Alternating chemotherapy with VDC-IE as effective first-line treatment in a patient with SMARCA4-deficient undifferentiated tumor

Abstract

SummarySMARCA4-deficient undifferentiated tumor (SMARCA4-UTs) is an extremely rare and aggressive entity where no consensus on systemic treatment exists to date. We report the case of a 43-year-old woman with thoracic SMARCA4-UT who presented with rapid progression of disease after surgical resection and achieved complete radiologic remission under VDC-IE chemotherapy (vincristine, doxorubicin, and cyclophosphamide [VDC], alternating with ifosfamide and etoposide [IE]). The detailed case report is followed by a brief discussion and overview of current literature.