Pearls & Oy-sters: Pivoting Treatment Regimens of Pediatric Atypical Teratoid Rhabdoid Tumors to Optimize Care in Adult ATRT: A Case Report

Rohan Rao, From the Department of Neurology and Rehabilitation Medicine (R.R., A.K., Y.R., S.S.), University of Cincinnati College of Medicine; Barrett Cancer Center (B.T., J.D.), University of Cincinnati; Departments of Family and Community Medicine (M.E.) and Pathology & Laboratory Medicine (M.H.), University of Cincinnati College of Medicine; Department of Anatomy & Clinical Pathology (T.S.B.), Jewish Hospital; Departments of Radiology (A.M.), Ophthalmology (K.G.) and Neurosurgery (M.Z.), University of Cincinnati College of Medicine; Mayfield Clinic (Y.M.G.); Department of Hematology Oncology (E.R.B.), Jewish Hospital, Cincinnati, OH; and Department of Pediatric Neuro-Oncology (S.N.C.), Dana Farber/Boston Children's Cancer and Blood Disorder Center, Boston, MA.
Abigail Koehler, From the Department of Neurology and Rehabilitation Medicine (R.R., A.K., Y.R., S.S.), University of Cincinnati College of Medicine; Barrett Cancer Center (B.T., J.D.), University of Cincinnati; Departments of Family and Community Medicine (M.E.) and Pathology & Laboratory Medicine (M.H.), University of Cincinnati College of Medicine; Department of Anatomy & Clinical Pathology (T.S.B.), Jewish Hospital; Departments of Radiology (A.M.), Ophthalmology (K.G.) and Neurosurgery (M.Z.), University of Cincinnati College of Medicine; Mayfield Clinic (Y.M.G.); Department of Hematology Oncology (E.R.B.), Jewish Hospital, Cincinnati, OH; and Department of Pediatric Neuro-Oncology (S.N.C.), Dana Farber/Boston Children's Cancer and Blood Disorder Center, Boston, MA.
Yehudit Rothman, From the Department of Neurology and Rehabilitation Medicine (R.R., A.K., Y.R., S.S.), University of Cincinnati College of Medicine; Barrett Cancer Center (B.T., J.D.), University of Cincinnati; Departments of Family and Community Medicine (M.E.) and Pathology & Laboratory Medicine (M.H.), University of Cincinnati College of Medicine; Department of Anatomy & Clinical Pathology (T.S.B.), Jewish Hospital; Departments of Radiology (A.M.), Ophthalmology (K.G.) and Neurosurgery (M.Z.), University of Cincinnati College of Medicine; Mayfield Clinic (Y.M.G.); Department of Hematology Oncology (E.R.B.), Jewish Hospital, Cincinnati, OH; and Department of Pediatric Neuro-Oncology (S.N.C.), Dana Farber/Boston Children's Cancer and Blood Disorder Center, Boston, MA.
Brandi Turner, From the Department of Neurology and Rehabilitation Medicine (R.R., A.K., Y.R., S.S.), University of Cincinnati College of Medicine; Barrett Cancer Center (B.T., J.D.), University of Cincinnati; Departments of Family and Community Medicine (M.E.) and Pathology & Laboratory Medicine (M.H.), University of Cincinnati College of Medicine; Department of Anatomy & Clinical Pathology (T.S.B.), Jewish Hospital; Departments of Radiology (A.M.), Ophthalmology (K.G.) and Neurosurgery (M.Z.), University of Cincinnati College of Medicine; Mayfield Clinic (Y.M.G.); Department of Hematology Oncology (E.R.B.), Jewish Hospital, Cincinnati, OH; and Department of Pediatric Neuro-Oncology (S.N.C.), Dana Farber/Boston Children's Cancer and Blood Disorder Center, Boston, MA.
Jamie Denlinger, From the Department of Neurology and Rehabilitation Medicine (R.R., A.K., Y.R., S.S.), University of Cincinnati College of Medicine; Barrett Cancer Center (B.T., J.D.), University of Cincinnati; Departments of Family and Community Medicine (M.E.) and Pathology & Laboratory Medicine (M.H.), University of Cincinnati College of Medicine; Department of Anatomy & Clinical Pathology (T.S.B.), Jewish Hospital; Departments of Radiology (A.M.), Ophthalmology (K.G.) and Neurosurgery (M.Z.), University of Cincinnati College of Medicine; Mayfield Clinic (Y.M.G.); Department of Hematology Oncology (E.R.B.), Jewish Hospital, Cincinnati, OH; and Department of Pediatric Neuro-Oncology (S.N.C.), Dana Farber/Boston Children's Cancer and Blood Disorder Center, Boston, MA.
Melissa Erickson, From the Department of Neurology and Rehabilitation Medicine (R.R., A.K., Y.R., S.S.), University of Cincinnati College of Medicine; Barrett Cancer Center (B.T., J.D.), University of Cincinnati; Departments of Family and Community Medicine (M.E.) and Pathology & Laboratory Medicine (M.H.), University of Cincinnati College of Medicine; Department of Anatomy & Clinical Pathology (T.S.B.), Jewish Hospital; Departments of Radiology (A.M.), Ophthalmology (K.G.) and Neurosurgery (M.Z.), University of Cincinnati College of Medicine; Mayfield Clinic (Y.M.G.); Department of Hematology Oncology (E.R.B.), Jewish Hospital, Cincinnati, OH; and Department of Pediatric Neuro-Oncology (S.N.C.), Dana Farber/Boston Children's Cancer and Blood Disorder Center, Boston, MA.
Matthew Hagen, From the Department of Neurology and Rehabilitation Medicine (R.R., A.K., Y.R., S.S.), University of Cincinnati College of Medicine; Barrett Cancer Center (B.T., J.D.), University of Cincinnati; Departments of Family and Community Medicine (M.E.) and Pathology & Laboratory Medicine (M.H.), University of Cincinnati College of Medicine; Department of Anatomy & Clinical Pathology (T.S.B.), Jewish Hospital; Departments of Radiology (A.M.), Ophthalmology (K.G.) and Neurosurgery (M.Z.), University of Cincinnati College of Medicine; Mayfield Clinic (Y.M.G.); Department of Hematology Oncology (E.R.B.), Jewish Hospital, Cincinnati, OH; and Department of Pediatric Neuro-Oncology (S.N.C.), Dana Farber/Boston Children's Cancer and Blood Disorder Center, Boston, MA.
Timothy S. Braverman, From the Department of Neurology and Rehabilitation Medicine (R.R., A.K., Y.R., S.S.), University of Cincinnati College of Medicine; Barrett Cancer Center (B.T., J.D.), University of Cincinnati; Departments of Family and Community Medicine (M.E.) and Pathology & Laboratory Medicine (M.H.), University of Cincinnati College of Medicine; Department of Anatomy & Clinical Pathology (T.S.B.), Jewish Hospital; Departments of Radiology (A.M.), Ophthalmology (K.G.) and Neurosurgery (M.Z.), University of Cincinnati College of Medicine; Mayfield Clinic (Y.M.G.); Department of Hematology Oncology (E.R.B.), Jewish Hospital, Cincinnati, OH; and Department of Pediatric Neuro-Oncology (S.N.C.), Dana Farber/Boston Children's Cancer and Blood Disorder Center, Boston, MA.
Abdelkader Mahammedi, From the Department of Neurology and Rehabilitation Medicine (R.R., A.K., Y.R., S.S.), University of Cincinnati College of Medicine; Barrett Cancer Center (B.T., J.D.), University of Cincinnati; Departments of Family and Community Medicine (M.E.) and Pathology & Laboratory Medicine (M.H.), University of Cincinnati College of Medicine; Department of Anatomy & Clinical Pathology (T.S.B.), Jewish Hospital; Departments of Radiology (A.M.), Ophthalmology (K.G.) and Neurosurgery (M.Z.), University of Cincinnati College of Medicine; Mayfield Clinic (Y.M.G.); Department of Hematology Oncology (E.R.B.), Jewish Hospital, Cincinnati, OH; and Department of Pediatric Neuro-Oncology (S.N.C.), Dana Farber/Boston Children's Cancer and Blood Disorder Center, Boston, MA.
Karl Golnik, From the Department of Neurology and Rehabilitation Medicine (R.R., A.K., Y.R., S.S.), University of Cincinnati College of Medicine; Barrett Cancer Center (B.T., J.D.), University of Cincinnati; Departments of Family and Community Medicine (M.E.) and Pathology & Laboratory Medicine (M.H.), University of Cincinnati College of Medicine; Department of Anatomy & Clinical Pathology (T.S.B.), Jewish Hospital; Departments of Radiology (A.M.), Ophthalmology (K.G.) and Neurosurgery (M.Z.), University of Cincinnati College of Medicine; Mayfield Clinic (Y.M.G.); Department of Hematology Oncology (E.R.B.), Jewish Hospital, Cincinnati, OH; and Department of Pediatric Neuro-Oncology (S.N.C.), Dana Farber/Boston Children's Cancer and Blood Disorder Center, Boston, MA.
Mario Zuccarello, From the Department of Neurology and Rehabilitation Medicine (R.R., A.K., Y.R., S.S.), University of Cincinnati College of Medicine; Barrett Cancer Center (B.T., J.D.), University of Cincinnati; Departments of Family and Community Medicine (M.E.) and Pathology & Laboratory Medicine (M.H.), University of Cincinnati College of Medicine; Department of Anatomy & Clinical Pathology (T.S.B.), Jewish Hospital; Departments of Radiology (A.M.), Ophthalmology (K.G.) and Neurosurgery (M.Z.), University of Cincinnati College of Medicine; Mayfield Clinic (Y.M.G.); Department of Hematology Oncology (E.R.B.), Jewish Hospital, Cincinnati, OH; and Department of Pediatric Neuro-Oncology (S.N.C.), Dana Farber/Boston Children's Cancer and Blood Disorder Center, Boston, MA.
Yair M. Gozal, From the Department of Neurology and Rehabilitation Medicine (R.R., A.K., Y.R., S.S.), University of Cincinnati College of Medicine; Barrett Cancer Center (B.T., J.D.), University of Cincinnati; Departments of Family and Community Medicine (M.E.) and Pathology & Laboratory Medicine (M.H.), University of Cincinnati College of Medicine; Department of Anatomy & Clinical Pathology (T.S.B.), Jewish Hospital; Departments of Radiology (A.M.), Ophthalmology (K.G.) and Neurosurgery (M.Z.), University of Cincinnati College of Medicine; Mayfield Clinic (Y.M.G.); Department of Hematology Oncology (E.R.B.), Jewish Hospital, Cincinnati, OH; and Department of Pediatric Neuro-Oncology (S.N.C.), Dana Farber/Boston Children's Cancer and Blood Disorder Center, Boston, MA.
E Randolph Broun, From the Department of Neurology and Rehabilitation Medicine (R.R., A.K., Y.R., S.S.), University of Cincinnati College of Medicine; Barrett Cancer Center (B.T., J.D.), University of Cincinnati; Departments of Family and Community Medicine (M.E.) and Pathology & Laboratory Medicine (M.H.), University of Cincinnati College of Medicine; Department of Anatomy & Clinical Pathology (T.S.B.), Jewish Hospital; Departments of Radiology (A.M.), Ophthalmology (K.G.) and Neurosurgery (M.Z.), University of Cincinnati College of Medicine; Mayfield Clinic (Y.M.G.); Department of Hematology Oncology (E.R.B.), Jewish Hospital, Cincinnati, OH; and Department of Pediatric Neuro-Oncology (S.N.C.), Dana Farber/Boston Children's Cancer and Blood Disorder Center, Boston, MA.
Susan N. Chi, From the Department of Neurology and Rehabilitation Medicine (R.R., A.K., Y.R., S.S.), University of Cincinnati College of Medicine; Barrett Cancer Center (B.T., J.D.), University of Cincinnati; Departments of Family and Community Medicine (M.E.) and Pathology & Laboratory Medicine (M.H.), University of Cincinnati College of Medicine; Department of Anatomy & Clinical Pathology (T.S.B.), Jewish Hospital; Departments of Radiology (A.M.), Ophthalmology (K.G.) and Neurosurgery (M.Z.), University of Cincinnati College of Medicine; Mayfield Clinic (Y.M.G.); Department of Hematology Oncology (E.R.B.), Jewish Hospital, Cincinnati, OH; and Department of Pediatric Neuro-Oncology (S.N.C.), Dana Farber/Boston Children's Cancer and Blood Disorder Center, Boston, MA.
Soma Sengupta, From the Department of Neurology and Rehabilitation Medicine (R.R., A.K., Y.R., S.S.), University of Cincinnati College of Medicine; Barrett Cancer Center (B.T., J.D.), University of Cincinnati; Departments of Family and Community Medicine (M.E.) and Pathology & Laboratory Medicine (M.H.), University of Cincinnati College of Medicine; Department of Anatomy & Clinical Pathology (T.S.B.), Jewish Hospital; Departments of Radiology (A.M.), Ophthalmology (K.G.) and Neurosurgery (M.Z.), University of Cincinnati College of Medicine; Mayfield Clinic (Y.M.G.); Department of Hematology Oncology (E.R.B.), Jewish Hospital, Cincinnati, OH; and Department of Pediatric Neuro-Oncology (S.N.C.), Dana Farber/Boston Children's Cancer and Blood Disorder Center, Boston, MA.

Document Type

Article

Abstract

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.

Medical Subject Headings

Adult; Central Nervous System Neoplasms (diagnostic imaging, drug therapy); Child; Female; Humans; Neoplasm Recurrence, Local; Neoplasms, Germ Cell and Embryonal; Rhabdoid Tumor (diagnostic imaging, drug therapy, surgery); Teratoma (diagnostic imaging, drug therapy, surgery)

Publication Date

4-26-2022

Publication Title

Neurology

E-ISSN

1526-632X

Volume

98

Issue

17

First Page

726

Last Page

730

PubMed ID

35256482

Digital Object Identifier (DOI)

10.1212/WNL.0000000000200196

Link to Full Text

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