The dismal prognosis of patients with disseminated Ewing sarcoma necessitates the development of novel treatment strategies. Pazopanib is an oral multi-targeted tyrosine kinase inhibitor that is active against advanced soft tissue sarcoma. However, the clinical activity and feasibility of pazopanib for treating Ewing sarcoma remain poorly understood. Moreover, clinical information on the use of tandem high-dose chemotherapy for Ewing sarcoma is limited. A 14-year-old boy with Ewing sarcoma was transferred to our hospital for treatment. Magnetic resonance imaging, computed tomography scans, and bone scintigraphy revealed multiple lesions in the pubis, ilium, ischium, femur, rib, cranial bone, thoracic vertebrae, sacrum, obturator muscle, adductor magnus muscle, testicular cord, and lungs. Bone scintigraphy after intensive chemotherapies confirmed that multiple abnormal accumulations were still present in the cranial bone and pubis. Subsequently, the patient received tandem high-dose chemotherapy including topotecan, and radiotherapy. Abnormal accumulations have disappeared in bone scintigraphy. Subsequently, pazopanib maintenance therapy was initiated. Despite the presence of innumerable lesions at diagnosis, the patient has been in near-complete remission for the past 1 year with pazopanib administration. This confirms that adding pazopanib maintenance therapy after tandem high-dose chemotherapy is a therapeutic option for cases with disseminated Ewing sarcoma.
Disseminated Ewing sarcoma Pazopanib Tandem high-dose chemotherapy
This is a preview of subscription content, log in to check access.
Compliance with ethical standards
Conflict of interest
The authors have no conflict of interest to disclose. This study was conducted in accordance with the Declaration of Helsinki. Written and oral informed consent for publication was obtained from the patient’s guardians.
Ladenstein R, Pötschger U, Le Deley MC et al (2010) Primary disseminated multifocal Ewing sarcoma: results of the Euro-EWING 99 trial. J Clin Oncol 28:3284–3291CrossRefPubMedGoogle Scholar
Gaspar N, Hawkins DS, Dirksen U et al (2015) Ewing sarcoma: current management and future approaches through collaboration. J Clin Oncol 33:3036–3046CrossRefPubMedGoogle Scholar
Paulussen M, Ahrens S, Burdach S et al (1998) Primary metastatic (stage IV) Ewing tumor: survival analysis of 171 patients from the EICESS studies. European Intergroup Cooperative Ewing Sarcoma Studies. Ann Oncol 9:275–281CrossRefPubMedGoogle Scholar
Loschi S, Dufour C, Oberlin O et al (2015) Tandem high-dose chemotherapy strategy as first-line treatment of primary disseminated multifocal Ewing sarcomas in children, adolescents and young adults. Bone Marrow Transpl 50:1083–1088CrossRefGoogle Scholar
Thiel U, Wawer A, von Luettichau I et al (2016) Bone marrow involvement identifies a subgroup of advanced Ewing sarcoma patients with fatal outcome irrespective of therapy in contrast to curable patients with multiple bone metastases but unaffected marrow. Oncotarget 7:70959–70968CrossRefPubMedGoogle Scholar
van der Graaf WT, Blay JY, Chawla SP et al (2012) Pazopanib for metastatic soft-tissue sarcoma (PALETTE): a randomised, double-blind, placebo-controlled phase 3 trial. Lancet 379:1879–1886CrossRefPubMedGoogle Scholar
Juergens C, Weston C, Lewis I et al (2006) Safety assessment of intensive induction with vincristine, ifosfamide, doxorubicin, and etoposide (VIDE) in the treatment of Ewing tumors in the EURO-E.W.I.N.G. 99 clinical trial. Pediatr Blood Cancer 47:22–29CrossRefPubMedGoogle Scholar
Casey DA, Wexler LH, Merchant MS et al (2009) Irinotecan and temozolomide for Ewing sarcoma: the Memorial Sloan-Kettering experience. Pediatr Blood Cancer 53:1029–1034CrossRefPubMedGoogle Scholar
Burke MJ, Walterhouse DO, Jacobsohn DA et al (2007) Tandem high-dose chemotherapy with autologous peripheral hematopoietic progenitor cell rescue as consolidation therapy for patients with high-risk Ewing family tumors. Pediatr Blood Cancer 49:196–198CrossRefPubMedGoogle Scholar
Donato ML, Gershenson DM, Wharton JT et al (2001) High-dose topotecan, melphalan, and cyclophosphamide (TMC) with stem cell support: a new regimen for the treatment of advanced ovarian cancer. Gynecol Oncol 82:420–426CrossRefPubMedGoogle Scholar
Kazmi SM, Saliba RM, Donato M et al (2011) Phase II trial of high-dose topotecan, melphalan and CY with autologous stem cell support for multiple myeloma. Bone Marrow Transpl 46:510–515CrossRefGoogle Scholar
Yamamoto Y, Nozawa M, Shimizu N et al (2014) Pazopanib for recurrent extraosseous Ewing’s sarcoma of the retroperitoneum. Int J Urol 21:1183–1184CrossRefPubMedGoogle Scholar
Attia S, Okuno SH, Robinson SI et al (2015) Clinical activity of pazopanib in metastatic extraosseous Ewing sarcoma. Rare Tumors 7:5992CrossRefPubMedGoogle Scholar