Frequency, Risk‐Factors and Survival of Children With Atypical Teratoid Rhabdoid Tumors (AT/RT) of the CNS Diagnosed between 1988 and 2004, and Registered to the German HIT Database

Abstract

The incidence of AT/RT in children below 1 year is higher than previously reported. A subset of patients with favorable clinical risk factors profits from intensive multimodal treatment. Prospective clinical and biological studies are needed to further define prognostic factors and optimize therapy.

Survival outcomes in atypical teratoid rhabdoid tumor for patients undergoing radiotherapy in a Surveillance, Epidemiology, and End Results analysis

“…Gross total resection was achieved in 39% of patients. Maximal primary tumor resection has been associated with improved OS and progression-free survival 9,11,13 ; however, we did not observe an association between the extent of resection and OS. The benefits of greater tumor resection have not been consistent in other studies, suggesting that resection alone is not sufficient for long-term survivorship in patients with ATRT and establishing the need for adequate adjuvant therapy.…”

“…The benefits of greater tumor resection have not been consistent in other studies, suggesting that resection alone is not sufficient for long-term survivorship in patients with ATRT and establishing the need for adequate adjuvant therapy. 9,10,12,14 Data describing chemotherapeutic regimens were not available for analysis; however, chemotherapy often is administered in the initial postoperative period to patients with ATRT. Because most children aged 3 years receive chemotherapy as a way of postponing RT, chemotherapeutic strategies for ATRT have been among the best studied issues in ATRT management.…”

“…1,4,8 However, it is believed that the true incidence may be higher because of misdiagnosis given similarities in imaging and histologic appearance to other high-grade embryonal CNS malignancies. 2,3,8,9 Current knowledge describing clinical characteristics, prognosis, and therapy for ATRT is derived mostly from small, retrospective, observational studies. The prognosis for patients with ATRT remains dismal, and the historic median overall survival (OS) ranges from 6 months to 18 months.…”

“…The prognosis for patients with ATRT remains dismal, and the historic median overall survival (OS) ranges from 6 months to 18 months. 1,3,4,9,10 Therapeutic approaches for ATRT historically have relied on data from treatments of other childhood CNS tumors, particularly primitive neuroendocrine tumors and medulloblastoma, which have a considerably better prognoses. 5 However, preliminary results from the first prospective clinical trial in children with ATRT indicate that intensive multimodality treatment, including resection, CNS radiotherapy (RT), and chemotherapy based on the Intergroup Rhabdomyosarcoma Study Group (IRS) III protocol, may lead to longer survival, with an estimated 2-year OS survival rate of 70% AE 10%.…”

“…Given the rarity of ATRT, large retrospective analyses are important sources of information to guide the development of treatment strategies. 1,9,10,[12][13][14][15] The objectives of the current study were to identify clinical and prognostic factors for ATRT and to determine whether the use of RT has changed in the last decade in light of information suggesting its importance in the management of ATRT using the National Cancer Institute (NCI) Surveillance, Epidemiology, and End Results (SEER) database. The SEER Program collects cancer survival and incidence information from population-based cancer registries that encompass approximately 28% of the US population, which provides an opportunity to study rare entities like ATRT.…”