Abstract
BACKGROUND: Rhabdoid tumours of the central nervous system are highly malignant and extremely rare in adults. To the best of our knowledge, only 87 cases of malignant rhabdoid tumour have been reported to date, inclusive of 4 cases with presumed radiation-induced aetiology. We report a case of malignant rhabdoid tumour in an adult with presumed radiation-induced aetiology to enrich the armamentarium of this disease entity, which may have some implications for early diagnosis and treatment of this rare disease in the future. CASE SUMMARY: A 27-year-old male, who was exposed to cranial irradiation at the age of 4 years as part of the treatment for acute lymphoblastic leukaemia, presented with symptoms of raised intracranial pressure for one week. Brain magnetic resonance imaging revealed a heterogeneously enhancing lesion at the hypothalamus. Stereotactic biopsy was performed. Histopathological examination of the lesion showed malignant rhabdoid tumour. The disease progressed rapidly, with manifestation of leptomeningeal spread. He was started on craniospinal irradiation but treatment was suspended after 5.4 Gy, as he developed myelosuppression. His clinical condition deteriorated rapidly, and he succumbed to his illness within 2 mo. CONCLUSION: This fifth case of radiation-induced central nervous system rhabdoid tumour re-enforces the aggressive nature of this disease with poor prognosis.