Ependymoma

Glioma derived from ependymocytes that tend to present as malignant intracranial tumors in children and as benign intraspinal neoplasms in adults. It may arise from any level of the ventricular system or central canal of the spinal cord. Intracranial ependymomas most frequently originate in the FOURTH VENTRICLE and histologically are densely cellular tumors which may contain ependymal tubules and perivascular pseudorosettes. Spinal ependymomas are usually benign papillary or myxopapillary tumors. (From DeVita et al., Principles and Practice of Oncology, 5th ed, p2018; Escourolle et al., Manual of Basic Neuropathology, 2nd ed, pp28-9)

Also Known As:

Networked: 894 relevant articles (23 outcomes, 106 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Neoplasms (Cancer)
2.	Medulloblastoma
3.	Astrocytoma (Pilocytic Astrocytoma)
4.	Brain Neoplasms (Brain Tumor)
5.	Glioblastoma (Glioblastoma Multiforme)

Experts

1.	Merchant, Thomas E: 12 articles (03/2014 - 04/2002)
2.	Gilbertson, Richard J: 11 articles (12/2015 - 10/2002)
3.	Gajjar, Amar: 11 articles (12/2015 - 07/2009)
4.	Grill, Jacques: 9 articles (09/2015 - 07/2002)
5.	Ellison, David W: 9 articles (08/2015 - 10/2002)
6.	Korshunov, Andrey: 8 articles (12/2012 - 07/2002)
7.	Kun, Larry E: 7 articles (03/2014 - 03/2009)
8.	Stewart, Clinton F: 6 articles (12/2015 - 09/2010)
9.	Vassal, Gilles: 6 articles (03/2015 - 07/2002)
10.	Perry, Arie: 6 articles (07/2013 - 05/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Ependymoma:

1.	Etoposide (VP 16)FDA LinkGeneric 10/01/2001 - "Primary ependymoma of the ovary, in which long-term oral etoposide (VP-16) was effective in prolonging disease-free survival." 09/01/2005 - "In this study the authors retrospectively evaluated the feasibility and effectiveness of prolonged oral etoposide therapy in children with recurrent ependymoma. " 09/01/2002 - "A prospective Phase II study to determine toxicity and response to chronic oral etoposide in patients with recurrent low-grade intramedullary spinal cord ependymoma (SCE) was conducted. " 02/01/2001 - "This study was performed to assess the toxicity and activity of chronic oral etoposide in the management of children with recurrent intracranial nondisseminated ependymoma. " 09/01/2005 - "These results emphasize that oral etoposide is an attractive option for childhood recurrent ependymomas in terms of administration, tolerability, and neuroradiologic response."
2.	bevacizumabFDA Link 11/01/2015 - "Clinical response associated with radiographic regression of a cervicomedullary ependymoma in a NF2 patient treated by bevacizumab." 12/01/2013 - "Universal guidance is lacking in the management of non-operable irradiated recurrent ependymoma and there are worthy therapeutic avenues for further investigation, in particular the role of radical re-irradiation and also the potential of bevacizumab in advanced disease. " 11/17/2009 - "Bevacizumab for recurrent ependymoma." 01/01/2013 - "We report a case of anaplastic ependymoma with distant metastases to the vertebral bones, lungs, liver, and lymph nodes following treatment with bevacizumab. " 01/01/2013 - "Extraneural ependymoma: distant bone, lung, liver, and lymph node metastases following bevacizumab."
3.	Protons (Proton)IBA 09/01/2011 - "With the caveat of a limited number of patients, the multivariate model seems to suggest improved overall survival for younger patients (<54 years of age), ependymoma histology, and photon vs. proton treatment. " 08/01/2015 - "The aim of this study was to evaluate the efficacy of proton beam therapy for pediatric patients with ependymoma. " 09/01/2015 - "Imaging Changes in Pediatric Intracranial Ependymoma Patients Treated With Proton Beam Radiation Therapy Compared to Intensity Modulated Radiation Therapy." 08/01/2015 - "Proton therapy appears safe and efficacious for the re-treatment of recurrent intracranial ependymoma." 08/01/2015 - "To report disease control, survival and treatment-associated toxicity with the use of proton therapy (PRT) for re-irradiation of intracranial ependymoma. "
4.	Cisplatin (Platino)FDA LinkGeneric 01/01/2003 - "Cisplatin, at a dose of 120 mg/m(2) (cumulated response rate of 34% [95% CI 19-54%]) is the only single agent that has reproducibly shown some efficacy in ependymoma. " 05/01/1997 - "Four of the 10 children with medulloblastoma and ependymoma who relapsed are now disease free at 7.5, 11.7, 12.2 and 13.5 yrs post relapse after receiving salvage therapy with cisplatin (n = 1) or irradiation (n = 3). " 12/15/1990 - "Of 33 children with medulloblastoma, ependymoma, or central nervous system PNET, five of 12 with no prior cisplatin exposure had responses, and two of 21 with prior cisplatin exposure had responses (P = 0.03). " 01/01/2013 - "Cisplatin and temozolomide combination in the treatment of supratentorial anaplastic ependymoma." 12/01/1986 - "Peak CSF and serum platinum levels were examined in a patient with ependymoma after each of four consecutive cisplatin doses of 100 mg/m2 administered by either 30-minute or 3-hour infusion. "
5.	irinotecan (Camptosar)FDA LinkGeneric 04/01/2002 - "CPT-11, given in this schedule, appears to be active in children with malignant glioma, medulloblastoma, and ependymoma with acceptable toxicity. " 03/01/1998 - "Irinotecan displayed a broad spectrum of activity in paediatric solid tumour xenografts, including rhabdo-myosarcoma, neuroblastoma, peripheral primitive neuroectodermal tumour, medulloblastoma, ependymoma, malignant glioma and juvenile colon cancer. " 11/01/2012 - "A phase II study of bevacizumab (BVZ) plus irinotecan (CPT-11) was conducted in cases of pediatric recurrent ependymoma (EPN) to estimate sustained objective response rate and progression-free survival (PFS). " 11/01/2012 - "Lack of efficacy of bevacizumab + irinotecan in cases of pediatric recurrent ependymoma--a Pediatric Brain Tumor Consortium study." 08/01/2013 - "Twenty-four children with brain tumors in a phase II study of bevacizumab and irinotecan underwent brain MR and (18)F-FDG PET within 2 wk. Tumor types included supratentorial high-grade astrocytoma (n = 7), low-grade glioma (n = 9), brain stem glioma (n = 4), medulloblastoma (n = 2), and ependymoma (n = 2). "
6.	temozolomide (Temodar)FDA LinkGeneric 01/01/2016 - "We report two pediatric patients with refractory anaplastic ependymoma treated with temozolomide (TMZ). " 01/01/2016 - "Temozolomide Treatment for Pediatric Refractory Anaplastic Ependymoma with Low MGMT Protein Expression." 07/01/2015 - "Role of Evaluating MGMT Status and 1p36 Deletion in Radiosurgery-Induced Anaplastic Ependymoma That Rapidly and Completely Resolved by Temozolomide Alone: Case Report and Review of the Literature." 08/01/2014 - "Ependymoma growth might be impaired by temozolomide through preferential depletion of a less differentiated, more tumorigenic, MGMT-negative cell population with stem-like properties." 08/01/2014 - "Differentiation-committed ependymoma SCs acquired resistance to temozolomide. "
7.	Estrogens (Estrogen)FDA Link 02/01/2014 - "Aromatase inhibitor treatment could be effective in cases of extraaxial ependymoma with prominent estrogen receptor expression." 06/01/1988 - "Estrogen and progestin receptors in an ovarian ependymoma." 08/01/1992 - "Together with a previously reported case of recurrent ovarian ependymoma with estrogen and progesterone receptors, this case suggests that hormonal responsiveness of this rare neoplasm may be pathogenically significant." 01/01/1984 - "We examined the cytosolic estrogen receptor (ER) level in tumor tissue from 77 patients: 36 meningiomas, 20 gliomas (12 glioblastomas, 2 cerebellar astrocytomas, 2 ependymomas, and 4 medulloblastomas), 8 neurinomas, 7 pituitary adenomas (2 prolactin-producing adenomas, 1 growth hormone-producing adenoma, and 4 nonfunctioning adenomas), and 6 metastatic brain tumors (1 from breast cancer, 4 from lung cancers, and 1 from colon cancer). " 03/01/2011 - "The latter had a central nervous system phenotype different from that previously reported in ovarian ependymomas of extra-axial types, being negative for estrogen and progesterone receptors, epithelial membrane antigen and cytokeratin 34βE12, cell adhesion molecule 5.2, and cytokeratin 7. Furthermore, its benign behavior contrasted with the aggressive course of the other 2 ependymomas of extra-axial types, in which peritoneal invasion was present at the time of diagnosis. "
8.	Cyclophosphamide (Cytoxan)FDA LinkGeneric 01/01/1994 - "These studies suggest that busulfan may be active against medulloblastomas, high-grade gliomas, and ependymomas as well as against cyclophosphamide-resistant neoplasms." 11/01/2012 - "This is the first report showing an impressive efficacy of metronomic the cyclophosphamide, cisplatin and bevacizumab chemotherapeutic regimen for the treatment of refractory anaplastic ependymoma." 11/01/2012 - "Here, we report two cases of anaplastic ependymoma with prolonged radiological and clinical responses to a metronomic cyclophosphamide, cisplatin and bevacizumab regimen. " 11/01/2012 - "Metronomic cyclophosphamide with cisplatin and bevacizumab: a new chemotherapeutic regimen for refractory anaplastic ependymoma." 07/01/2001 - "4-Demethyl-4-methoxyacetylpenclomedine showed inferior activity to current clinical brain tumor drugs against a glioma cell line, superior activity to temozolomide and procarbazine against the derived mismatch repair-deficient cell line, and superior activity to cyclophosphamide and carmustine but inferior activity to temozolomide against two ependymoma cell lines, all of which were implanted s.c. "
9.	Carboplatin (JM8)FDA LinkGeneric 07/01/2007 - "Protocol III (carboplatin and vincristine) in 3 children with medulloblastoma, ependymoma and glioblastoma multiformae. " 02/01/1992 - "Neither drug demonstrated appreciable activity in the treatment of medulloblastoma (two of 26 responses to carboplatin, one of 14 responses to iproplatin), ependymoma (two of 17 responses to carboplatin, none of seven responses to iproplatin), high-grade glioma (two of 19 responses to carboplatin, one of 14 responses to iproplatin), or brain-stem tumors (one of 23 responses to carboplatin, none of 14 responses to iproplatin). " 07/01/1998 - "Following conventional chemotherapy with carboplatin/etoposide and after achieving complete or partial remission (CR or PR) 10 patients aged from 3.2 to 25.5 years (median, 10.3 years) with refractory or recurrent malignant brain tumors (anaplastic astrocytoma/glioblastoma, n = 2; medulloblastoma/PNET, n = 6; ependymoma, n = 1; plexus carcinoma, n = 1) received in a pilot study one course of HDCT with ABSCT. " 05/01/1995 - "In this study, several courses of intensive chemotherapy (1 course: VP-16 300mg/m2 x 5 days + carboplatin 400-500mg/m2 x 3 days) and one course of ultra-high dose chemotherapy (1 course: VP-16 400mg/m2 x 8 days + carboplatin 800mg/m2 x 5 days + MCNU 250, 200mg/m2 x each day) with PBSC transfusion were applied in four cases of pediatric malignant brain tumors (2 cases of medulloblastoma, one case of pineoblastoma and anaplastic ependymoma) after surgical reduction. " 07/15/2009 - "Fifty-three patients aged <age 3 years with embryonal CNS tumor medulloblastoma (n = 20), ependymoma (EP, n = 21), choroid plexus carcinoma (CPCA, n = 5), and primitive embryonal neoplasms including atypical teratoid rhabdoid tumors (n = 7) were treated with cyclophosphamide, etoposide, and carboplatin. "
10.	Aromatase (CYP19)IBA 02/01/2014 - "Treatment of ovarian anaplastic ependymoma by an aromatase inhibitor." 02/01/2014 - "Aromatase inhibitor treatment could be effective in cases of extraaxial ependymoma with prominent estrogen receptor expression."

Therapies and Procedures

1.	Radiotherapy 01/01/2009 - "Radiotherapy appears beneficial for survival in patients with infratentorial ependymoma." 05/01/2007 - "The impact of radiotherapy was found to be beneficial for incompletely resected low-grade ependymomas and to a lesser extent for completely removed high-grade tumours. " 07/01/2002 - "Preliminary data using hyperfractionated radiotherapy doses of greater than 65 Gy indicate an improvement in progression-free survival for subtotally respected ependymoma. " 04/01/1998 - "We conclude that postoperative radiotherapy is effective in preventing regrowth of intracranial ependymoma following subtotal resection in adults. " 10/01/1998 - "Despite the improved outcome for patients with ependymoma treated by surgery and radiotherapy, their prognosis remains relatively poor. "
2.	Drug Therapy (Chemotherapy) 07/01/2005 - "An improvement in the efficacy of radiation therapy in addition to an understanding of chemotherapy protocols and treatment durations will hopefully provide further means for successfully treating ependymomas." 07/01/2005 - "A closer look at the efficacy of tailored radiation therapy and the possible use of chemotherapy to delay radiation therapy sheds light on potential treatment modalities for ependymomas. " 07/01/2007 - "The purpose of this study is to investigate the efficacy of an intensive chemotherapy induction regimen followed by myeloablative chemotherapy and autologous hematopoietic stem cell rescue (AHSCR) in children with newly diagnosed ependymoma. " 12/15/2012 - "Phase II study of pre-irradiation chemotherapy for childhood intracranial ependymoma. " 12/01/2012 - "Chemotherapy has not played a role in most treatment regimens for ependymoma to date, but due to the ongoing therapeutic challenges for a subset of patients, this modality is being reinvestigated in a few ongoing studies. "
3.	Aftercare (After-Treatment) 01/01/2014 - "We identified only two studies that met our criteria and we found an incidence of 9% of secondary neoplasias after treatment for spinal ependymoma. " 03/15/2014 - "The use of photon CRT for ependymoma is associated with relatively stable emotional and behavioral functioning during the first 5 years after treatment. " 06/01/1995 - "At the assessment 1 year after treatment, intellectual outcome was somewhat better in the ependymoma group, but the difference was not statistically significant. " 06/01/1993 - "By contrast, almost one out of two patients with malignant ependymoma was alive 5 years after treatment. " 05/01/1984 - "Patients with ependymomas of the infratentorium were found to have shorter disease-free intervals and to live for a shorter time after treatment than those with tumors of the cauda equina. "
4.	Salvage Therapy 07/01/2006 - "When used, chemotherapy for ependymomas has been administered primarily to children aged younger than 3 years as adjuvant therapy and to patients with recurrent disease who are not considered surgical candidates as salvage therapy. " 02/01/2001 - "Recurrent intracranial ependymoma in children: salvage therapy with oral etoposide." 05/01/1997 - "Four of the 10 children with medulloblastoma and ependymoma who relapsed are now disease free at 7.5, 11.7, 12.2 and 13.5 yrs post relapse after receiving salvage therapy with cisplatin (n = 1) or irradiation (n = 3). "
5.	Adjuvant Chemotherapy 07/01/2015 - "In addition, a regimen of adjuvant chemotherapy for anaplastic ependymoma (AE) has not been established. " 08/01/2001 - "Adjuvant chemotherapy for ependymoma: is it necessary for all children under five years of age?" 04/01/2001 - "The value of adjuvant chemotherapy on the prognosis of children with ependymoma seems to be limited. " 07/15/1997 - "Adjuvant chemotherapy for the treatment of intracranial ependymoma of childhood." 04/01/1994 - "This is the first time that ependymal tumours have been demonstrated to express the protein, and we conclude that its expression may contribute to the reported failure of adjuvant chemotherapy to improve outcome in ependymomas."