Abstract
Высокодозная полихимиотерапия с аутологичной трансплантацией гемопоэтических стволовых клеток у детей с атипичной тератоид-рабдоидной опухолью центральной нервной системы
Highlights
Central nervous system (CNS) atypical teratoid rhabdoid tumor (ATRT) is a rare aggressive malignancy, which comprises 1-2% of brain and spinal neoplasms [1, 2]
Among children of ATRT-SHH molecular subgroup 2 died of progression and 2 were alive and in remission
CNS ATRT is a rare malignancy with poor prognosis that is predominantly diagnosed in young children [3, 4]
Summary
Central nervous system (CNS) atypical teratoid rhabdoid tumor (ATRT) is a rare aggressive malignancy, which comprises 1-2% of brain and spinal neoplasms [1, 2]. There are no established standards in the treatment of CNS ATRT. Various management approaches exist for different countries and institutions. Despite this uncertainty, surgery followed by adjuvant chemotherapy and radiotherapy (in older children) is, without doubt, the cornerstone of their treatment [7, 8]. HDCT/auto-HSCT is used in children under 3 years, intending for canceling or postponing radiation therapy (RT) and reduce the risk of longterm neuro-cognitive disorders and improve outcome [9, 10, 11]. Prognosis for CNS ATRT remains dismal, especially in children with residual tumor and metastatic disease
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