A Systematic Review of Atypical Teratoid Rhabdoid Tumor in Adults

Abstract

Background: Atypical teratoid/rhabdoid tumor in adults is a relatively rare malignant neoplasm. It is characterized by the presence of rhabdoid cells in combination with loss of either the INI1 or BRG1protein from the tumor cells.Methods: A systematic review was conducted using MEDLINE using the terms “atypical teratoid rhabdoid tumor” AND “adult.” The systematic review was supplemented with relevant articles from the references. Cases were included if the pathology was confirmed by loss of INI1 or BRG1. We included a case from our institution. The dataset was analyzed using descriptive statistics and log-rank test.Results: A total of 50 cases from 29 articles were included in this study. The average age at diagnosis was 36.7 years. The most common locations reported are the sellar region and cerebral hemispheres (without deep gray matter involvement). Of the 50 cases, 14 were reported to show evidence of dissemination. The average overall survival was 20 months. There was a significant difference in survival between the adjuvant therapy groups (p = < 0.0001).Conclusion: Atypical teratoid rhabdoid tumor of the central nervous system in adults is a rare neoplasm associated with a poor prognosis in a majority of patients. The treatment and clinical course are highly variable, and it remains unclear which factors impact prognosis.

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

“…ATRT is a highly malignant neoplasm associated with a poor prognosis. It is primarily found in infancy; however, it has been diagnosed both antenatally and in adults [1,10,17]. Due to the lack of large prospective studies regarding ATRT, there is still uncertainty regarding the optimal treatment for these children and therefore, management strategies vary.…”

“…Germline mutations of SMARCB1/SMARCA4 have also been associated with poorer survival [10,13,27,28], while newer evidence purports an association with molecular subgrouping [17,31]. Germline mutations of SMARCB1 appear to predispose patients to synchronous rhabdoid tumors within and out-with the CNS [3,4].…”

Atypical Teratoid/Rhabdoid Tumor of the Sellar Region: A Case Report and Review of the Literature

“…The prognosis of AT/RT is poor both in adults and in the pediatric population. Chan et al reported the average survival rate of adult AT/RT was 20 months [8]. This is comparable to the reported median survival of 13.5 to 16.8 months in the pediatric population [10,25].…”

“…Ostrom et al reported that the most common location in adults is the cerebral hemisphere, followed by the sellar region [7]. In contrast, Chan et al found that the most common location was the sellar region (46%), followed by cerebral hemisphere (32%) [8]. Sellar AT/RTs were reported to be almost exclusively in females and not in the pediatric population [9,10].…”

Intraventricular atypical teratoid rhabdoid tumour in an adult: a case report and literature review

“…Atypical teratoid/rhabdoid tumour (AT/RT) is a malignant neoplasm representing less than 5% of central nervous system tumours [7,11]. Although this tumour is typically seen in the pediatric population, cases of AT/RT have been reported in adults [2]. The most common locations in adults are the cerebral hemisphere and the sellar region [2,18].…”