Page 27 - TSC related Angiomyolipoma - Management Strategies and Case Studies
P. 27

TSC Affects Multiple Organ Systems                                                               1,2





            Neurologic

               • Epilepsy                             90%

               • Infantile seizures  20-30%
                                                                                                                                 A large
                                                                                                                             SEGA along

               • Brain tumors                                                                                                 the midline

                 - Cortical tubers                    90 %                                                                    of the brain
                                                                                                                                near the
                 - SENs                               95%                                                                     foramen of


                 - SEGAs                              6-19%                                                                      Monro



                                                                              SEGAs are well circumscribed, slow-

                                                                              growing, low-grade tumors                  3


                                                                              Can obstruct the CSF circulation,
                                                                              leading to hydrocephalus


                                                                              Current intervention options:

                                                                                  • Surgical resection, if feasible
                                                                                  • Shunt implantation

  SEGA, subependymal giant-cell astrocytoma; SEN, subependymal nodule

  1. Crino. N Engl J Med 2006;355:1345-1356; 2. Leung. J Pediatr Health Care. 2007; 21:108-114; 3.   Buccoliero, et al. Neuropathology 2009;29:25-30.
   22   23   24   25   26   27   28   29   30   31   32