Introduction and importance: Atypical teratoid/rhabdoid tumor (AT/RT) is a rare and highly aggressive malignancy in children. Management of this aggressive tumor is associated with various diagnostic and therapeutic challenges. Maximum safe resection, followed by radiation therapy and multiagent chemotherapy, is the best course of treatment. This study aims to demonstrate the aggressive progression of the clinical presentation and radiographic features for an AT/RT after surgical resection. Case presentation: We reported a case of a 7-years-old girl with recurrent tumor mass in the left parieto-occipital region after performing craniotomy surgical resection. The tumor mass aggressively grew within a month after surgery. Conclusion: AT/RT is a rare and highly progressive malignancy in the children population. This tumor aggressively grows after the first surgery. The INI-1 gene has been found as a diagnostic tumor marker in AT/RT. The characteristic of AT/RT is an absence of INI-1 staining in tumor cells. The treatment in AT/RT serves as palliative treatment, aiming to improve patient's quality of life. The poor prognosis is associated with MR imaging evidence of disseminated leptomeningeal tumor.

Original languageEnglish
Article number106790
JournalInternational Journal of Surgery Case Reports
Publication statusPublished - Feb 2022


  • Aggressive growth
  • Atypical teratoid/rhabdoid tumor (AT/RT)
  • Child health
  • Malignancy
  • Recurrence


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