Thelansis

  Atypical teratoid/rhabdoid tumor (ATRT) is a rare and aggressive type of embryonal tumor of the central nervous system (CNS) occurring in childhood, primarily occurring at fewer than three years. ATRTs are associated with an inferior prognosis, with a reported median survival of 6–12months in most pediatric cases. ATRTs demonstrate pathological findings typical of primitive neuroectodermal tumors (PNET), featuring necrosis with a high rate of mitotic activity and rhabdoid cells. Most AT-RTs also show a loss of INI-1 nuclear staining; ATRTs can form anywhere in the CNS spread to the spinal cord. ATRTs can start outside the CNS, such as in the kidney. ATRTs develop from several different very young cells called embryonal cells. These cells include rhabdoid, neuroepithelial, epithelial, and mesenchymal. Due to the infrequent incidence of this tumor in adult populations, diagnosis is often delayed. It falls much lower on the differential diagnosis than other more common tumors. ...