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Characterization of recurrence patterns and outcomes of medulloblastoma in adults: The University of Texas MD Anderson Cancer Center experience

BACKGROUND: Medulloblastoma in adults is rare and treatment decisions are largely driven from pediatric literature. We sought to characterize recurrent medulloblastoma in adults. METHODS: From a single-institution dataset of 200 adult patients diagnosed with medulloblastoma during 1978–2017, those w...

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Detalles Bibliográficos
Autores principales: Gregory, Timothy A, Mastall, Maximilian, Lin, Heather, Hess, Kenneth R, Yuan, Ying, Martin-Bejarano Garcia, Manuela, Fuller, Gregory N, Alfaro, Kristin D, Gule-Monroe, Maria K, Huse, Jason T, Khatua, Soumen, Rao, Ganesh, Sandberg, David I, Wefel, Jeffrey S, Yeboa, Debra N, Paulino, Arnold C, McGovern, Susan L, Zaky, Wafik, Mahajan, Anita, Suki, Dima, Weathers, Shiao-Pei, Harrison, Rebecca A, de Groot, John F, Puduvalli, Vinay K, Penas-Prado, Marta, Majd, Nazanin K
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Oxford University Press 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10129387/
https://www.ncbi.nlm.nih.gov/pubmed/37114244
http://dx.doi.org/10.1093/noajnl/vdad032
Descripción
Sumario:BACKGROUND: Medulloblastoma in adults is rare and treatment decisions are largely driven from pediatric literature. We sought to characterize recurrent medulloblastoma in adults. METHODS: From a single-institution dataset of 200 adult patients diagnosed with medulloblastoma during 1978–2017, those with recurrence were analyzed for clinical features, treatment, and outcome. RESULTS: Of the 200 patients, 82 (41%) with median age of 29 years (18–59) had recurrence after a median follow-up time of 8.4 years (95% CI = 7.1, 10.3). Of these, 30 (37%) were standard-risk, 31 (38%) were high-risk, and 21 (26%) had unknown-risk diseases at the time of initial diagnosis. Forty-eight (58%) presented with recurrence outside the posterior fossa, of whom 35 (43%) had distant recurrence only. Median Progression-free survival (PFS) and OS from initial surgery were 33.5 and 62.4 months, respectively. Neither PFS nor OS from initial diagnosis differed between the standard-risk and high-risk groups in those who experience recurrence (P = .505 and .463, respectively). Median OS from first recurrence was 20.3 months, also with no difference between the standard-risk and high-risk groups (P = .518). Recurrences were treated with combinations of re-resection (20 patients; 25%), systemic chemotherapy (61 patients; 76%), radiation (29 patients; 36%), stem cell transplant (6 patients; 8%), and intrathecal chemotherapy (4 patients; 5%). Patients who received radiation at recurrence had better OS (32.9 months) than those who did not (19.2 months) (P = .034). CONCLUSIONS: Recurrent medulloblastoma in adults has a poor prognosis irrespective of initial risk stratification. Recurrence commonly arises outside the posterior fossa years after initial diagnosis.