title: Metastatic extraskeletal Ewing's sarcoma treated with trabectedin: A case report.
abstract: The Ewing's sarcoma family of tumors (ESFT) comprises a number of rare malignant tumors. Standard first-line treatment for patients with these tumors includes chemotherapy with a five-drug regimen of vincristine, doxorubicin (Adriamycin®) and cyclophosphamide, alternating with ifosfamide and etoposide (VAC/IE). In cases of inadequate response, there are a number of second-line regimens available. However, further treatment options are required for those patients with disease unresponsive to standard treatment. Trabectedin is a novel treatment option for patients with ESFT. The present study reports the case of a Caucasian 69-year-old female patient who presented with a soft tissue mass on the chest wall that had developed 7 months earlier. A computed tomography scan revealed a 9×8×7-cm mass on the anterior chest wall above the pectoral muscle. Histopathological evaluations and molecular analysis indicated that it was consistent with a metastatic extraskeletal Ewing's sarcoma. The patient was treated with an alternating VAC/IE regimen; however, an inadequate response was observed. The patient received second-line treatment with a gemcitabine and dacarbazine combination regimen, but the disease progressed. Subsequently, treatment with trabectedin (1.5 mg/m2as a 24-h continuous infusion every 3 weeks) was initiated. Trabectedin treatment resulted in long-lasting (18 months) progression-free survival. It is vital that novel drugs continue to being developed for patients with ESFT following progression subsequent to standard chemotherapy. The current report presents a case of a patient with metastatic, pre-treated Ewing's sarcoma achieving disease stabilization with trabectedin. Based on these results and the observed tolerability profile, trabectedin represents an alternative treatment for patients with ESFT. Further studies are required in order to determine the efficacy of trabectedin as monotherapy or in combination with other drugs. It is also important to identify which tumor subtypes, specific translocations and patient profiles will benefit the most from treatment with trabectedin.
authors.affiliation: Department of Medical Oncology and Pathology, University Hospital Miguel Servet, 50008 Zaragoza, Spain.
authors.forename: Jorge
authors.initials: J
authors.lastname: Hernando-Cubero
authors.affiliation: Department of Medical Oncology and Pathology, University Hospital Miguel Servet, 50008 Zaragoza, Spain.
authors.forename: Pilar
authors.initials: P
authors.lastname: Sanz-Moncasi
authors.affiliation: Department of Medical Oncology and Pathology, University Hospital Miguel Servet, 50008 Zaragoza, Spain.
authors.forename: Alba
authors.initials: A
authors.lastname: Hernández-García
authors.affiliation: Department of Medical Oncology and Pathology, University Hospital Miguel Servet, 50008 Zaragoza, Spain.
authors.forename: Isabel
authors.initials: I
authors.lastname: Pajares-Bernard
authors.affiliation: Department of Medical Oncology and Pathology, University Hospital Miguel Servet, 50008 Zaragoza, Spain.
authors.forename: Javier
authors.initials: J
authors.lastname: Martínez-Trufero
keywords: Ewing's sarcoma; extraskeletal metastases; metastatic sarcoma; palliative chemotherapy; trabectedin