 9% of all childhood brain tumors representing approximately 200 cases per year in the United States.[1,2]

The classification of brain tumors is based on both histopathological characteristics and location in the brain. Ependymomas are divided into the following categories:

• Subependymoma (WHO Grade I).
• Myxopapillary ependymoma (WHO Grade I).
• Ependymoma (WHO Grade II). Variants include cellular, papillary, tanycytic, clear cell, and mixed.
• Anaplastic (also known as malignant) ependymoma (WHO Grade III).

The most recent World Health Organization classification of brain tumors maintains the term “ependymoma” for tumors that are histologically benign and malignant ependymoma for those that have malignant characteristics.[3] These categories are based on the nuclear/cytoplasmic ratio, number of nuclei and mitotic figures, and the degree of nuclear atypia. Contemporary studies have failed to show significant differences in how these tumors behave on the basis of histologic classification alone,[4,5,6,7] although a small experience from a single-institution study suggested that patients with clear cell ependymoma may be at higher risk for treatment failure,[8] confirmation is required in a larger group of unselected patients.

There are some subtypes of tumors that have been classified with ependymomas, although they carry a different prognosis. Ependymoblastomas, which generally behave more like medulloblastomas or cortical neuroectodermal tumors, are considered separate entities from ependymomas and are now classified with the embryonal tumors.[3] Myxopapillary ependymomas, which are typically benign and present in the filum terminale and cauda equina, are also considered a separate entity. The pathologic classification of pediatric brain tumors is a specialized area that is undergoing evolution; review of the diagnostic tissue by a neuropathologist who has particular expertise in this area is strongly recommended.

References:

1. Gurney JG, Smith MA, Bunin GR: CNS and miscellaneous intracranial and intraspinal neoplasms. In: Ries LA, Smith MA, Gurney JG, et al., eds.: Cancer incidence and survival among children and adolescents: United States SEER Program 1975-1995. Bethesda, Md: National Cancer Institute, SEER Program, 1999. NIH Pub.No. 99-4649., Chapter 3, pp 51-63. Also available online. Last accessed March 14, 2007.
2. Central Brain Tumor Registry of the United States.: Statistical Report: Primary Brain Tumors in the United States, 1997-2001. Hinsdale, Ill: Central Brain Tumor Registry of the United States, 2004. Also available online. Last accessed July 20, 2006.
3. Kleihues P, Burger PC, Scheithauer BW: The new WHO classification of brain tumours. Brain Pathol 3 (3): 255-68, 1993.
4. Goldwein JW, Leahy JM, Packer RJ, et al.: Intracranial ependymomas in children. Int J Radiat Oncol Biol Phys 19 (6): 1497-502, 1990.
5. Rousseau P, Habrand JL, Sarrazin D, et al.: Treatment of intracranial ependymomas of children: review of a 15-year experience. Int J Radiat Oncol Biol Phys 28 (2): 381-6, 1994.
6. Chiu JK, Woo SY, Ater J, et al.: Intracranial ependymoma in children: analysis of prognostic factors. J Neurooncol 13 (3): 283-90, 1992.
7. Pollack IF, Gerszten PC, Martinez AJ, et al.: Intracranial ependymomas of childhood: long-term outcome and prognostic factors. Neurosurgery 37 (4): 655-66; discussion 666-7, 1995.
8. Fouladi M, Helton K, Dalton J, et al.: Clear cell ependymoma: a clinicopathologic and radiographic analysis of 10 patients. Cancer 98 (10): 2232-44, 2003.

Stage Information for Childhood Ependymoma

Although there is no formal staging system, ependymomas can be divided into supratentorial and infratentorial tumors. They usually originate in the ependymal linings of ventricles in the posterior fossa or supratentorial region, and have access to the cerebral spinal fluid (CSF) and therefore may spread throughout the entire neuraxis. Thirty percent of childhood ependymomas arise outside of the posterior fossa.[1,2,3] Every patient with ependymoma should be evaluated with diagnostic imaging of the spinal cord and whole brain. The most sensitive method available for evaluating spinal cord subarachnoid metastasis is spinal magnetic resonance imaging (MRI) performed with gadolinium. If MRI is used, the entire spine is generally imaged in at least 2 planes with contiguous MR slices performed after gadolinium enhancement. In addition, CSF cytological evaluation should be conducted. While a number of factors have sometimes been associated with an unfavorable outcome (younger age at diagnosis, lower doses of radiation, anaplastic histology, subtotal resection, higher proliferation marker, MIB-1 labeling index),[1,4,5,6,7,8,9] age, histology, and extent of resection have consistently been the most important factors.[5,6,10,11] These prognostic variables must be evaluated in the context of the treatment received.

References:

1. Goldwein JW, Leahy JM, Packer RJ, et al.: Intracranial ependymomas in children. Int J Radiat Oncol Biol Phys 19 (6): 1497-502, 1990.
2. Kovnar E, Kun L, Burger J, et al.: Patterns of dissemination and recurrence in childhood ependymoma: preliminary results of Pediatric Oncology Group protocol #8532. Ann Neurol 30(3): 457, 1991.
3. Vanuytsel LJ, Bessell EM, Ashley SE, et al.: Intracranial ependymoma: long-term results of a policy of surgery and radiotherapy. Int J Radiat Oncol Biol Phys 23 (2): 313-9, 1992.
4. Shaw EG, Evans RG, Scheithauer BW, et al.: Postoperative radiotherapy of intracranial ependymoma in pediatric and adult patients. Int J Radiat Oncol Biol Phys 13 (10): 1457-62, 1987.
5. Horn B, Heideman R, Geyer R, et al.: A multi-institutional retrospective study of intracranial ependymoma in children: identification of risk factors. J Pediatr Hematol Oncol 21 (3): 203-11, 1999 May-Jun.
6. Pollack IF, Gerszten PC, Martinez AJ, et al.: Intracranial ependymomas of childhood: long-term outcome and prognostic factors. Neurosurgery 37 (4): 655-66; discussion 666-7, 1995.
7. Merchant TE, Jenkins JJ, Burger PC, et al.: Influence of tumor grade on time to progression after irradiation for localized ependymoma in children. Int J Radiat Oncol Biol Phys 53 (1): 52-7, 2002.
8. Wolfsberger S, Fischer I, Höftberger R, et al.: Ki-67 immunolabeling index is an accurate predictor of outcome in patients with intracranial ependymoma. Am J Surg Pathol 28 (7): 914-20, 2004.
9. Kurt E, Zheng PP, Hop WC, et al.: Identification of relevant prognostic histopathologic features in 69 intracranial ependymomas, excluding myxopapillary ependymomas and subependymomas. Cancer 106 (2): 388-95, 2006.
10. Bouffet E, Perilongo G, Canete A, et al.: Intracranial ependymomas in children: a critical review of prognostic factors and a plea for cooperation. Med Pediatr Oncol 30 (6): 319-29; discussion 329-31, 1998.
11. Korshunov A, Golanov A, Sycheva R, et al.: The histologic grade is a main prognostic factor for patients with intracranial ependymomas treated in the microneurosurgical era: an analysis of 258 patients. Cancer 100 (6): 1230-7, 2004.

Treatment Option Overview for Childhood Ependymoma

Many of the improvements in survival in childhood cancer have been made as a result of clinical trials that have attempted to improve on the best available, accepted therapy. Clinical trials in pediatrics are designed to compare new therapy with therapy that is currently accepted as standard. This comparison may be done in a randomized study of 2 treatment arms or by evaluating a single new treatment and comparing the results with those previously obtained with existing therapy.

Because of the relative rarity of cancer in children, all patients with brain tumors should be considered for entry into a clinical trial. To determine and implement optimum treatment, treatment planning by a multidisciplinary team of cancer specialists who have experience treating childhood brain tumors is required. Radiation therapy of pediatric brain tumors is technically very demanding and should be carried out in centers that have experience in that area in order to ensure optimal results.

In the past, treatment for childhood ependymoma has included surgery with radiation therapy. There is evidence to suggest that more extensive surgical resections are related to an improved rate of survival.[1,2,3] Chemotherapy has been shown to be active in patients with recurrent ependymoma.[4] One relatively small, prospective, randomized trial suggests that its activity in newly diagnosed cases is limited,[5] and current treatment approaches do not include chemotherapy as a component of primary therapy for most children with newly diagnosed ependymoma. Children younger than 3 years are particularly susceptible to the adverse effect of radiation on brain development. Debilitating effects on growth and neurologic development have frequently been observed, especially in younger children.[6,7,8] For this reason, conformal radiation approaches that minimize damage to normal brain tissue are under evaluation for infants and children with ependymoma.[9] Long-term management of these patients is complex and requires a multidisciplinary approach.

There is evidence that surveillance neuroimaging in childhood ependymoma will identify tumors that have recurred when the patient is asymptomatic; however, it is unclear whether this detection will change the ultimate prognosis of the patient.[10]

The designations in PDQ that treatments are “standard” or “under clinical evaluation” are not to be used as a basis for reimbursement determinations.

References:

1. Pollack IF, Gerszten PC, Martinez AJ, et al.: Intracranial ependymomas of childhood: long-term outcome and prognostic factors. Neurosurgery 37 (4): 655-66; discussion 666-7, 1995.
2. Horn B, Heideman R, Geyer R, et al.: A multi-institutional retrospective study of intracranial ependymoma in children: identification of risk factors. J Pediatr Hematol Oncol 21 (3): 203-11, 1999 May-Jun.
3. van Veelen-Vincent ML, Pierre-Kahn A, Kalifa C, et al.: Ependymoma in childhood: prognostic factors, extent of surgery, and adjuvant therapy. J Neurosurg 97 (4): 827-35, 2002.
4. Goldwein JW, Glauser TA, Packer RJ, et al.: Recurrent intracranial ependymomas in children. Survival, patterns of failure, and prognostic factors. Cancer 66 (3): 557-63, 1990.
5. Evans AE, Anderson JR, Lefkowitz-Boudreaux IB, et al.: Adjuvant chemotherapy of childhood posterior fossa ependymoma: cranio-spinal irradiation with or without adjuvant CCNU, vincristine, and prednisone: a Childrens Cancer Group study. Med Pediatr Oncol 27 (1): 8-14, 1996.
6. Packer RJ, Sutton LN, Atkins TE, et al.: A prospective study of cognitive function in children receiving whole-brain radiotherapy and chemotherapy: 2-year results. J Neurosurg 70 (5): 707-13, 1989.
7. Johnson DL, McCabe MA, Nicholson HS, et al.: Quality of long-term survival in young children with medulloblastoma. J Neurosurg 80 (6): 1004-10, 1994.
8. Packer RJ, Sutton LN, Goldwein JW, et al.: Improved survival with the use of adjuvant chemotherapy in the treatment of medulloblastoma. J Neurosurg 74 (3): 433-40, 1991.
9. Merchant TE, Mulhern RK, Krasin MJ, et al.: 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 22 (15): 3156-62, 2004.
10. Good CD, Wade AM, Hayward RD, et al.: Surveillance neuroimaging in childhood intracranial ependymoma: how effective, how often, and for how long? J Neurosurg 94 (1): 27-32, 2001.

Treatment of Newly Diagnosed Childhood Ependymoma

In the newly diagnosed patient, careful evaluation to fully determine the extent of disease must precede the treatment of ependymoma. Surgery should be performed in an attempt at maximal tumor reduction; children have improved progression-free survival if there is minimal residual disease present after surgery.[1,2] Postoperatively, magnetic resonance imaging (MRI) should be performed to determine the extent of resection. If not performed preoperatively, MRI of the entire neuroaxis should be obtained to evaluate for disease dissemination. Patients with residual tumor or disseminated disease should be considered at high risk for relapse and should be treated on protocols specifically designed for them. Those with no evidence of residual tumor still have an approximate 20% to 40% relapse risk in spite of postoperative radiation therapy.

Postsurgical Treatment Options

Standard treatment options

• NO RESIDUAL DISEASE; NO DISSEMINATED DISEASE:

  The traditional postsurgical treatment for these patients has been radiation therapy consisting of 5,400 to 5,580 cGy to the tumor bed for children aged 3 years and older and is under evaluation for children younger than 3 years. It is not necessary to treat the entire central nervous system (CNS) (whole brain and spine) because these tumors usually recur at the local site.[2,3] When possible, patients should be treated in a center experienced with this therapy. There is no evidence that adjuvant chemotherapy improves the outcome for patients with totally resected, nondisseminated ependymoma. The 3-year progression-free survival rate in 74 patients aged between 1 and 21 years treated with radiation therapy following surgery was 77.6% ± 5.8%.[4] Limited experience with surgery alone for completely resected supratentorial nonanaplastic tumors suggests that, in select cases, this may be an option.[5]

• RESIDUAL DISEASE; NO DISSEMINATED DISEASE:

  Second-look surgery should be considered because patients who have complete resections have better disease control. The traditional postsurgical treatment for children aged 3 years and older has been radiation therapy consisting of 5,400 to 5,580 cGy to the tumor bed. It is not necessary to treat the entire CNS (whole brain and spine) because these tumors usually recur at the local site. In subtotally resected patients, treatment with radiation therapy results in 3- to 5-year progression-free survival in 30% to 50% of patients.[4,6] There is no evidence that adjuvant chemotherapy, including high-dose chemotherapy with stem cell rescue, is of any benefit.[7]

• CNS DISSEMINATED DISEASE:

  In children with disseminated disease, long-term survivors have been reported and aggressive therapy is warranted. Regardless of degree of surgical resection, these patients require radiation therapy to the entire CNS (whole brain and spine) along with boosts to local disease and bulk areas of disseminated disease. The traditional local postsurgical radiation doses in these patients have been 5,400 to 5,580 cGy. Doses of approximately 3,600 cGy to the entire neuraxis (i.e., the whole brain and spine) should also be administered, but may be modulated depending on the age of the patient. Boosts between 4,140 cGy and 5,040 cGy to bulk areas of spinal disease should be administered, with doses depending on the age of the patient and the location of the tumor. When possible, patients should be treated in a center experienced with this therapy. Trials are ongoing to evaluate the possible role of radiation therapy and chemotherapy in these patients.

• MANAGEMENT OF CHILDREN YOUNGER THAN 3 YEARS:

  Because of the known effects of radiation on growth and neurocognitive development, radiation therapy immediately after surgery in children younger than 3 years has traditionally been limited, with attempts to delay its administration through the use of chemotherapy.[8,9,10,11] When analyzing neurologic outcome following treatment of young children with ependymoma, it is important to consider that not all long-term deficits can be ascribed to radiation therapy, as deficits may be present in young children before therapy is begun.[4] For example, the presence of hydrocephalus at diagnosis is associated with lower intelligence quotient as measured following surgical resection and prior to administration of radiation therapy.[12]

  Chemotherapy is able to induce objective responses in some children younger than 3 years with newly diagnosed ependymoma,[8,9,10] though not all chemotherapy regimens induce objective responses.[11] Most young children with ependymoma treated with chemotherapy alone following their initial surgical resection will eventually develop progressive disease, though a minority of children may achieve long-term survival with chemotherapy alone.[10,11] The need and timing of radiation therapy for children who have successfully completed chemotherapy and have no residual disease is still to be determined. Current approaches to treating young children with ependymoma do not generally employ chemotherapy to delay use of radiation therapy.

  Conformal radiation therapy is an alternative approach for minimizing radiation-induced neurologic damage in young children with ependymoma. The initial experience with this approach suggests that children with ependymoma younger than 3 years have neurologic deficits at diagnosis that improve with time following conformal radiation treatment.[4] The current Children's Oncology Group (COG) protocol for children with ependymoma includes young children aged 12 to 36 months and is evaluating whether conformal radiation therapy can minimize neurologic late effects while producing long-term survival.

Treatment options under clinical evaluation

The following is an example of a national and/or institutional clinical trial that is currently being conducted. Information about ongoing clinical trials is available from the NCI Web site.

A COG trial, ACNS0121,[13] is evaluating several questions of therapy for different subgroups of children aged 1 year and older with ependymoma as described below:

No Residual Disease; No Disseminated Disease

• Children who have supratentorial nonanaplastic ependymoma for whom a gross total resection can be performed: These children are being carefully observed following surgical resection to determine whether they can be cured with surgery alone.
• Children with supratentorial anaplastic ependymoma and children with infratentorial ependymoma who have a near total resection or better: These children receive conformal radiation therapy directed at the primary site to determine whether cure can be achieved with this approach while minimizing radiation-associated long-term toxicities. Children with supratentorial nonanaplastic ependymoma with a near-total resection or better but who are not eligible for the observation also receive conformal radiation.

Residual Disease; No Disseminated Disease

• Children with initial incompletely resected ependymoma: These children receive chemotherapy in an attempt to achieve a complete resection with second surgery prior to conformal radiation therapy.

References:

1. Hukin J, Epstein F, Lefton D, et al.: Treatment of intracranial ependymoma by surgery alone. Pediatr Neurosurg 29 (1): 40-5, 1998.
2. Horn B, Heideman R, Geyer R, et al.: A multi-institutional retrospective study of intracranial ependymoma in children: identification of risk factors. J Pediatr Hematol Oncol 21 (3): 203-11, 1999 May-Jun.
3. Evans AE, Anderson JR, Lefkowitz-Boudreaux IB, et al.: Adjuvant chemotherapy of childhood posterior fossa ependymoma: cranio-spinal irradiation with or without adjuvant CCNU, vincristine, and prednisone: a Childrens Cancer Group study. Med Pediatr Oncol 27 (1): 8-14, 1996.
4. Merchant TE, Mulhern RK, Krasin MJ, et al.: 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 22 (15): 3156-62, 2004.
5. Goldwein JW, Leahy JM, Packer RJ, et al.: Intracranial ependymomas in children. Int J Radiat Oncol Biol Phys 19 (6): 1497-502, 1990.
6. Pollack IF, Gerszten PC, Martinez AJ, et al.: Intracranial ependymomas of childhood: long-term outcome and prognostic factors. Neurosurgery 37 (4): 655-66; discussion 666-7, 1995.
7. Grill J, Kalifa C, Doz F, et al.: A high-dose busulfan-thiotepa combination followed by autologous bone marrow transplantation in childhood recurrent ependymoma. A phase-II study. Pediatr Neurosurg 25 (1): 7-12, 1996.
8. Duffner PK, Horowitz ME, Krischer JP, et al.: The treatment of malignant brain tumors in infants and very young children: an update of the Pediatric Oncology Group experience. Neuro-oncol 1 (2): 152-61, 1999.
9. Duffner PK, Horowitz ME, Krischer JP, et al.: Postoperative chemotherapy and delayed radiation in children less than three years of age with malignant brain tumors. N Engl J Med 328 (24): 1725-31, 1993.
10. Geyer JR, Sposto R, Jennings M, et al.: Multiagent chemotherapy and deferred radiotherapy in infants with malignant brain tumors: a report from the Children's Cancer Group. J Clin Oncol 23 (30): 7621-31, 2005.
11. Grill J, Le Deley MC, Gambarelli D, et al.: Postoperative chemotherapy without irradiation for ependymoma in children under 5 years of age: a multicenter trial of the French Society of Pediatric Oncology. J Clin Oncol 19 (5): 1288-96, 2001.
12. Merchant TE, Lee H, Zhu J, et al.: The effects of hydrocephalus on intelligence quotient in children with localized infratentorial ependymoma before and after focal radiation therapy. J Neurosurg 101 (2 Suppl): 159-68, 2004.
13. Merchant TE, Children's Oncology Group: Phase II Study of Conformal Radiotherapy For Pediatric Patients With Localized Ependymoma, Chemotherapy Prior to Second Surgery in Pediatric Patients With Incompletely Resected Ependymoma, and Observation Only in Pediatric Patients With Completely Resected Differentiated, Supratentorial Ependymoma, COG-ACNS0121, Clinical trial, Temporarily closed.

Recurrent Childhood Ependymoma

Recurrence is not uncommon in both benign and malignant childhood brain tumors and may develop many years after initial treatment. For ependymoma, delays beyond 10 to 15 years have been reported.[1,2] Disease generally recurs at the primary tumor site, even in children with malignant ependymomas.[3,4] Systemic relapse is extremely rare. At time of relapse, a complete evaluation for extent of recurrence is indicated for all patients. The need for surgical intervention must be individualized on the basis of the extent of tumor, the length of time between initial treatment and the reappearance of the recurrent lesion, and the clinical picture. Patients with recurrent ependymomas who have not previously received radiation therapy and/or chemotherapy should be considered for treatment with these modalities. In addition, patients may be candidates for focal retreatment with various radiation modalities, including stereotactic radiosurgery. Active agents include cyclophosphamide, cisplatin, carboplatin, lomustine, and etoposide. Entry into studies of novel therapeutic approaches should be considered. Information about ongoing clinical trials is available from the NCI Web site.

References:

1. Pollack IF, Gerszten PC, Martinez AJ, et al.: Intracranial ependymomas of childhood: long-term outcome and prognostic factors. Neurosurgery 37 (4): 655-66; discussion 666-7, 1995.
2. Vanuytsel LJ, Bessell EM, Ashley SE, et al.: Intracranial ependymoma: long-term results of a policy of surgery and radiotherapy. Int J Radiat Oncol Biol Phys 23 (2): 313-9, 1992.
3. Goldwein JW, Corn BW, Finlay JL, et al.: Is craniospinal irradiation required to cure children with malignant (anaplastic) intracranial ependymomas? Cancer 67 (11): 2766-71, 1991.
4. Merchant TE, Haida T, Wang MH, et al.: Anaplastic ependymoma: treatment of pediatric patients with or without craniospinal radiation therapy. J Neurosurg 86 (6): 943-9, 1997.

Changes to This Summary (07/21/2006)

The PDQ cancer information summaries are reviewed regularly and updated as new information becomes available. This section describes the latest changes made to this summary as of the date above.

STAGE INFORMATION FOR CHILDHOOD EPENDYMOMA

Added text to include the MIB-1 labeling index in the list of prognostic factors sometimes associated with an unfavorable outcome (cited Kurt et al. as reference 9).

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Date Last Modified: 2006-07-21