ArticleFrontiers in neurology2017
Atypical Teratoid Rhabdoid Tumor: Two Case Reports and an Analysis of Adult Cases with Implications for Pathophysiology and Treatment.
Article in Frontiers in neurology, 2017. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers, 1 of them a synthesis that pooled it.
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Who cites it
20 citing papers in PubMed, 1 synthesis or guideline pooled it.
- Atypical teratoid/rhabdoid tumor in adults: a systematic review of the literature with meta-analysis and additional reports of 4 cases.Journal of neuro-oncology · 2022Pooled it
- Pediatric Atypical Teratoid Rhabdoid Tumor of Central Nervous System: A Case Series with Review of Literature.Indian journal of surgical oncology · 2025Article
- Autologous stem cell transplantation in adults with atypical teratoid rhabdoid tumor: a case report and review.CNS oncology · 2024Review
- Adult Primary Central Nervous System Atypical Teratoid/Rhabdoid Tumor Metastasizing to the Cervical Lymph Node.Cureus · 2024Article
- Epidemiology, Characteristics, and Prognostic Factors of Primary Atypical Teratoid/Rhabdoid Tumors in the Spinal Canal: A Systematic Review.Neurospine · 2024Article
- A rare case of atypical teratoid rhabdoid tumor at the sellar region in an adult: Case report and review of literature.Brain & spine · 2024Review
- Sellar atypical teratoid/rhabdoid tumor in adults: survival analysis of treatment strategies. Illustrative case.Journal of neurosurgery. Case lessons · 2023Article
- Atypical Teratoid Rhabdoid Tumor: Proposal of a Diagnostic Pathway Based on Clinical Features and Neuroimaging Findings.Diagnostics (Basel, Switzerland) · 2023Review
- Primary adult sellar SMARCB1/INI1-deficient tumor represents a subtype of atypical teratoid/rhabdoid tumor.Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc · 2022Article
- Sellar Atypical Teratoid/Rhabdoid Tumors (AT/RT): A Systematic Review and Case Illustration.Cureus · 2022Review
- Atypical Teratoid Rhabdoid Tumor: A Possible Oriented Female Pathology?Frontiers in oncology · 2022Article
- A 71-year-old man with a rare rhabdoid brain tumour: using a multidisciplinary medical and rehabilitative model of care.BMJ case reports · 2020Article
- Dural-based atypical teratoid/rhabdoid tumor in an adult: DNA methylation profiling as a tool for the diagnosis.CNS oncology · 2020Article
- Adult Sellar Region Atypical Teratoid/Rhabdoid Tumor: A Retrospective Study and Literature Review.Frontiers in neurology · 2020Article
- Fulminant presentation of a SMARCB1-deficient, anterior cranial fossa tumor in adult.Surgical neurology international · 2020Article
- Radiation-induced malignant rhabdoid tumour of the hypothalamus in an adult: A case report.World journal of clinical oncology · 2019Article
- Article
- A Systematic Review of Atypical Teratoid Rhabdoid Tumor in Adults.Frontiers in oncology · 2018Article
- Atypical teratoid rhabdoid tumor in a 65-year-old man presenting with disseminated leptomeningeal disease: A case report and review of the literature.SAGE open medical case reports · 2018Article
- Primary intracerebral INI1-deficient rhabdoid tumor with CD34 immunopositivity in a young adult.Surgical neurology international · 2018Article
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Authors and funding
13 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
We present the first quantitative analysis of atypical teratoid rhabdoid tumors (ATRT) in adults, including two patients from our own institutions. These are of interest as one occurred during pregnancy and one is a long-term survivor. Our review of pathological findings of 50 reported cases of adult ATRT leads us to propose a solely ectodermal origin for the tumor and that epithelial-mesenchymal transition (EMT) is a defining feature. Thus, the term ATRT may be misleading. Our review of clinical findings shows that ATRT tends to originate in mid-line structures adjacent to the CSF, leading to a high rate of leptomeningeal dissemination. Thus, we hypothesize that residual undifferentiated ectoderm in the circumventricular organs, particularly the pituitary and pineal glands, is the most common origin for these tumors. We note that if growth is not arrested soon after diagnosis, or after the first relapse/progression, death is almost universal. While typically rapidly fatal (as in our first case), long-term remission is possible (as in our second). Significant predictors of prognosis were the extent of resection and the use of chemotherapy. Glial differentiation (GFAP staining) was strongly associated with leptomeningeal metastases (chi-squared p = 0.02) and both predicted markedly worse outcomes. Clinical trials including adults are rare. ATRT is primarily a disease of infancy and radiotherapy is generally avoided in those aged less than 3 years old. Treatment options in adults differ from infants in that cranio-spinal irradiation is a viable adjunct to systemic chemotherapy in the adult population. Given the grave prognosis, this combined approach appears reasonable. As effective chemotherapy is likely to cause myelosuppression, we recommend that stem-cell rescue be available locally.
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