Hyperfractionated radiotherapy and chemotherapy for childhood ependymoma: final results of the first prospective AIEOP (Associazione Italiana di Ematologia-Oncologia Pediatrica) study

Massimino, Maura; Gandola, Lorenza; Giangaspero, Felice; Sandri, Alessandro; Valagussa, Pinuccia; Perilongo, Giorgio; Garrè, Maria Luisa; Ricardi, Umberto; Forni, Marco; Genitori, Lorenzo; Scarzello, Giovanni; Spreafico, Filippo; Barra, Salvina; Mascarin, Maurizio; Pollo, Bianca; Gardiman, Martina; Cama, Armando; Navarria, Pierina; Brisigotti, Maurizio; Collini, Paola; Balter, Rita; Fidani, Paola; Stefanelli, Maurizio; Burnelli, Roberta; Potepan, Paolo; Podda, Marta; Sotti, Guido; Madon, E

doi:10.1016/j.ijrobp.2003.08.030

Cited by 94 publications

(53 citation statements)

References 52 publications

(93 reference statements)

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“…Several studies have linked the morphological features of anaplasia to a poor outcome or suggested ependymoma grade affects survival postradiotherapy (1,5,13,14,(27)(28)(29)(30)(31)51). However other studies refuted this or failed to establish an association between anaplasia and prognosis, including those adopting a WHO classification system (reviewed in refs.…”

Section: Histology Tumor Morphology and Prognosismentioning

confidence: 99%

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Pediatric Ependymoma: Biological Perspectives

Kilday

Rahman

Dyer

et al. 2009

Molecular Cancer Research

167

204

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Pediatric ependymomas are enigmatic tumors that continue to present a clinical management challenge despite advances in neurosurgery, neuroimaging techniques, and radiation therapy. Difficulty in predicting tumor behavior from clinical and histological factors has shifted the focus to the molecular and cellular biology of ependymoma in order to identify new correlates of disease outcome and novel therapeutic targets. This article reviews our current understanding of pediatric ependymoma biology and includes a meta-analysis of all comparative genomic hybridization (CGH) studies done on primary ependymomas to date, examining more than 300 tumors. From this meta-analysis and a review of the literature, we show that ependymomas in children exhibit a different genomic profile to those in adults and reinforce the evidence that ependymomas from different locations within the central nervous system (CNS) are distinguishable at a genomic level. Potential biological markers of prognosis in pediatric ependymoma are assessed and the ependymoma cancer stem cell hypothesis is highlighted with respect to tumor resistance and recurrence. We also discuss the shifting paradigm for treatment modalities in ependymoma that target molecular alterations in tumor-initiating cell populations. (Mol Cancer Res 2009;7(6):765-86)

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Section: Histology Tumor Morphology and Prognosismentioning

confidence: 99%

“…Several studies have shown conflicting results about an association between grade and patient outcome (1,4,5,13,14,18,22,(27)(28)(29)(30)(31).…”

Section: Introductionmentioning

confidence: 99%

Pediatric Ependymoma: Biological Perspectives

Kilday

Rahman

Dyer

et al. 2009

Molecular Cancer Research

167

204

View full text Add to dashboard Cite

show abstract

“…Even recent reports exploring hyperfractionated radiotherapy for ependymomas do not provide any additional outcome benefit compared with standard fractionated radiotherapy. 3,11,18) Only total tumor resection is considered as a reliable prognostic factor for predicting longer survival time. 1,19) Therefore, major improvement in outcomes might come from changing the treatment approach rather than refining the currently available treatment options.…”

Section: Discussionmentioning

confidence: 99%

Huge Supratentorial Extraventricular Anaplastic Ependymoma Presenting With Massive Calcification -Case Report-

Hamano

Tsutsumi

Nonaka

et al. 2010

Neurol. Med. Chir.(Tokyo)

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A 15-year-old boy presented with an anaplastic supratentorial ependymoma causing massive intratumoral calcification, without contributory medical and family history, and manifesting as persistent headache for 2 months. Physical examination found no neurological deficit except for visual defect in the right lower quadrant, with intact visual acuity. Blood examination showed no abnormalities. Cranial computed tomography revealed a huge calcified mass in the left parietooccipital lobe, with extensive perilesional brain edema. Cranial radiography showed diffuse and symmetrical thinning of the calvarial bone. Magnetic resonance imaging confirmed the tumor as an assembly of medullated masses with extraventricular location, 7 × 6.5 × 6.5 cm in diameter, and appearing as heterogeneous intensity on both T 1 -and T 2 -weighted images with inhomogeneous enhancement except for the central cores. The patient underwent tumor resection. Intraoperative findings revealed that the cortical veins overlying the tumor were reddish and moderately engorged. The hypervascular tumor, entirely extraventricular in location, was totally resected without neurological deterioration. Histological examination revealed that the tumor was highly cellular with hyperchromatic nuclei and cell atypia. Necrosis, mitotic figures, and perivascular pseudorosette formations were frequently seen. Immunohistochemical study showed positive staining for glial fibrillary acidic protein, S-100 protein, vimentin, and epithelial membrane antigen, but negative for synaptophysin. The MIB-1 labeling index was 26.5%. The findings were compatible with anaplastic ependymoma (World Health Organization classification grade 3). Ependymoma should be included in the differential diagnosis of a supratentorially located, extraventricular mass with massive intratumoral calcification.

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“…34 Recent genetic and molecular analysis has shown that location can also predict the tumor's biological behavior, particularly when comparing intracranial and spinal ependymomas. 4,6,16,26,27 Histological grade has been described as an important prognostic factor in some studies, 21,29,36 but not others. 8,15,17,28,50 Not surprisingly, there are differences in survival associated with histological grade, with poor outcomes in patients who have anaplastic (WHO Grade III) lesions, while those with Grade I and Grade II ependymomas have a more favorable prognosis.…”

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confidence: 99%

Surgical outcomes in spinal cord ependymomas and the importance of extent of resection in children and young adults

Safaee¹,

Mummaneni

et al. 2014

PED

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Object Ependymomas are a common type of CNS tumor in children, although only 13% originate from the spinal cord. Aside from location and extent of resection, the factors that affect outcome are not well understood. Methods The authors performed a search of an institutional neuropathology database to identify all patients with spinal cord ependymomas treated over the past 20 years. Data on patient age, sex, clinical presentation, symptom duration, tumor location, extent of resection, use of radiation therapy, surgical complications, presence of tumor recurrence, duration of follow-up, and residual symptoms were collected. Pediatric patients were defined as those 21 years of age or younger at diagnosis. The extent of resection was defined by the findings of the postoperative MR images. Results A total of 24 pediatric patients with spinal cord ependymomas were identified with the following pathological subtypes: 14 classic (Grade II), 8 myxopapillary (Grade I), and 2 anaplastic (Grade III) ependymomas. Both anaplastic ependymomas originated in the intracranial compartment and spread to the spinal cord at recurrence. The mean follow-up duration for patients with classic and myxopapillary ependymomas was 63 and 45 months, respectively. Seven patients with classic ependymomas underwent gross-total resection (GTR), while 4 received subtotal resection (STR), 2 received STR as well as radiation therapy, and 1 received radiation therapy alone. All but 1 patient with myxopapillary ependymomas underwent GTR. Three recurrences were identified in the Grade II group at 45, 48, and 228 months. A single recurrence was identified in the Grade I group at 71 months. The mean progression-free survival (PFS) was 58 months in the Grade II group and 45 months in the Grade I group. Conclusions Extent of resection is an important prognostic factor in all pediatric spinal cord ependymomas, particularly Grade II ependymomas. These data suggest that achieving GTR is more difficult in the upper spinal cord, making tumor location another important factor. Although classified as Grade I lesions, myxopapillary ependymomas had similar outcomes when compared with classic (Grade II) ependymomas, particularly with respect to PFS. Long-term complications or new neurological deficits were rare. Among patients with long-term follow-up, those who underwent GTR had a recurrence rate of 20% compared with 40% among those with STR or biopsy only, suggesting that extent of resection is perhaps a more important prognostic factor than histological grade in predicting PFS, which has been suggested by other data in the literature. Given the relative paucity of these lesions, collaborative multiinstitutional studies are needed, and such efforts should also focus on molecular and genetic analysis to refine the current classification system.

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Hyperfractionated radiotherapy and chemotherapy for childhood ependymoma: final results of the first prospective AIEOP (Associazione Italiana di Ematologia-Oncologia Pediatrica) study

Cited by 94 publications

References 52 publications

Pediatric Ependymoma: Biological Perspectives

Pediatric Ependymoma: Biological Perspectives

Huge Supratentorial Extraventricular Anaplastic Ependymoma Presenting With Massive Calcification -Case Report-

Surgical outcomes in spinal cord ependymomas and the importance of extent of resection in children and young adults

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