Özet:

Background/Aims: Pediatric Rhabdoid tumors are highly aggressive tumor with poor prognosis. There are no consensus about standart treatments currently. It was aimed to evaluate clinical characteristics and outcomes of pediatric rhabdoid tumors. Material and Method: Eight malignant rhabdoid tumor were evaluated retrospectively from data set between 2013 to 2020. Results: Of 8, 5 patients were male (Male/female: 5/3). The median age was 24 months, 6 patients (70%) were below 3 years (4 months-10 years). Tumor localizations were heterogeneous: (5 central nervous system, 1 multifocal, 1 kidney, and 1 bladder). Genetic analysis revealed germline heterozygous SMARCB1 mutation in one (12%) patient. Patients experienced various toxicities such as Wernicke's-like encephalopathy, vincristine neuropathy, veno-occlusive disease mainly haematological toxicity/mucositis and febrile neutropenia. Five patients died due to progressive disease (62%). The median follow-up time of all patients was 24.5 months (ranges 6-41 months). The 2-year- event-free and overall survival rates are 37.5% and 50%, respectively. Conclusion: It should keep in mind pediatric rhabdoid tumors might present with various ages and localisations, but mainly under 3 years old and central nervous system. The experience is limited due to rarity, but addition of high-dose chemotherapy with autologous hematopoietic stem cell transplantation could be efficious in subset of patients who achieved complete remission before transplantation. The toxicities resulted from intensive treatments could be managable yet new targeted therapies are required to improve survival rates.

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