title: Neoadjuvant chemotherapy for atypical teratoid rhabdoid tumors: case report.
abstract: Atypical teratoid rhabdoid tumors (ATRTs) are a rare pediatric brain tumor with high mortality rate. Several large series have reported achieving gross-total resection (GTR) in less than 50% of patients due to the lesions' large size, vascularity, and limited blood volume in young patients. While neoadjuvant chemotherapy for choroid plexus carcinomas in pediatric patients has become widely accepted, it has not been used as widely for other pediatric brain tumors. To the best of the authors' knowledge, there are only 3 published cases of neoadjuvant chemotherapy for ATRTs. In the present report, the authors present a fourth case of neoadjuvant chemotherapy for ATRT and review the available literature on this strategy. A 17-month-old child presented with a left ventricular ATRT for which imaging raised concern for a highly vascularized tumor. The authors undertook neoadjuvant chemotherapy with 2 cycles of Head Start II therapy, which reduced the size of the ventricular tumor by 35% and decreased the vascularity of the lesion on imaging. The estimated blood loss during resection was 425 ml and GTR was achieved. The patient continued with postoperative chemotherapy but suffered an on-therapy recurrence. While higher-quality data are necessary, available evidence suggests that neoadjuvant chemotherapy can reduce the size and vascularity of ATRTs and facilitate a surgical avenue for large or "inoperable" tumors.
authors.affiliation: Department of Neurosurgery, University of Louisville School of Medicine.
authors.forename: Meena
authors.initials: M
authors.lastname: Thatikunta
authors.affiliation: Department of Neurosurgery, University of Louisville School of Medicine.|Pediatric Neurosurgery, Norton Children's Hospital.|Norton Neuroscience Institute, Louisville, Kentucky.
authors.forename: Ian
authors.initials: I
authors.lastname: Mutchnick
authors.affiliation: Pediatric Hematology/Oncology, UC San Diego School of Medicine.|Rady Children's Hospital-San Diego, California.
authors.forename: Jennifer
authors.initials: J
authors.lastname: Elster
authors.affiliation: Pediatric and Perinatal Pathology Associates, Norton Children's Hospital.
authors.forename: Matthew P
authors.initials: MP
authors.lastname: Thompson
authors.affiliation: Pediatric Cancer & Blood Disorders, University of Louisville School of Medicine; and.
authors.forename: Michael A
authors.initials: MA
authors.lastname: Huang
authors.affiliation: Radiation Oncology, Norton Cancer Institute, Norton Healthcare, Louisville, Kentucky.
authors.forename: Aaron C
authors.initials: AC
authors.lastname: Spalding
authors.affiliation: Department of Neurosurgery, University of Louisville School of Medicine.|Pediatric Neurosurgery, Norton Children's Hospital.|Norton Neuroscience Institute, Louisville, Kentucky.
authors.forename: Thomas
authors.initials: T
authors.lastname: Moriarty
mesh_terms: D001921:Brain / Q000000981:diagnostic imaging / Q000601:surgery; D001932:Brain Neoplasms / Q000134:cerebrospinal fluid / Q000000981:diagnostic imaging / Q000188:drug therapy* / Q000473:pathology; D017809:Fatal Outcome; D006801:Humans; D007223:Infant; D008297:Male; D020360:Neoadjuvant Therapy; D018335:Rhabdoid Tumor / Q000134:cerebrospinal fluid / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery; D013724:Teratoma / Q000134:cerebrospinal fluid / Q000188:drug therapy* / Q000473:pathology / Q000601:surgery
keywords: ATRT; ATRT = atypical teratoid rhabdoid tumor; CPC = choroid plexus carcinoma; EBL/EBV = estimated blood loss/estimated blood volume; EVD = external ventricular drain; GTR = gross-total resection; Head Start; NTR = near-total resection; SIADH = syndrome of inappropriate antidiuretic hormone; atypical teratoid rhabdoid tumor; choroid plexus carcinoma; gross-total resection; neoadjuvant chemotherapy; oncology; pediatric brain tumor