Analysis of patients with supratentorial primitive neuro-ectodermal tumours entered into the SIOP/UKCCSG PNET 3 study

Pizer, Barry; Weston, Claire; Robinson, K. L.; Ellison, David W.; Ironside, James W.; Saran, Frank; Lashford, L. S.; Tait, Diana; Lucraft, Helen; Walker, David; Bailey, Chris; Taylor, Roger

doi:10.1016/j.ejca.2006.01.039

Cited by 103 publications

(58 citation statements)

References 18 publications

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“…The 3-year and 5-year OS in the combination group was 52.3% and 45% respectively, compared with a 3-year and 5-year OS of 58.3 % and 54.2%, respectively, in patients just treated with radiation therapy. 59 A combined analysis of 17 patients enrolled in the CCG 9883 and MSKCC-89-173 trials investigated high-dose chemotherapy (combination of thiotepa, etoposide, with or without carboplatin) with ASCR specifically for patients with recurrent sPNET. Ten patients experienced tumor relapse at a median of 160 days after ASCR and died.…”

Section: Chemotherapymentioning

confidence: 99%

Pediatric Brain Tumors: Current Treatment Strategies and Future Therapeutic Approaches

2009

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Summary:Pediatric CNS tumors are the most common solid tumors of childhood and the second most common cancer after hematological malignancies accounting for approximate 20 to 25% of all primary pediatric tumors. With over 3,000 new cases per year in the United States, childhood CNS tumors are the leading cause of death related to cancer in this population. The prognosis for these patients has improved over the last few decades, but current therapies continue to carry a high risk of significant side effects, especially for the very young. Currently a combination of surgery, radiation, and chemotherapy is often used in children greater than 3 years of age. This article will outline current and future therapeutic strategies for the most common pediatric CNS tumors, including primitive neuroectodermal tumors such as medulloblastoma, as well as astrocytomas and ependymomas.

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Section: Chemotherapymentioning

confidence: 99%

Pediatric Brain Tumors: Current Treatment Strategies and Future Therapeutic Approaches

2009

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“…Despite multi-modal therapy, less than half of affected patients will survive 5 years post-diagnosis, and late effects associated with current treatment protocols are a significant complication in survivors. 1 Advances in our understanding of the molecular basis of sPNET development will be critical to improve outcome. The biological mechanisms underlying sPNET pathogenesis, however, remain poorly understood and afford few opportunities for the application of novel or targeted therapeutic approaches to achieve this objective.…”

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confidence: 99%

Frequent IDH1 mutations in supratentorial primitive neuroectodermal tumors (sPNET) of adults but not children

Hayden¹,

Frühwald²,

Hasselblatt³

et al. 2009

Cell Cycle

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“…3,5) Compared to other embryonic malignant tumors such as medulloblastoma with better established treatment regimes, the optimum treatment of this rare and heterogeneous tumor group is still the subject of research and many clinical studies are ongoing. 10,14,19,22) Today, surgery is recommended for the care of PNET patients (À3 years of age) followed by radiation 14) and chemotherapy. 8,20) No standards have been established, high dose chemotherapy with different regimes may be better before radiation in younger children.…”

Section: Discussionmentioning

confidence: 99%

Small Supratentorial, Extraaxial Primitive Neuroectodermal Tumor Causing Large Intracerebral Hematoma -Case Report-

Burkhardt

Kockro

Dohmen-Scheufler

et al. 2011

Neurol. Med. Chir.(Tokyo)

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A 16-year-old boy presented with an unusual case of a supratentorial, extraaxial small round blue cell tumor of the central nervous system, which was most likely a primitive neuroectodermal tumor (PNET). Preoperative computed tomography and magnetic resonance imaging showed a large multistage hematoma in the left central region. Intraoperatively, a small, superficial tumorous lesion was found between the sagittal sinus and a large cortical vein hidden by the hematoma. The histological diagnosis was PNET. This tumor is one of the most aggressive intracerebral tumors, not only in children, so treatment strategies must be early, profound, and interdisciplinary. This case represents an important example of atypical extraaxial appearance of this lesion, which should be considered in the differential diagnosis of cortical or subcortical hemorrhage, since complete resection of this lesion is critical for the successful treatment and outcome.

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Analysis of patients with supratentorial primitive neuro-ectodermal tumours entered into the SIOP/UKCCSG PNET 3 study

Cited by 103 publications

References 18 publications

Pediatric Brain Tumors: Current Treatment Strategies and Future Therapeutic Approaches

Pediatric Brain Tumors: Current Treatment Strategies and Future Therapeutic Approaches

Frequent IDH1 mutations in supratentorial primitive neuroectodermal tumors (sPNET) of adults but not children

Small Supratentorial, Extraaxial Primitive Neuroectodermal Tumor Causing Large Intracerebral Hematoma -Case Report-

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