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

Abstract

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.

A review of Leila Rose Foundation support for families affected by rare childhood cancer in Australia over the past decade

“… 15 Similarly, the Foundation's figures illustrate that atypical teratoid rhabdoid tumor patients have been enrolled with an age range of 1 to 4 years and a mean average of 2 years which is again in line with the published literature which reflects a peak diagnosis at less than 3 years of age. 16 Retinoblastomas, have an age range of 6 months to 4 years at enrollment with a mean average of 1 year, 11 months which is consistent with the data provided in the American Cancer Society, Cancer Facts & Figures 2020 suggesting that the average age of children at diagnosis is 2 years. 17 If nothing else this supports the idea that for these tumors at least, the period between diagnosis and enrolment with the Foundation is very short.…”

Atypical teratoid rhabdoid tumor in the cerebellum of a 7‐year‐old boy presenting with headache after a fall

“…13 Improved outcomes have also been associated with multimodal therapy, radiotherapy in patients older than 3 years of age, and gross total resection compared with less extensive surgery. 14,15 Our patient had no metastasis and underwent gross total resection. He was subsequently enrolled in the Saint Jude's Phase 2 Study of Alisertib Therapy for Rhabdoid Tumors (SJATRT) 16 and received craniospinal radiation along with Alisertib treatment.…”

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

“…It has been shown that the extent of resection is critical to ATRT patient outcomes. Richards et al revealed that complete total resection had a significantly higher overall survival (66.6%) compared to near-total resection (29%) or subtotal resection (12.5%) in children with newly diagnosed ATRT [5].…”

“…A study revealed that complete total resection had a significantly higher overall survival (66.6%) compared with near-total resection (29%) or subtotal resection (12.5%) in children with newly diagnosed ATRT. 5…”

“…A study revealed that complete total resection had a significantly higher overall survival (66.6%) compared with near-total resection (29%) or subtotal resection (12.5%) in children with newly diagnosed ATRT. 5 Pediatric ATRT is treated with aggressive IV or intrathecal chemotherapy rather than RT due to the possible negative neurocognitive effects. However, recent research suggests that even in pediatric populations, early RT may prolong progression-free survival.…”