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Updated: Feb 2, 2026

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Spontaneous Regression of Atypical Teratoid Rhabdoid Tumor Without Therapy in a Patient With Uncommon Regional

Jo Elle G Peterson1, Abhishek Bavle2, Vidya P Mehta3

  • 11 Department of Pathology, College of Medicine, University of Oklahoma, Oklahoma City, Oklahoma.

Pediatric and Developmental Pathology : the Official Journal of the Society for Pediatric Pathology and the Paediatric Pathology Society
|November 25, 2018
PubMed

Insights

A rare congenital atypical teratoid/rhabdoid tumor (ATRT) spontaneously regressed in a patient. Molecular analysis revealed distinct INI1-intact and INI1-deficient cell populations, offering insights into ATRT behavior.

Area of Science:

  • Pediatric Oncology
  • Neuro-oncology
  • Cancer Genetics

Background:

  • Atypical teratoid/rhabdoid tumor (ATRT) is an aggressive pediatric brain tumor with a poor prognosis.
  • INI1 (SMARCB1) gene inactivation is a hallmark of ATRT, leading to loss of nuclear INI1 protein.
  • Current treatments offer limited success, highlighting the need for understanding ATRT biology.

Observation:

  • A case of congenital ATRT in a patient who experienced spontaneous tumor regression without any therapy.
  • Histopathology confirmed rhabdoid cells typical of ATRT, with heterogeneous INI1 nuclear staining.

Findings:

  • Laser microdissection separated INI1-intact and INI1-deficient tumor cells for molecular analysis.
  • INI1-negative cells harbored a deletion and mutation in the hSNF5/INI1 gene, while INI1-intact cells had wild-type INI1.
  • This demonstrates molecular heterogeneity for SMARCB1 inactivation within the tumor.

Implications:

  • This is the first reported case of spontaneous ATRT regression.
  • The findings suggest potential therapeutic vulnerabilities in ATRT related to SMARCB1 status.
  • Long-term disease-free survival without recurrence was observed, offering hope for future ATRT management.
Keywords:
INI1atypical teratoid rhabdoid tumorfocal inactivationspontaneous regression

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