A modified IRS-III chemotherapy regimen leads to prolonged survival in children with embryonal tumor with multilayer rosettes

Neuro-oncology Advances
Derek HansonSusan Chi

Abstract

Embryonal tumor with multilayer rosettes (ETMR) is a rare CNS malignancy affecting young children that carries a very poor prognosis. Treatment with intensive surgical resection, radiotherapy, and high-dose chemotherapy is insufficient treatment in the vast majority of cases. Effective, biologically based therapies for this tumor are therefore desperately needed. The Dana-Farber Cancer Institute-modified IRS-III protocol incorporates preclinically active agents, such as doxorubicin and actinomycin D, into the treatment regimen for ETMR and may improve patient outcomes. The authors present a case series of 5 children with ETMR treated with an IRS-III-based chemotherapy backbone. All 5 patients received a gross-total tumor resection. Patients received between 12 and 51 weeks of IRS-III therapy at the discretion of their treating physician. Four patients received focal radiation therapy, with the fifth patient instead receiving a cycle of high-dose chemotherapy with autologous stem cell rescue. Four patients have progression-free survival of more than 18 months. Chemotherapy treatment was reasonably tolerated by all 5 patients with one case of mild sinusoidal obstructive syndrome and one case of Grade 3 peripheral neuropathy. The ...Continue Reading

References

Nov 7, 2008·The American Journal of Surgical Pathology·Marco GessiCharles G Eberhart
Dec 10, 2008·Journal of Clinical Oncology : Official Journal of the American Society of Clinical Oncology·Susan N ChiMark W Kieran
Oct 7, 2015·Child's Nervous System : ChNS : Official Journal of the International Society for Pediatric Neurosurgery·Meryl HorwitzNicolas Andre
May 26, 2018·Journal of Neuroimaging : Official Journal of the American Society of Neuroimaging·Bing WangLeena M Ketonen
Dec 26, 2018·Clinical and Translational Radiation Oncology·Sergio JaramilloSusan L McGovern
Mar 18, 2019·Journal of Neuro-oncology·Kayleen BaileyKim Kramer
Oct 12, 2019·Nature Reviews. Cancer·Costas D ArvanitisRakesh K Jain
Dec 6, 2019·Nature·Sander LamboMarcel Kool

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Methods Mentioned

BETA
xenograft
methylation profiling
surgical resection

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