Primary malignant rhabdoid tumours of brain, clinicoradiological findings of two cases.

ABSTRACT

Malignant rhabdoid tumours (MRT) are extremely malignant, highly aggressive and uncommon renal neoplasms of childhood with very poor prognosis. About fifteen cases of primary intracranial MRT (with their clinical details) are reported in English literature, following the recognition of this entity in 1978. Two cases of MRT are reported here. The first case, one year male baby was admitted with a very large, infiltrative, posterior fossa mass. He required elective ventilation, following the tumour decompression but ultimately died of respiratory failure during the process of weaning from the ventilator. The second child was operated for an extremely vascular, very friable, solid and lobulated tumour of temporal lobe. Radical microsurgical decompression of mass was achieved, however the child developed massive recurrence, documented five weeks after the surgery while on radiotherapy. His recurrence showed partial response to radiotherapy and chemotherapy. The child is alive at 8 month's follow up, but probably passing the terminal days of his life. Hence the recognition of this entity is very essential for the aggressive management and prognostication of the patient, which obviously seems to be different from primitive neuroectodermal tumour.