MBCL-46. TREATMENT OF RECURRENT WINGLESS-ACTIVATED MEDULLOBLASTOMA (Wnt-MB) INCORPORATING MARROW-ABLATIVE THIOTEPA AND CARBOPLATIN CHEMOTHERAPY (HDCx) AND AUTOLOGOUS HEMATOPOIETIC PROGENITOR CELL RESCUE (AuHPCR): A DUAL REPORT. (4th December 2020)

• Record Type: Journal Article
• Authors: Harris, Micah K; Funk, Zachary N; Boué, Daniel R; Pierson, Christopher R; Jones, Jeremy; Leonard, Jeffrey; Abu-Arja, Rolla; Auletta, Jeffrey; Osorio, Diana S; Shatara, Margaret; Paul, Stephan R; Finlay, Jonathan L; AbdelBaki, Mohamed S
• Is Part Of: Neuro-oncology. Volume 22(2020)Supplement 3
• Page Start: iii397
• Page End: iii398
• Publication Date: 2020-12-04
• Subjects: Brain Neoplasms -- Periodicals; Brain -- Tumors -- Periodicals; Brain -- Cancer -- Periodicals; Nervous system -- Cancer -- Periodicals; 616.99481
• DOI: 10.1093/neuonc/noaa222.517
• Languages: English
• ISSNs: 1522-8517

Abstract

Abstract: BACKGROUND: Wnt-MB infers an excellent prognosis, and metastatic disease is rare. However, specific treatment strategies and patterns of failure for patients with recurrent Wnt-MB are unknown. We report two cases of recurrent beta-catenin nucleopositive Wnt-MBs treated with an irradiation-sparing strategy, incorporating HDCx/AuHPCR. PATIENT 1: A nine-year-old female experienced local recurrence of a non-metastatic Wnt-MB nine months after gross total resection (GTR) followed by 18Gy craniospinal irradiation (CSI) with primary site boost to 54Gy, accompanied by weekly vincristine, followed by a maintenance regimen of nine cycles of cisplatin/lomustine/vincristine alternating with cyclophosphamide/vincristine every third cycle. GTR of the relapsed tumor was followed by three cycles of HDCx/AuHPCR. She is disease-free for over three years following relapse treatment. PATIENT 2: A 17-year-old male initially underwent GTR, followed by 23.4Gy CSI with 54Gy posterior fossa boost with concomitant weekly vincristine, followed by a maintenance regimen that included nine alternating cycles of vincristine/lomustine/cisplatin and cyclophosphamide/vincristine. Isolated right frontal horn metastatic recurrence developed 19 months post-treatment; three cycles of irinotecan/temozolomide/bevacizumab and gamma-knife radiosurgery produced complete response. A second isolated metastatic recurrence within the left frontal horn occurred 13 months post-treatment, which was treated with two cycles of cyclophosphamide/etoposide followed by two cycles of HDCx/AuHPCR. MRI of the brain showed no residual tumor one month post-treatment. He currently awaits follow-up stereotactic radiosurgery. CONCLUSION: Patients with recurrent Wnt-MB may be treated with curative intent using a multi-disciplinary approach that includes HDCx/AuHPCR, and minimization or avoidance of re-irradiation.