Background Adamantinomas are rare bone tumors, commonly affecting the tibia. Due to the rare nature of disease, previous studies are small or from multiple centers. The purpose of this study is to investigate outcomes of patients with adamantinoma treated in a single institution. Methods Forty‐six histological confirmed adamantinomas of the extremities were reviewed at our institution between 1939 and 2012. Follow‐up data included clinical and radiographical information focusing on complications, local recurrence, metastasis, and overall survival after the treatment. The mean follow‐up was 16 years (range 2‐42 years). Results The most common location was the tibia (n = 31). Patients commonly presented with pain and swelling. The mean age was 24 years (7‐79 years). Thirty‐seven patients were treated with limb salvage. The 39% of patients required a reoperation. The 10‐year disease specific‐ and recurrence free survival was 92% and 72%, with three patients having a recurrence over 15 years postoperative. Older (> 20 years) patients and males were at increased risk of local recurrence (P < 0.05). Conclusion Treatment of adamantinoma of the long bone consists of limb‐salvage surgery. Male patients should be cautioned on their increased risk of disease recurrence, and advocate for continued surveillance of patients even greater than 15‐years postoperatively due to late tumor recurrence.

Level IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

We report a response to pazopanib in a 69-year-old man with heavily pre-treated metastatic extraosseous Ewing sarcoma in addition to molecular profiling of his tumor. To our knowledge, this case is the earliest to demonstrate activity of an oral multi-targeted kinase inhibitor in Ewing sarcoma. This case provides rationale for adding a Ewing sarcoma arm to SARC024, a phase II study of regorafenib, another multi-targeted kinase inhibitor, in patients with liposarcoma, osteosarcoma and Ewing and Ewing-like sarcomas (NCT02048371). This national multi-institutional study is ongoing.