Pediatric suprasellar atypical teratoid rhabdoid tumor arising from the third ventricle: A rare tumor at a very rare location

Atypical teratoid rhabdoid tumor (ATRT) is a rare, highly malignant tumor of the central nervous system, commonly affecting children below 3 years of age, with around 300 cases reported in the literature. Suprasellar area is a very rare location for such tumor in the pediatric population, with technical difficulties in complete excision. Third ventricular ATRT is very rare. Here, we report the case of a 2-year-old male child who presented with lethargy and vomiting. He had features of raised intracranial pressure with reduced vision in both eyes. Magnetic resonance imaging of the brain revealed a heterogeneously enhancing lobulated giant lesion in the suprasellar location, occupying the third ventricle and hypothalamus with encasement of both carotids. He underwent pericoronal parasagittal craniotomy, interhemispheric transcallosal interforniceal approach and gross total excision of the lesion. Postoperatively, the child had altered sensorium and diabetes insipidus, both of which recovered over a span of 10 days. Histopathological examination of the specimen was consistent with the diagnosis of World Health Organization Grade IV ATRT. In spite of all our efforts, he succumbed to his illness 5 months postoperatively.

Key-words:

Atypical teratoid rhabdoid tumor - diabetes insipidus - pediatric - suprasellar - third ventricle

Publication History

Article published online:
14 September 2022