Re-classification for therapeutic application in SMARCB1/INI1 deficient tumors

儿童SMARCB 1/INI 1（INI 1）缺陷型肿瘤可重新分为三组：常规型、非典型畸胎瘤/横纹肌样（AT/R）型和小细胞型。Akt-mTOR通路的激活在AT/R和小细胞型中比常规型更频繁。同时，在大多数的上皮样肉瘤（epithelioid sarcoma，ES）（包括常规型和近端型）中，Akt-mTOR通路被激活，提示抑制mTOR是治疗AT/R和小细胞型儿童INI 1缺陷型肿瘤和ES的潜在治疗选择。ES和恶性横纹肌样瘤被认为是不同的肿瘤实体。

Pediatric SMARCB1/INI1 (INI1)-deficient tumors were able to be re-classified into the three groups: conventional type, atypical teratoid/rhabdoid (AT/R) type and small cell type. Activation in Akt-mTOR pathway was found more frequently in AT/R and small cell types than in conventional type. Meanwhile, in most cases of epithelioid sarcoma (ES) including both conventional and proximal types, the Akt-mTOR pathway was activated.It is suggested that mTOR inhibition is a potential treatment option for AT/R and small cell types of pediatric INI1-deficient tumor and ES. ES and malignant rhabdoid tumor are suggested to be distinct tumor entities.