Based on previous studies, in many human tumors including tuberous sclerosis complex (TSC) and lymphangioleiomyomatosis (LAM), mTORC1‐hyperactivity upregulates B7‐H3/CD276 to inhibit antitumor T cells and drive tumor immune evasion, while mTORC1 inhibitors could display strong antitumor effects and increase antitumor immunity.[28] In line with this, our results showed that the chordoma case with aberrant RAB3B/p‐S6 hyperactivation exhibited very low expression of CD4+ and CD8+ T cells and mTORC1 inhibition benefited this patient. Here, CD276 is linked to chordoma.