Adamantinoma is to be known as the one of the least common low-grade malignant bone tumors, commonly arising in the center of long bones, mostly in the tibial mid shaft. Adamantinomas are usually managed with wide local excision and reconstruction, unless unresectable or locally recurrent. Even though adamantinomas are managed as locally aggressive tumors, they usually recur and metastasize to sites including the lymph nodes, lungs, skeleton, liver and brain. Radiotherapy and chemotherapy do not seem to be effective treatment modalities for adamantinoma; recently published case reports suggest tyrosine kinase inhibitors as an alternative therapeutic strategy. We report a case of a patient with metastatic adamantinoma who progressed after first line therapy with doxorubicin and cisplatin, and responded to pazopanib (selective multi-targeted receptor tyrosine kinase inhibitor that inhibits angiogenesis) and alisertib (small molecule Aurora A kinase inhibitor) combination therapy. Tumor response to pazopanib and alisertib in combination as a third line therapy has led us to conclude that tyrosine kinase therapy could be considered in this setting.

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