Intensive pediatric chemotherapy regimen (PNET HR+5) in adult high-risk medulloblastoma and pineoblastoma patients

Louis Larrouquere, Christelle Dufour, Cécile Faure-Conter, Claire Alapetite, David Meyronet, Stéphanie Bolle, Alice Bonneville-Levard, Marie Pierre Sunyach, Valérie Laurence, Didier Frappaz

    Résultats de recherche: Contribution à un journalArticleRevue par des pairs

    Résumé

    Background. High-risk medulloblastoma (HRMB) is rare in adults. The 5-year overall survival rate is less than 60%. We present here a retrospective analysis of adults treated with an intensive pediatric chemo-radiotherapy regimen PNET HR + 5: NCT00936156. Methods. Eighteen patients over the age of 20 (range, 20–33 years) with HRMB (n = 13), pinealoblastoma (n = 4), and central nervous system embryonal tumor (n = 1) were treated with 2 courses of carboplatin-etoposide followed by 2 courses of high-dose thiotepa (HDT) with autologous hematopoietic stem-cell rescue. A craniospinal irradiation (CSI; 36 Gy craniospinal axis then a boost of 18 Gy to the primary tumor site) was then initiated within 150 days of surgery, completed with 6 cycles of temozolomide; the axis irradiation was not mandatory for non-metastatic pinealoblastoma. Results. We observed no progression under chemotherapy and no toxic death. Four patients received only 1 HDT. Two non-metastatic pinaloblastomas received only focal irradiation. One medulloblastoma received only 25 Gy on the axis. 56% (10/18) received 6 cycles of temozolomide. No long-term toxicity was recorded. The median time between surgery and CSI was 175 days (range, 115–250). With a median follow-up of 6.0 years (range, 2.6–9), the progression-free survival and overall survival rates for medulloblastoma were respectively 65% (95% CI: 31%–86%) and 76% (95% CI: 42%–91%) at 5 years. Conclusions. The PNET HR + 5 regimen showed promising results in an adult population, with a meaningful improvement in progression-free survival and overall survival in patients with HRMB.

    langue originaleAnglais
    Numéro d'articlevdae141
    journalNeuro-Oncology Advances
    Volume6
    Numéro de publication1
    Les DOIs
    étatPublié - 1 janv. 2024

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