Parietotemporal atypical teratoid/rhabdoid tumor (ATRT) in a 5 years old male: A Case Report

Abstract Background Atypical teratoid/rhabdoid tumor (AT/RT) is a rare and highly aggressive embryonal tumor of the central nervous system (CNS), primarily affecting children under three years of age. Characterized by rapid growth and dissemination, AT/RT has a poor prognosis, with median survival times often measured in months. Recent advances in treatment, including autologous stem cell transplantation (ASCT), aim to improve outcomes but lack a definitive protocol. Case Presentation : We report a case of a 5-year-old male presenting with acute left-sided weakness, right-sided mouth deviation, and confusion. Initial imaging at another facility revealed a right parietotemporal intra-axial lesion with significant edema and midline shift. Upon transfer to our institution, Magnetic resonance imaging (MRI) confirmed a large supratentorial mass involving critical structures without invasion of major arteries. Surgical intervention was necessary, and a right pterional craniotomy was performed for maximal safe resection. During surgery, the tumor was identified as grayish, highly vascularized, and necrotic. Successful debulking was achieved, with careful preservation of surrounding neural structures. Postoperatively, the patient demonstrated significant neurological improvement, with a Glasgow Coma Scale (GCS) score of 15/15. Histopathological examination confirmed the diagnosis of AT/RT, WHO grade 4, characterized by malignant blue cells and loss of INI1 immunoreactivity. Conclusion This case underscores the importance of early diagnosis and aggressive intervention in managing AT/RT. The complexities of its presentation and imaging necessitate a multidisciplinary approach, integrating neurosurgery and oncology. Our findings support the use of surgical resection, contemporary chemotherapy, and ASCT as part of a multimodal treatment strategy to enhance survival outcomes. However, the inherent aggressiveness of AT/RT highlights the urgent need for innovative treatments and further research to improve prognoses for affected patients.