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Rhabdoid tumours (RTs) are highly aggressive tumours of infancy, frequently localized in the central nervous system (CNS) where they are termed atypical teratoid/rhabdoid tumours (AT/RTs) and characterized by bi-allelic inactivation of the SMARCB1 tumour suppressor gene. In this study, by temporal control of tamoxifen injection in Smarcb1(flox/flox);Rosa26-Cre(ERT2) mice, we explore the phenotypes associated with Smarcb1 inactivation at different developmental stages. Injection before E6, at birth or at 2 months of age recapitulates previously described phenotypes including embryonic lethality, hepatic toxicity or development of T-cell lymphomas, respectively. Injection between E6 and E10 leads to high penetrance tumours, mainly intra-cranial, with short delays (median: 3 months). These tumours demonstrate anatomical, morphological and gene expression profiles consistent with those of human AT/RTs. Moreover, intra- and inter-species comparisons of tumours reveal that human and mouse RTs can be split into different entities that may underline the variety of RT cells of origin.

Purpose: To describe a risk of malignant neoplasm in patients with Behçet's disease treated with ciclosporin., Methods: Case report., Results: A patient with Behçet's disease who was treated with ciclosporin developed a mass in his right femur. Histological examination revealed this mass was a malignant rhabdoid tumor., Conclusion: This is the first known case of malignant tumor developing in a patient with Behçet's disease treated with ciclosporin. This case underscores the importance of assessing possible risks in the treatment of Behçet's disease with ciclosporin.