Summary: Ewing sarcoma (ES) is the second most common type of primary bone malignancy, and retains a high propensity to metastasize; the prognosis of patients with disseminated disease is very poor, with an event-free survival rate of <20%. Current multimodality treatment for ES consists of combined chemotherapy before and concurrent with surgery and local radiotherapy for the involved bone. Cisplatin is one of the most widely used drugs for the treatment of bone tumors in children, but is not currently used in ES. We describe a child with multifocal ES, treated with a phase II trial including a single-drug window therapy, which displayed a dramatic response to 2 courses of cisplatin and had a favorable outcome.Key Words: Ewing sarcoma, advanced metastatic disease, window therapy, cisplatin, outcome (J Pediatr Hematol Oncol 2013;35:478-481) E wing sarcoma (ES) is the second most common bone malignancy in children and young adults, with a slight male predominance. 1 ES has a high propensity to metastasize, and about 25% of patients show disseminated involvement at the beginning of disease. Multimodal treatment approaches, including surgery, radiotherapy (RT), and intensive multidrug chemotherapy, have led to notable improvement in the outcome of patients with localized disease, up to about 70% of event-free survival (EFS). 2,3 In contrast, the prognosis of patients with primary disseminated disease remains very poor, with an EFS of <20%. 4 Patients with primary pulmonary metastases show a better outcome than patients with primary bone and/or bone marrow involvement. 4-8 Conventional treatment regimens for localized ES generally consist of a combination of vincristine, actinomycin-D, cyclophosphamide, doxorubicin, ifosfamide, and etoposide. Adjunctive surgical resection with or without radiation therapy is used for local control. Most episodes of disease recurrence occur after completion of therapy, and most recurrences (approximately 80%) occur within 2 years from initial diagnosis. 9 Cisplatin (CDDP) and other platinum compounds are widely used drugs for the treatment of solid tumors in adults and children, especially when failure of first-line therapy occurs, but data concerning its activity and efficacy are very limited in ES.We describe an impressive response to front-line CDDP as a single drug in a child with metastatic ES with a favorable clinical outcome.
CASE REPORTA 10-year-old African boy was admitted 10 months after the onset of left elbow pain, followed by progressive swelling. He was diagnosed with an osteosarcoma (small cell variant) of the left arm in a Nigerian Hospital. He did not receive any therapy in Nigeria because they concentrate the few economical resources available on patients with more chances of healing. An international committee organized a transfer abroad to verify the histologic diagnosis and to explore the possibility of a therapeutic approach.On admission the boy showed a huge mass (circumference 43 cm, whereas the contralateral was 15 cm) in the left elbow, and packed lymph n...