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Continuous remission in an infant with chest wall malignant rhabdoid tumor after relapse
Hajime Hosoi1, T Iehara, K Tsuchiya
1Department of Pediatrics, Kyoto Prefectural University of Medicine, Kawaramachi-Hirokoji, Kamigyo-ku, Kyoto 602-8566, Japan. hhosai@koto.kpu-m.ac.jp
Insights
Long-term survival was achieved in a pediatric patient with malignant rhabdoid tumor (MRT) through aggressive treatment. This included complete surgical resection, intensive chemotherapy, and radiotherapy, establishing a strategy for this aggressive childhood cancer.
Area of Science:
- Pediatric Oncology
- Cancer Biology
Background:
- Malignant rhabdoid tumor (MRT) is a rare and aggressive pediatric cancer with a poor prognosis, particularly in infants.
- Early diagnosis and effective treatment strategies are crucial for improving outcomes in children with MRT.
Observation:
- A 5-month-old boy diagnosed with chest wall MRT experienced a local recurrence after initial treatment.
- The patient underwent a second complete tumor resection followed by chemotherapy and local radiotherapy.
Findings:
- The patient achieved a second complete remission and has remained disease-free for 5 years.
- The child is now 7.5 years old and in good health, demonstrating long-term survival post-treatment.
Implications:
- This case highlights the importance of complete surgical resection for localized MRT.
- Intensive multiagent chemotherapy and radiotherapy are vital for managing minimal disseminated disease and local control.
- A multimodal treatment approach combining surgery, chemotherapy, and radiotherapy can lead to successful long-term outcomes for pediatric MRT.
Abstract:
Malignant rhabdoid tumor (MRT) is a highly aggressive tumor that occurs in infancy or childhood. The prognosis, especially in infants, is very poor. Here we report the long-term survival of a 5-month-old boy with MRT that arose from the chest wall. After total resection of the tumor, the patient was given 4 cycles of doxorubicin, vincristine, and cyclophosphamide, alternating with ifosfamide and etoposide. After 18 months off therapy, he had a local recurrence at the same site. After a second total resection, he was given additional chemotherapy with 30.6-Gy local irradiation. No further recurrence has been observed for 5 years since the second complete remission. Currently, he is alive and well at 7.5 years post-onset. Our experience in this case suggests a fundamental strategy of successful treatment of this highly malignant pediatric tumor: (1) complete resection of the localized tumor, (2) intensive multiagent chemotherapy for the minimal disseminated disease, and (3) radiotherapy for local control of the disease.
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