TY - JOUR
T1 - Characterization of recurrence patterns and outcomes of medulloblastoma in adults
T2 - The University of Texas MD Anderson Cancer Center experience
AU - Gregory, Timothy A.
AU - Mastall, Maximilian
AU - Lin, Heather
AU - Hess, Kenneth R.
AU - Yuan, Ying
AU - Martin-Bejarano Garcia, Manuela
AU - Fuller, Gregory N.
AU - Alfaro, Kristin D.
AU - Gule-Monroe, Maria K.
AU - Huse, Jason T.
AU - Khatua, Soumen
AU - Rao, Ganesh
AU - Sandberg, David I.
AU - Wefel, Jeffrey S.
AU - Yeboa, Debra N.
AU - Paulino, Arnold C.
AU - McGovern, Susan L.
AU - Zaky, Wafik
AU - Mahajan, Anita
AU - Suki, Dima
AU - Weathers, Shiao Pei
AU - Harrison, Rebecca A.
AU - De Groot, John F.
AU - Puduvalli, Vinay K.
AU - Penas-Prado, Marta
AU - Majd, Nazanin K.
N1 - Publisher Copyright:
© 2023 The Author(s). Published by Oxford University Press, the Society for Neuro-Oncology and the European Association of Neuro-Oncology.
PY - 2023/1/1
Y1 - 2023/1/1
N2 - 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.
AB - 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.
KW - adult medulloblastoma
KW - recurrent medulloblastoma
KW - risk stratification
KW - treatment at recurrence
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U2 - 10.1093/noajnl/vdad032
DO - 10.1093/noajnl/vdad032
M3 - Article
C2 - 37114244
AN - SCOPUS:85160935863
SN - 2632-2498
VL - 5
JO - Neuro-Oncology Advances
JF - Neuro-Oncology Advances
IS - 1
ER -