Rhabdoid tumors, which are rare in childhood, are aggressive cancers. It can be particularly seen in 3 different anatomical regions, mostly in the central nervous system, kidneys, and soft tissue in early childhood. In this study, it was aimed to evaluate the clinical, radiological and pathological features of pediatric patients with rhabdoid tumors who were followed up and treated in 3 different pediatric oncology reference centers.

Erciyes University Faculty of Medicine, Kahramanmaraş Sütçü İmam University Faculty of Medicine, Health Practice and Research Hospital and Adana City Training and Research Hospital, 17 patients diagnosed with rhabdoid tumor between 2002-2021 were retrospectively analyzed.

Of the patients, 6 (35%) were female and 11 (65%) were male. Chemotherapy (Doxorubicin, Ifosfamide, Carboplatinum, Etoposide, Vincristine, Actinomycin-D, Cyclophosphamide) was administered to the patients at different times. Radiotherapy was applied to 8 (47%) of the patients. The tumor was in the brain in 8 (47%) of the patients, in the kidney in 4 (23%), in the skin in 4 (23%), and the liver in 1 (6%).

In this study, the incidence of rhabdoid tumors was higher in males. This may be due to the small number of cases. The 2 years overall survival rates were 50% in brain tumors, 6% in kidney tumors, and 12% in others, according to tumor localization. The localization and stage of the tumor were determinants of the survival of the patients. More clinical studies are needed to improve survival and identify more effective treatment strategies in these tumors.