Survival benefit with gross total resection and adjuvant radiotherapy in childhood atypical teratoid/rhabdoid tumors: results of a single-center cohort of 27 cases

Abstract

AIM:To share a single center experience with 27 atypical teratoid/rhabdoid tumor (AT/RT) cases, and to determine the effect of gross total tumor resection and other clinical characteristics on the overall survival rate of AT/RT. MATERIAL and METHODS:We included 27 patients-with a histopathologically confirmed primary intracranial childhood AT/ RT-who were operated in our clinic between January 2000 and December 2017. Age, sex, tumor location, disseminated disease, the presence of hydrocephalus, symptom duration till diagnosis, the extent of resection, and adjuvant radiotherapy were evaluated for their influence on overall survival. RESULTS:Median age at diagnosis for 27 patients was 19.1 months (7.2 months-5 years). Gross total resection was possible in 13 (48.72%) patients. Except for three patients who died of perioperative complications, all patients received chemotherapy and 11 received radiotherapy. In univariate analysis, male sex, older age at diagnosis (≥24 months), gross total resection, and radiotherapy were associated with overall longer survival; however, radiotherapy remained the only significant parameter in multivariate analysis. CONCLUSION:AT/RT is a rare and dreadful brain tumor that has low survival rates despite contemporary treatment. Radiotherapy seems to prolong survival; however, large-scale studies are needed to establish prognostic factors.

Neurocognitive Effects and Necrosis in Childhood Cancer Survivors Treated With Radiation Therapy: A PENTEC Comprehensive Review

“…More specifically, approximately 3300 new benign or malignant primary CNS tumors are diagnosed annually in patients younger than age 16 years in the United States, and in this age group, brain tumors are the most common solid tumor indication for RT. Many of these patients require large-field radiation such as craniospinal irradiation (CSI) and/or focal RT (45)(46)(47)(48)(49)(50)(51)(52)(53)(54)(55)(56)(57)(58)(59)(60).…”

“…For example, despite reluctance to expose children younger than 3 years of age to any cranial RT, optimal disease control and survival for children as young as 18 months with ependymoma, medulloblastoma, or atypical teratoid rhabdoid tumors appear to justify RT. [49][50][51][52] Reirradiation is being increasingly used as definitive management of locally recurrent disease despite the increased risk of brain necrosis; however, specific reirradiation guidelines are lacking. [53][54][55][56]…”

Molecular targeted therapies for pediatric atypical teratoid/rhabdoid tumors

“…Some studies have reported a tendency to relapse among patients who receive delayed radiotherapy. [18][19][20] Other studies reported that the risks of delayed radiotherapy were negligible among patients who received tailored regimens. 3,21,22 After extensive explorations of optimal treatment strategies, survival outcomes have substantially improved among patients with AT/RTs.…”

“…Some studies have reported a tendency to relapse among patients who receive delayed radiotherapy. 18 , 19 , 20 Other studies reported that the risks of delayed radiotherapy were negligible among patients who received tailored regimens. 3 , 21 , 22 …”

Long-term outcomes of pediatric meningioma surgery: single center experience with 23 patients

“…In contrast to other childhood central nervous system (CNS) tumors (3,13), prognosis and survival are better for pediatric or adolescent meningiomas (10,15,18). There is paucity of reports on pediatric meningioma cases that report longterm survival data in the magnetic resonance imaging (MRI) era.…”