Background: Spindle epithelial tumor with thymus-like differentiation (SETTLE) is a very rare tumor that occurs mainly in pediatric patients and young adults. Only few of these patients develops metastatic disease; therefore, clinical data regarding treatment and outcome of metastatic SETTLE are extremely limited. Several chemotherapy agents have been used in SETTLE but due to the limited number of patients no evidence-based therapy exists. Case Report: We present a case of metastatic SETTLE presenting with high tumor burden and paraneoplastic hypercalcemia. Prolonged disease control with several lines of platinum-based chemotherapy, anti-epidermal growth factor receptor therapy and additional radiotherapy was achieved. Conclusion: Multi-agent chemotherapy is an active treatment in metastatic SETTLE and can induce sustained tumor control.

Keywords:
Spindle epithelial tumor with thymus-like differentiation, SETTLE, Chemotherapy, Hypercalcemia
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2018
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