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Atypical teratoid/rhabdoid tumor with 26-year overall survival: case report
Junko Takahashi-Fujigasaki1, Masayoshi Matumoto, Issei Kan
1Division of Neuropathology, The Jikei University School of Medicine, Tokyo, Japan.
Insights
A rare case of atypical teratoid/rhabdoid tumor (AT/RT) in an infant survived for 26 years, highlighting potential for long-term outcomes. This AT/RT case emphasizes careful consideration of early radiation therapy benefits versus toxicity in young children.
Area of Science:
- Pediatric Oncology
- Neuro-oncology
- Developmental Biology
Background:
- Atypical teratoid/rhabdoid tumor (AT/RT) is a highly aggressive embryonic brain tumor primarily affecting infants and young children.
- The prognosis for AT/RT, particularly in patients under three years old, remains very poor despite intensive treatment protocols.
Observation:
- An infant diagnosed with a fourth ventricle AT/RT at 6 months underwent resection and radiation therapy.
- The patient experienced a 26-year disease-free interval before developing a spinal cord tumor.
- The recurrent tumor was a collision neoplasm comprising benign schwannoma and recurrent AT/RT.
Findings:
- This case represents the longest reported survival for a patient with infantile-onset AT/RT, exceeding 20 years.
- The patient succumbed to tumor dissemination 5 months post-excision of the recurrent spinal cord tumor.
Implications:
- Long-term survival is achievable in infantile-onset AT/RT, challenging the historically dismal prognosis.
- The therapeutic value of early, upfront radiation therapy for AT/RT warrants careful evaluation, balancing potential benefits against significant toxicity in very young children.
Abstract:
Atypical teratoid/rhabdoid tumor (AT/RT) is an aggressive embryonic brain tumor predominantly seen in young children. The authors report an unusual case of a fourth ventricle AT/RT in an infant who survived for 26 years. The tumor was resected when the patient was 6 months of age, and radiation therapy (40-Gy total dose) was performed thereafter. The patient was free from the disease for 26 years until a recurrent tumor was found in the spinal cord. The spinal cord neoplasm was a "collision tumor" with 2 components: benign schwannoma and recurrent AT/RT. The patient died of dissemination of the recurrent tumor 5 months after it was excised. This is the longest survival of a patient with AT/RT ever reported and indicates that long-term survival, more than 20 years, can be achieved in infantile-onset AT/RT. Despite intensive treatment, the prognosis for AT/RT is very poor, especially in children younger than 3 years of age. The benefits of upfront radiation therapy for AT/RT should be carefully assessed with respect to its inevitable toxicity in very young children. However, early upfront radiation therapy may be of therapeutic interest to prevent aggressive progression of the disease.
