Although generalized conclusion cannot be depicted from a single case, these findings suggest that immunotherapy approaches, which include tumor-specific vaccine and antibodies directed to immunological checkpoints, such as ipilimumab (anti-CTLA4) or nivolumab (anti-PD1), may offer, alone or in association with targeted-therapies, a new therapeutic option for advanced CCS patients, for which no successful therapies are currently available. The gene discussed is CTLA4; the disease is neoplasm.