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Pediatric Oncology

Ewing’s Sarcoma Family of Tumors: Current Management

^a Ste-Justine Hospital, University of Montreal, Montreal, Canada; ^b Children’s Cancer Research Institute, Vienna, Austria; ^c University Children’s Hospital Basel, Basel, Switzerland; ^d Huntsman Cancer Institute & Primary Children’s Medical Center, University of Utah, Salt Lake City, Utah, USA; ^e Department of Radiotherapy, University Hospital Muenster, Münster, Germany; ^f Department of Pathology, University of Pittsburgh School of Medicine and Children’s Hospital of Pittsburgh, Pittsburgh, Pennsylvania, USA; ^g University of Muenster, Children’s Hospital, Paediatric Haematology and Oncology, Muenster, Germany

Key Words. Ewing’s sarcoma • Bone cancer • Multimodal therapy • Pediatrics • Adolescents and young adults

Correspondence: Mark Bernstein, M.D., F.R.C.P.(C)., Service of Hematology/Oncology, Ste-Justine Hospital, University of Montreal, 3175 Cote Ste. Catherine Road, Montreal, Quebec, H3T 1C5, Canada. Telephone: 514-345-4969; Fax: 514-345-4792; e-mail: mark. lawrence.bernstein{at}umontreal.ca

Ewing’s sarcoma is the second most frequent primary bone cancer, with approximately 225 new cases diagnosed each year in patients less than 20 years of age in North America. It is one of the pediatric small round blue cell tumors, characterized by strong membrane expression of CD99 in a chain-mail pattern and negativity for lymphoid (CD45), rhabdomyosarcoma (myogenin, desmin, actin) and neuroblastoma (neurofilament protein) markers. Pathognomonic translocations involving the ews gene on chromosome 22 and an ets-type gene, most commonly the fli1 gene on chromosome 11, are implicated in the great majority of cases. Clinical presentation is usually dominated by local bone pain and a mass. Imaging reveals a technetium pyrophosphate avid lesion that, on plain radiograph, is destructive, diaphyseal and classically causes layered periosteal calcification. Magnetic resonance best defines the extent of the lesion. Biopsy should be undertaken by an experienced orthopedic oncologist. Approximately three quarters of patients have initially localized disease. About two thirds survive disease-free. Management, preferably at a specialist center with a multi-disciplinary team, includes both local control—either surgery, radiation or a combination—and systemic chemotherapy. Chemotherapy includes cyclic combinations, incorporating vincristine, doxorubicin, cyclophosphamide, etoposide, ifosfamide and occasionally actinomycin D. Topotecan in combination with cyclophosphamide has shown preliminary activity. Patients with initially metastatic disease fare less well, with about one quarter surviving. Studies incorporating intensive therapy followed by stem cell infusion show no clear benefit. New approaches include anti-angiogenic therapy, particularly since vascular endothelial growth factor is an apparent downstream target of the ews-fli1 oncogene.

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