Figure 1

SEGA volume reduction during Everolimus treatment. A boy with a TSC2 mutation showed progressively growing SEGAs that caused a first acute hydrocephalus when he was 17 years old. He therefore underwent a partial resection of the lesion, with hydrocephalus resolution. However, two years later, the contralateral SEGAs determined a new episode of hydrocephalus and he underwent surgery once again. However, both lesions presented a slow regrowth after surgeries, and figure (A) shows their aspect when the boy was 24 years of age. He later presented a new episode of subacute hydrocephalus, which required an external derivation. He subsequently started Everolimus treatment, with a partial response (total lesion volume 46% of baseline) of SEGAs after 12 months of treatment (B).