Treatment of Metastatic Spindle Epithelial Tumor with Thymus-Like Differentiation (SETTLE) - Long-Term Disease Control by Multimodal Therapy

Abstract

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: Chemotherapy; Hypercalcemia; SETTLE; Spindle epithelial tumor with thymus-like differentiation.