Teratoid rhabdoid tumors are highly malignant, rare and aggressive. The prognosis is very poor, with a pejorative and rapidly lethal evolution. The objective of this study was to show diagnostic and therapeutic approach through the report of four observations of rhabdoid tumor of the kidney in children, treated in the oncology unit at the pediatric department CHU Hassan II Fez Morocco, collected over a period of 10 years. The ages of the patients varied from 8 months and 5 and a half, with 3 girls and a boy. All children have abdominal distention with the discovery of a mass on clinical examination. The patients were treated as nephroblastoma by neoadjuvant chemotherapy followed by enlarged total nephrectomy. The pathological study confirmed the diagnosis of a teratoid rhabdoid tumor. Adjuvant chemotherapy was given in all four children combined with radiotherapy in three cases. The evolution was fatal in three children. Malignant rhabdoid tumors are a particular pathological entity requiring a well codified therapeutic protocol to improve survival which does not exceed 15% to 20%.
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