No abstract

Summary: Patients and methodsEighteen patients were entered into the study. There were Eighteen patients with poor risk Ewing's sarcoma (including 11 patients with metastatic disease at nine females. The median age at diagnosis was 14.2 years (range 2.75-30 years). Primary tumours were of the central presentation) received consolidation therapy of busulphan and melphalan with autologous stem cell rescue.skeleton in 10 cases and long bones in eight cases. Eleven cases were metastatic at initial presentation, 10 with pulThere were nine females. The median age at diagnosis was 14.2 years (range 2.75-30 years). There was one monary disease and one with bone marrow involvement. The primary tumour of the metastatic cases was central early death due to cytomegalovirus pneumonitis. One patient developed a single generalised convulsion during skeleton in six, and long bones in five cases. Six of the seven non-metastatic patients had bulky primary tumours busulphan therapy. Severe renal toxicity was not encountered. One patient developed veno-occlusive dis-(у100 ml) and the seventh patient was in second complete remission at the time of high-dose therapy. In total 12 ease of the liver (VOD) which eventually resolved. With a median follow up of 2 years, 13 patients survive patients were in first complete remission (CR1), four in CR2, one in partial remission (PR) and one with progressincluding six with initial metastatic disease. We conclude that high-dose busulphan/melphalan is well-tolerive disease (PD) at the time of HDT.

Over a period of 14 years, 50 patients (12 children and 38 adults) of whom 46 had acute myeloid leukemia (AML) and 4 had myelodysplastic syndrome characterized by the t(8;21)(q22;q22) translocation were referred to the Royal Marsden Hospital. The clinicopathological features of these cases were analyzed to determine the influence of age, secondary karyotype abnormalities, and expression of the lymphoid marker CD19 on event free survival, and presence of extramedullary leukemia on overall survival. They were treated with a variety of chemotherapy protocols and some had bone marrow transplantation. There appeared to be no difference in survival between children (age <17 years) and adults (age >16 years). Out of the 50 cases, 16 (32%) had the (8;21) translocation alone, 17 (34%) had additional loss of a sex chromosome and the remaining 17 (34%) had other karyotype abnormalities of which deletion or translocation of the long arms of a #9 was most common (observed in 8 of the 17 patients). The karyotype groups had a significant impact on survival, the group with loss of a sex chromosome having a poorer outcome and the group with abnormalities of chromosome 9 having a better outcome. CD19 positivity was seen in 21 of the 33 cases (63%) in whom it was measured compared to 11% observed in controls with AML without a t(8;21). CD19 status did not exert any influence on event free survival. Extramedullary leukemia (EML) occurred in 5 of the 50 cases (10%). In one patient it was observed at diagnosis but in the others it presented concurrent with bone marrow relapse. The overall survival of patients with EML was worse than that of the other patients but did not achieve statistical significance and was probably adversely affected by other factors.

Among 447 children with non-Hodgkin's lymphoma (NHL) on the childhood U.K. registry, seven children with follicular (NHL) were identified. Four were male and their age ranged from 4.25 to 13.5 years (median 7.5); all had localized disease, Murphy's stage I (n = 4) and II (n = 3). Sites involved at presentation were cervical lymph nodes and tonsils (n = 5), ileum (n = 1) and parotid gland (n = 1). Three had complete surgical excision only and four had complete (n = 1) or incomplete excision (n= 3) followed by a short multi-agent chemotherapy regimen (UKCCSG 9001 protocol). With a median follow-up of 1.5 years (range 0.25-5 years) from diagnosis, six are alive in complete remission (CR) including three who had no chemotherapy. These results confirm previous reports that follicular lymphomas in children are rare (1.5%) and tend to be localized at presentation. Their rarity makes it difficult to produce guidelines about treatment, but in localized cases a period of non-intervention may be justified.