Background: Central Nervous System (CNS) rhabdoid tumours are a highly malignant group of neoplasms usually occurring in children under 2 years of age with characteristic histopathologic findings but unclear histiogenesis and almost uniformly fatal outcome. There is still no proven curative therapy available.
Procedure: The clinical course and the successful outcome of therapy in two children with primary CNS rhabdoid tumour are described in this context. Both children had subtotal excision of the primary tumour and received chemotherapy based on the SIOP Malignant Mesenchymal Tumour (MMT-95) protocol with addition of triple intrathecal chemotherapy. Following this, one of the patients received high dose therapy (busulphan and thiotepa), whereas the other had craniospinal radiotherapy with a boost to the primary site.
Results: The treatment was reasonably well tolerated and both patients are alive with no evidence of disease 52 months and 65 months after the primary diagnosis. Their favourable outcomes are compared with those of 49 others reported in the literature.
Conclusions: Intensified therapy (with autologous bone marrow transplantation and intrathecal chemotherapy) may improve the prognosis of patients with malignant rhabdoid tumour.
Copyright 2003 Wiley-Liss, Inc.