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Molecularly guided precision therapy in metastatic adamantinoma: a case report
Aram A Musaelyan1,2, Svetlana V Odintsova1,2, Alexander O Ivantsov3
1Department of Oncology, Pavlov First St. Petersburg State Medical University, Saint Petersburg, Russia.
Abstract:
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.
Insights
A rare adamantinoma case showed success with targeted therapies. A PARP inhibitor and immunotherapy combination extended survival, offering new hope for metastatic bone tumors.
Area of Science:
- Oncology
- Genetics
- Pharmacology
Background:
- Adamantinoma is a rare bone tumor with poor prognosis in metastatic stages.
- Current treatments for metastatic adamantinoma lack standardization and show limited efficacy.
- Genetic mutations can influence treatment response in rare cancers.
Purpose of the Study:
- To report a case of metastatic adamantinoma with an XRCC2 mutation.
- To evaluate the efficacy of molecularly guided therapies in this patient.
- To highlight potential treatment strategies for refractory adamantinoma.
Main Methods:
- Case report of a young woman with metastatic adamantinoma.
- Genomic analysis revealing an XRCC2 frameshift mutation.
- Treatment with PARP inhibitor (olaparib), followed by atezolizumab and bevacizumab combination therapy.
Main Results:
- Sustained 15-month response to olaparib, improving performance status.
- Eight-month stable disease with atezolizumab and bevacizumab combination.
- Overall survival of 25 months achieved through molecularly guided treatment.
Conclusions:
- PARP inhibitors may be effective in adamantinoma with XRCC2 mutations.
- Combination immunotherapy and anti-angiogenic therapy can manage disease progression.
- Molecular profiling is crucial for guiding treatment in rare bone tumors like adamantinoma.
