Rosa Álvarez, Department of Medical Oncology, Hospital Universitario Gregorio Marañón, Madrid, Spain
Mar Galera López, Department of Medical Oncology, Hospital Universitario Gregorio Marañón, Madrid, Spain
Marta Arregui, Department of Medical Oncology, Hospital Universitario Gregorio Marañón, Madrid, Spain
Ana Álvarez, Department of Radiation Oncology, Hospital Universitario Gregorio Marañón, Madrid, Spain
Raphael A. González Crisostomo, De La Salle Medical and Health Sciences Institute, Damariñas, Cavite
Antonio Calles, Department of Medical Oncology, Hospital Universitario Gregorio Marañón, Madrid, Spain
David S. Juliao Caamaño, Department of Medical Oncology, Hospital Universitario Gregorio Marañón, Madrid, Spain
Natalia Gutiérrez Alonso, Department of Medical Oncology, Hospital Universitario Gregorio Marañón, Madrid, Spain
Enrique de Miguel, Department of Radiology, Hospital Universitario Gregorio Marañón, Madrid, Spain
Carolina Agra, Department of Anatomic Pathology, Hospital Universitario Gregorio Marañón, Madrid, Spain


We present the case of a 34-year-old male patient who was diagnosed with Stage IV desmoplastic small round cell tumor (DSRCT), an aggressive disease with a poor prognosis. The patient received multiple lines of treatment achieving long-term survival with a prolonged response to trabectedin (T) for more than 1 year.