Molecularly guided precision therapy in metastatic adamantinoma: a case report

Adamantinoma is a rare bone tumor characterized by a propensity for local recurrence and distant metastasis. Management of metastatic disease remains challenging owing to the absence of standardized therapy and the limited efficacy of conventional chemotherapy. This case report describes a young woman with adamantinoma harboring an XRCC2 frameshift mutation. She showed a sustained clinical response to fourth-line treatment with the PARP inhibitor olaparib, which lasted 15 months and led to improved performance status. Subsequent progression led to combination therapy with atezolizumab and bevacizumab, maintaining stable disease for eight months. Molecularly guided treatment resulted in an overall survival of 25 months.