# Navigating the complexity of atypical teratoid/rhabdoid tumor (ATRT) in pediatric neuro-oncology: Insights from clinical spectrum to therapeutic challenges

## TL;DR

This paper presents a case of a rare and aggressive pediatric brain tumor, showing that early diagnosis and aggressive treatment can lead to long-term remission.

## Contribution

The paper contributes a detailed case study demonstrating the effectiveness of multimodal therapy in managing atypical teratoid/rhabdoid tumor (ATRT).

## Key findings

- Early diagnosis and aggressive multimodal therapy led to no recurrence in a 2-year-old ATRT patient.
- Maximal safe resection followed by chemotherapy is critical for improving long-term outcomes in ATRT.
- MRI features like heterogeneous enhancement and solid-cystic lesions are typical for ATRT.

## Abstract

Atypical teratoid/rhabdoid tumor (ATRT) is a rare and aggressive pediatric central nervous system malignancy, accounting for only 1–2 % of cases. Primarily affecting children under three years old, ATRT poses significant diagnostic and therapeutic challenges, with high recurrence rates and poor prognosis due to its rapid progression and lack of a standardized treatment protocol.

We report the case of a 2-year-old male diagnosed with infratentorial ATRT after presenting with abnormal gait, vomiting, and ataxia following minor head trauma. Magnetic resonance imaging (MRI) revealed a mixed solid-cystic cerebellar lesion, prompting surgical resection. Despite postoperative chemotherapy, tumor progression was noted, leading to a second craniotomy, which achieved complete resection. Serial follow-up MRI until February 2025 showed no evidence of recurrence, and the patient remains symptom-free four years post-treatment.

This case underscores the complexities of ATRT management, emphasizing the importance of early diagnosis, aggressive multimodal therapy, and vigilant radiological follow-up. While ATRT shares imaging similarities with medulloblastomas, its distinct histopathological features necessitate tailored treatment strategies. Maximal safe resection, followed by intensive chemotherapy, remains the cornerstone of treatment, highlighting the need for a multidisciplinary approach to improve long-term outcomes in pediatric ATRT patients.

•ATRT is highly malignant in kids <3 yrs, needing early, intensive treatment for better outcomes.•Multimodal therapy (surgery + chemo) is vital, as shown in studies and our case.•MRI shows typical ATRT signs: heterogeneous enhancement and solid-cystic lesions.•Early aggressive treatment led to no recurrence and symptom-free status up to 2025.•This case supports early diagnosis and multimodal therapy for improved prognosis.

ATRT is highly malignant in kids <3 yrs, needing early, intensive treatment for better outcomes.

Multimodal therapy (surgery + chemo) is vital, as shown in studies and our case.

MRI shows typical ATRT signs: heterogeneous enhancement and solid-cystic lesions.

Early aggressive treatment led to no recurrence and symptom-free status up to 2025.

This case supports early diagnosis and multimodal therapy for improved prognosis.