Open AccessPearls & Oy-sters: Pivoting Treatment Regimens of Pediatric Atypical Teratoid Rhabdoid Tumors to Optimize Care in Adult ATRT: A Case Report
Author(s) -
Rohan Rao,
Abigail Koehler,
Yehudit Rothman,
Brandi Turner,
Jamie Denlinger,
Melissa Erickson,
Matthew Hagen,
Timothy Braverman,
Abdelkader Mahammedi,
Karl C. Golnik,
Mario Zuccarello,
Yair M. Gozal,
E. Randolph Broun,
N Susan,
Soma Sengupta
Publication year - 2022
Publication title -
neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.674
H-Index - 29
eISSN - 1526-632X
pISSN - 0028-3878
DOI - 10.1212/wnl.0000000000200196
Subject(s) - atypical teratoid rhabdoid tumor , medicine , headaches , chemotherapy , radiation therapy , cyclophosphamide , vincristine , oncology , pediatrics , surgery , pathology , medulloblastoma
Atypical teratoid rhabdoid tumor (ATRT) is a highly malignant embryonal tumor of the CNS, largely affecting pediatric patients, with exceedingly rare cases in adults at an estimated annual incidence of 1/1,000,000. We report a unique case of ATRT in a 43-year-old female patient who first presented with progressive focal headaches. Imaging revealed a sellar mass with suprasellar extensions, which was partially removed via a transsphenoidal resection. The tumor aggressively recurred just 1 month postoperatively. Her care team pursued a novel treatment plan by using a slightly modified COG ACNS 0332 regimen, which involved radiation, followed by 4 cycles of monthly chemotherapy including vincristine, cyclophosphamide, and cisplatin. Hematopoietic stem cells were collected between radiation and chemotherapy in the event that the patient required stem cell salvage therapy postadjuvant chemotherapy. The MRIs taken at 2 and 4 months postrecurrence indicated a substantial decrease in tumor volume, with corresponding clinical improvements to cranial nerve deficits. Given the scarcity of literature on adult cases of ATRT and the lack of a standard of care for these cases, discussing the efficacy of our patient's treatment plan may aid clinical decision making for adult ATRT cases.