Published online Nov 24, 2019. doi: 10.5306/wjco.v10.i11.375
Peer-review started: May 23, 2019
First decision: August 2, 2019
Revised: October 10, 2019
Accepted: October 15, 2019
Article in press: October 15, 2019
Published online: November 24, 2019
Processing time: 187 Days and 11.8 Hours
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.
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.
This fifth case of radiation-induced central nervous system rhabdoid tumour re-enforces the aggressive nature of this disease with poor prognosis.
Core tip: Malignant rhabdoid tumours of the central nervous system are highly malignant, with a grave prognosis. Occurrence in adults is extremely rare. Due to the paucity of available data, treatment recommendations for adults are derived mainly from paediatric data. Despite being a dismal disease, prognosis in children has been more encouraging recently due to the continuous refinement of treatment strategies. We report a case of malignant rhabdoid tumour in an adult with presumed radiation-induced aetiology, with the hope to enrich the armamentarium of this disease entity and provide some implications for early diagnosis and treatment of this disease in the future.