Primary malignant rhabdoid tumors of the central nervous system: Clinicoradiological study of three cases and their outcome

Abstract

Malignant rhabdoid tumors of the brain are extremely malignant, highly aggressive neoplasms found in infants and young children. These are rare and not associated with renal neoplasm as believed earlier. We report three cases of malignant rhabdoid tumors with reference to their clinicoradiological aspect and fatal outcome despite a multimodality approach of management (aggressive surgical resection and chemoradiotherapy). The first case was a two and one half-year-old girl who presented with seizures, raised intracranial pressure and right hemiparesis. Contrast magnetic resonance imaging showed a huge heterointense tumor of the left hemisphere, which was a soft, suckable, and very vascular, infiltrative mass with areas of necrosis. Postoperatively, the girl had a difficult course with multiple metastases detected on the 45th day and death on the 52nd day post diagnosis. The second patient was admitted with large and infiltrative posterior fossa mass that required ventilatory support in postoperative period for 3 weeks until his death. The third patient was operated for a large, vascular and friable mass of the temporal lobe, which recurred in 5 weeks. He was offered chemoradiotherapy but the length of his life was only 8 months.