Paediatric rhabdoid meningioma: clinical and therapeutic features findings: case series of 3 patients.

INTRODUCTION: Pediatric Rhabdoid Meningioma (RM) are the rarest, but most aggressive subtypes of meningioma, related to a severe prognosis. They account for 1-3% of all intracranial meningiomas.

CASE PRESENTATIONS: We report an institutional experience of 3 cases through which we discuss clinical, histological and therapeutic features of this tumour. Two of our patients were female-gendered (3 years old and 1 year and 6 months old), and one male-gendred (16 years old). Revealing symptoms were related to intracranial hypertension, cerebellar syndrome, cranial nerves palsy and skull tumefaction. Imaging showed extra-axial tumor located in the right Ponto-cerebellar angle in the first case, in the left occipital region in the second case, left parietal tumor in the third case. All patients underwent a surgical intervention with a gross total resection. Histological evaluation supported by immune-histochemistry confirmed the diagnosis of RM. Tumor recurrence was observed at 45 days in the first case with a fatal outcome. Despite adjuvant radiotherapy, both second and third cases had local recurrence after a mean follow up of 1 month following the radiotherapy.

CONCLUSIONS: RM are very aggressive tumours. Standardized therapeutic guidelines are still under debate as actual approaches are still inefficient to prevent quick recurrence and fatal outcome.