Graft-Versus–Ewing Sarcoma Effect and Long-Term Remission Induced by Haploidentical Stem-Cell Transplantation in a Patient With Relapse of Metastatic Disease

Abstract

TO THE EDITOR: The chance of cure is very low for patients with primary metastatic or relapsed rhabdomyosarcoma or Ewing tumors. 1-3 In the German multicenter studies CWS-81, -86, -91, and -96, patients older than 10 years at diagnosis with bone or bone marrow metastases had a 5-year event-free survival of 2%. 4 This poor outcome is due mainly to the high relapse rate after initial chemotherapy, and patients in remission after double high-dose chemotherapy (HDC) had a median time to relapse of 5.7 months (range, 3 to 9 months). Unfortunately, there is no single survivor registered after relapse. 4 Allogeneic transplantation might provide a possible graft-versustumor effect, and regression of metastatic lesions has been documented in patients with solid tumors. 5-8 To exert a graft-versus-tumor effect, we have performed allogeneic transplantation using a haploidentical donor in a patient with metastatic Ewing sarcoma who relapsed after double HDC. A previously healthy 15-year-old girl was diagnosed in April 1998 with a disseminated Ewing sarcoma. Magnetic resonance imaging (MRI) scans showed a 10 11-cm tumor originating from the thorax wall, with erosion and destruction of the 11th rib, with penetration into the retropleural thoracic and retrocrural space. It extended caudally to the upper left kidney pole and spleen, and laterally into the foramina intervertebralia between Th7 and Th11. Skeletal imaging showed metastatic lesions in the eighth rib and in vertebrae C7, Th1, Th5, Th6, Th7, and L5. A chemotherapy scan showed four lung nodules. Bone marrow aspirated from the right and left posterior iliac spines showed massive tumor cell infiltration at both sites. After initial chemotherapy, an almost complete remission was achieved (only residual tumor was seen in the primary site in the 11th rib), and she received consolidation with double HDC and autologous hematopoietic stem-cell rescue. MRI after the second HDC showed residual tumor in the 11th rib, which was locally irradiated. The patient remained well for 8 months when routine imaging showed a large new lytic lesion in the right parietal bone (Fig 1A), with a component occupying the extradural space (Fig 1B).