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Outcomes with respect to extent of surgical resection for pediatric atypical teratoid rhabdoid tumors

Richards, Alexandra, Ved, Ronak, Murphy, Christopher, Hennigan, Dawn, Kilday, John-Paul, Kamaly-Asl, Ian, Mallucci, Conor, Bhatti, Imran, Patel, Chirag and Leach, Paul 2020. Outcomes with respect to extent of surgical resection for pediatric atypical teratoid rhabdoid tumors. Child's Nervous System 36 (4) , pp. 713-719. 10.1007/s00381-019-04478-5

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Purpose To evaluate overall survival for atypical teratoid rhabdoid tumors (ATRTs) in relation to extent of surgical resection. Methods The neurosurgical tumor databases from three UK Pediatric centers (University Hospital of Wales, Alder Hey and Royal Manchester Children’s Hospital) were analyzed. Patients with a diagnosis of ATRT were identified between 2000 and 2018. Data was collected regarding demographics, extent of resection, complications, and overall survival. Results Twenty-four patients diagnosed with ATRT underwent thirty-eight operations. The age range was 20 days to 147 months (median 17.5 months). The most common location for the tumor was the posterior fossa (nine patients; 38%). Six patients (25%) underwent a complete total resection (CTR), seven (29%) underwent a near total resection (NTR), eight (33.3%) underwent a subtotal resection (STR), and three patients (12.5%) had biopsy only. Two-thirds of patients who underwent a CTR are still alive, as of March 2019, compared to 29% in the NTR and 12.5% in the STR groups. Out of the thirty-eight operations, there were a total of twenty-two complications, of which the most common was pseudomeningocele (27%). The extent of surgical resection (p = 0.021), age at surgery (p = 0.00015), and the presence of metastases at diagnosis (0.015) significantly affected overall survival. Conclusions Although these patients are a highly vulnerable group, maximal resection is recommended where possible, for the best chance of long-term survival. However, near total resections are likely beneficial when compared with subtotal resections and biopsy alone. Maximal surgical resection should be combined with adjuvant therapies for the best long-term outcomes.

Item Type: Article
Date Type: Publication
Status: Published
Schools: Medicine
Additional Information: This article is licensed under a Creative Commons Attribution 4.0 International License.
Publisher: Springer Verlag (Germany)
ISSN: 0256-7040
Date of First Compliant Deposit: 14 May 2021
Date of Acceptance: 17 December 2019
Last Modified: 19 May 2021 15:45

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