[Rhabdoid tumours of the central nervous system: five case studies]. / Les tumeurs rhabdoïdes du système nerveux central: à propos de cinq observations.

ABSTRACT

PURPOSE: The rhabdoid cerebral tumors were first identified by Briner et al. in 1985. Their frequency was estimated to be 2.1% of those affecting children under 18 months. Such tumors are equally characterized by a critically and speedly mortal development. Their historical genesis is still of a controversial issue. The aim of the present study was to discuss the various anatomicoclinical and therapeutic aspects of these rare tumors. PATIENTS AND METHODS: We report five cases diagnosed over a period of eight years (1997-2004) in the Laboratory of Anatomy and Pathological Cytology of the University Hospital of Sfax. RESULTS: The average age of patients was 20 years old, there were children under 14 years and 4 patients were male. Clinical symptomatology showed the predominance of intra-cranial hypertension syndrome. Radiography through magnetic resonance revealed a heterogeneous tumor process localized respectively on the spine (one case), the insula (one case), the temporofrontal lobes (two cases) and the medulla (one case). Histological examination of the tumors also showed a proliferation of giant cells with a hyaline-based cytoplasmic inclusion. These inclusions were positive for vimentin and keratin. An adjuvant radiotherapy treatment was prescribed for two patients. A recurrence of rhabdoid tumors occurred in two cases. All five patients died within eighteen months. CONCLUSION: The cerebral rhabdoid malignant tumor constitutes one of the most aggressive and life-threatening intracranial tumors. The optimal management of such tumors remains unknown.