United Kingdom Children's Cancer Study Group Research Articles

Is radiotherapy required in first-line treatment of stage I diffuse anaplastic Wilms tumor? A report of SIOP-RTSG, AIEOP, JWiTS, and UKCCSG.

As a significant proportion of relapses occurred in the tumor bed or abdomen on patients with the fifth National Wilms Tumor Study stage I anaplastic Wilms tumor (WT), flank radiotherapy was added for stage I anaplastic WT in the subsequent study of the Children's Oncology Group (AREN0321). Preliminary results revealed reduction of relapse rate and improved survival. In cases treated with preoperative chemotherapy, such as in International Society of Pediatric Oncology (SIOP), the value of radiotherapy has never been studied. The aim of this observational study is to describe the pattern of recurrence and survival of patients with stage I diffuse anaplastic WT (DAWT) after induction chemotherapy. Retrospective data analysis of the pattern of relapse and survival of all patients with stage I DAWT were included in recent SIOP, L'Associazone Italiana Ematologica Oncologia Pediatrica (AIEOP), Japan Wilms Tumor Study Group (JWiTS), United Kingdom Children's Cancer Study Group (UKCCSG) renal tumor registries. Postoperative treatment consisted of actinomycin D, vincristine, and doxorubicin for 28weeks without local irradiation. One hundred nine cases with stage I DAWT were identified, of which 95 cases received preoperative chemotherapy. Of these, seven patients underwent preoperative true-cut biopsy. Sixteen of the 95 patients relapsed (17%), six locally, four at distant site, and six combined, and all treated according to SIOP 2001 relapse protocol, which resulted in a 5-year overall survival of 93%. Despite 13% locoregional relapse rate, an excellent rescue rate was achieved after salvage treatment, in patients with stage I DAWT whose first-line treatment comprised three-drug chemotherapy (including doxorubicin), without flank irradiation. Therefore, we continue not to advocate the use of radiotherapy in first-line treatment after preoperative chemotherapy in stage I DAWT in the next SIOP protocol.

The effect of preoperative chemotherapy on histological subtyping and staging of Wilms tumors: The United Kingdom Children's Cancer Study Group (UKCCSG) Wilms tumor trial 3 (UKW3) experience.

Two principal approaches to Wilms tumor (WT) treatment are immediate surgery (IS) and preoperative chemotherapy (PCT), and both treatments use the risk-adapted approach that includes histological subclassification of the tumor, combined with additional prognostic factors. In the UKW3 trial, these two approaches were compared. The aim of the present study was to compare histological features between the two groups, to assess the impact of PCT on distribution of histological subtyping and staging and to evaluate whether PCT resulted in more staging discrepancies between local and central pathology review (CPR). The cases were identified from the UKW3 trial database. The criteria for inclusion in the study were unilateral, nonmetastatic, nonanaplastic WTs, and submitted for CPR with an adequate number of slides. They were subclassified according to the NWTS and later the SIOP 9301 criteria. There were 244 WTs in the IS and 182 in the PCT group subclassified as follows: blastemal 86 (35%) vs 9 (5%), epithelial 34 (14%) vs 12 (7%), stromal 12 (5%) vs 25 (14%), mixed 112 (46%) vs 45 (25%), respectively, plus 40% regressive and 10% completely necrotic WTs in the PCT group. The differences between the two groups for blastemal and mixed types were statistically significant. In the PCT group, there was a significant decrease in stage III tumors. The discrepancies in staging between local and CPR were not significant. PCT significantly altered histological features and typing of WTs. It resulted in fewer stage III tumors, and staging discrepancies were equally represented in both groups.

Childhood Lymphoblastic Lymphoma: Hospital Based Study.

Background: Lymphoblastic lymphomas (LBL) are neoplasms of precursor T cells and B cells, or lymphoblasts. The term lymphoblastic lymphoma has been used to describe predominantly lymph node– based disease; however, clinical distinction between LBL and acute lymphoblastic leukemia (ALL) has been arbitrary and has varied among different studies and institutionsObjectives: To determine the frequency of LBL among all Non-Hodgkin’s lymphoma (NHL) patients in children and to study the clinical and pathological features of LBL and assess the treatment outcome.Methods: A retrospective study included 28 children with newly diagnosed LBL (based on morphology) below the age of 14 years over 8 years period from January 1st, 2000 to December 31st, 2007 All the patients except one were treated by modified Medical Research Council UK National Randomized Trial For Children and young Adult with Acute Lymphoblastic Leukemia (MRC UKALL) regimen, one patient was misdiagnosed as B-cell NHL and treated with United Kingdom Children’s Cancer Study Group (UKCCSG) Non-Hodgkin’s Lymphoma protocol.Results: LBL forms 28/376 (7.1%) among NHL diagnosed in Children Welfare Teaching Hospital (CWTH) in the same period. The median age was 8.95 years (range 2 to 13.25 years) with male to female ratio of 3.7:1. Lymphadenopathy was present in 22 (78 .6%) of patients. The median duration of onset of symptoms was 7.25 months (range 1 week to 18 months). In response to treatment, 20 (71.4%) patients achieved complete remission (CR), 1 (3.6%) died during induction and 7 (25%) patients were non-responder (4 died and 3 abandoned). Of the 20 patients who achieved complete remission (CR); thirteen (46.4%) remained in continuous complete remission with a median follow up of 32.3 months, 3 (10.7%) patients died while inCR in an average of 16.6 weeks, 2 (7.1%) patients abandoned treatment and 2 (7.1%) patients relapsed.Conclusion: The study showed a low frequency of LBL in comparison with other studies which might be due to inadequate diagnostic facilities which differentiate LBL from other types of NHL, low survival rate might be due to advanced stages at presentation in addition to abandonment of treatment in some patients

A survey of public transport use by immunocompromised children in the United Kingdom

The advice given to immunocompromised children and their families on the use of public transport at our oncology unit has been inconsistent, with a lack of written or evidence based guidelines. We therefore carried out two surveys, one national and one local on this issue, to determine what advice is given to families. We also undertook an extensive literature search on this subject. The first survey was completed by the Paediatric Oncology Nurses Forum link member at each of the 22 United Kingdom Childrens' Cancer Study Group (UKCCSG) centres. We asked their current practice for use of public transport for hospital visits, school attendance, regular weekly use and holidays. We looked at recommendations for travel on holiday in the UK and abroad with reference to neutrophil and platelet counts. The second survey was completed by parents of 64 children attending our outpatient clinic over a 2-week period to ascertain whether the families comply with our verbal recommendations. There was a 100% response from UKCCSG centres. None of the centres had any written or evidence based guidelines. 55% of centres allowed public transport for hospital visits, 82% for school and 90% for holidays. Sixty-eight percent of centres allowed UK travel with a neutrophil count of 0.5-1 x 109/L and 18% if neutrophils <0.5 x 109/L. Eighteen percent of centres allowed foreign travel with a neutrophil count of 0.5-1 x 109/L but only 9% if <0.5 x 109/L. The second survey showed 2% used public transport to attend hospital, 5% for school, 17% for regular use and 50% travelled by plane for holidays. A Pubmed literature search revealed a lack of studies on immunocompromised patients. A certain use of public transport is necessary for patients to engage in as normal a life as possible. There is a need for further studies to produce sensible and consistent advice on this issue.

Outcome of children with nodular lymphocyte predominant Hodgkin lymphoma – a Children's Cancer and Leukaemia Group report

This report describes the clinical outcomes and follow-up records of 42 children with nodular lymphocyte predominant Hodgkin lymphoma (LPHL) treated on United Kingdom Children's Cancer Study Group (UKCCSG) HD1 (1982-1992) and HD2 protocols (1992-2000). The clinical records of 42 children with LPHL treated between 1982 and 2000 were reviewed retrospectively. All 42 had histology reviewed centrally and confirmed as LPHL by an expert panel. In both trials, only patients with stage IA disease had the option of being treated with either involved field radiation alone or combination chemotherapy consisting of chlorambucil, vinblastine, procarbazine and prednisolone (ChlVPP). Patients with all other stages were treated with ChlVPP chemotherapy. Thirty-five patients (83%) presented with early stage disease (Stages I & II). All 42 patients achieved a complete remission (CR). Six children relapsed after primary therapy. The 5- and 10-year relapse-free survival rates were 87% and 82% respectively. Forty-one are currently alive in CR. In conclusion, children with low-stage LPHL treated between 1982 and 2000 according to the UK strategy for classical Hodgkin lymphoma (HL) had an excellent prognosis. There have been no second malignancies or transformations to B-cell non-Hodgkin lymphoma.

Hodgkin’s lymphoma in children aged 5 years or less – The United Kingdom experience

Purpose The aim of this study is to describe the natural history of Hodgkin’s Lymphoma (HL) in a large unselected group of children aged 5 years or below at diagnosis, who were treated on a standard treatment programme in the United Kingdom between 1982 and 2000. Methods Eighty-one unselected children with HL aged 5 years or under at diagnosis, treated on the United Kingdom Children’s Cancer Study Group (UKCCSG) Hodgkin’s trials HD1 (1982–1992) and HD2 (1992–2000), were included in the study. Results Sixty-one patients (81%) presented with early stage disease ( n = 66). Fifty-three patients (65%) received combination chemotherapy, 28 (34%) received involved field radiotherapy (IF-RT) and 4 patients were treated with combined modality therapy. Eighteen children relapsed after primary therapy. Conclusions Children treated with IF-RT had a higher rate of primary treatment failures as well as increased late treatment-related morbidity.

Long-term outcome in children with Hodgkin’s lymphoma: The United Kingdom Children’s Cancer Study Group HD82 trial

Background The aim of United Kingdom Children’s Cancer Study Group (UKCCSG) HD82 was to establish the efficacy of chlorambucil/vinblastine/procarbazine/prednisolone (ChlVPP) in the treatment of childhood Hodgkin’s lymphoma stages II–IV and radiotherapy (RT) alone in stage I patients. We report on the status of these patients to a follow-up of 20 years. Methods Treatment consisted of 35 Gy involved-field RT for stage I and ChlVPP alone for stages II–IV. Adjuvant RT (35 Gy) was administered to those with bulky mediastinal disease. Results Of the 358 patients, the 10-year EFS/OS per stage is I (65.4%/92.6%), II (80.0%/93.3%), III (68.8%/85.0%), IV (45.5%/72.7%). The corresponding 20-year OS rates are similar with a combined (all stage) rate dropping from 89.3% to 89.0% over the decade. The cumulative 20-year malignancy rate is 7.29%. Conclusion Single modality treatment provided relatively low EFS at 10-years but comparable long-term OS, relative to contemporary published combined modality regimens, for stages I–III but not for stage IV patients.

Variation in policies for the management of febrile neutropenia in United Kingdom Children’s Cancer Study Group centres

Objective: To assess the variation in the current UK management strategies for the treatment of febrile neutropenia in childhood. Design and setting: A postal survey of all 21 United Kingdom...

Single agent efficacy of rituximab in childhood immunosuppression related lymphoproliferative disease: a United Kingdom Children's Cancer Study Group (UKCCSG) retrospective review

Survival in childhood lymphoproliferative disease (LPD) remains poor, particularly in non-transplant patients. The anti-CD20 antibody rituximab shows promise but data in children is scant. A retrospective study of 22 (aged 11 months to 18 years) children treated with rituximab is presented. Two had primary immunodeficiency, two had prolonged immunosuppression and 18 had post-transplant LPD (eight bone marrow, five liver, four heart, one kidney). Nine patients had multi-organ involvement and 13 single site disease. Seventeen out of 22 had rituximab alone. In 16, a dose of 375 mg/m2 i.v. weekly was used (less in one patient due to renal dysfunction). Twelve patients received four courses and ten patients received one to three courses. Fever was the main side-effect in four. Eight (47%) had single agent response; four complete and four partial. All had other treatment prior to rituximab. Median follow-up was 35 months (range 22 – 47 months). In childhood LPD unresponsive to standard treatment, rituximab showed single agent response and requires further evaluation.

β-Catenin Status Predicts a Favorable Outcome in Childhood Medulloblastoma: The United Kingdom Children's Cancer Study Group Brain Tumour Committee

Identifying pathobiological correlates of clinical behavior or therapeutic response currently represents a key challenge for medulloblastoma research. Nuclear accumulation of the beta-catenin protein is associated with activation of the Wnt/Wg signaling pathway, and mutations affecting components of this pathway have been reported in approximately 15% of sporadic medulloblastomas. We tested the hypothesis that nuclear immunoreactivity for beta-catenin is a prognostic marker in medulloblastoma, and assessed the relationship between nuclear beta-catenin immunoreactivity and mutations of CTNNB1 and APC. Medulloblastomas from children entered onto the International Society for Pediatric Oncology (SIOP)/United Kingdom Children's Cancer Study Group (UKCCSG) PNET3 trial (n = 109) were examined for beta-catenin immunoreactivity, and where tissue was available, evidence of CTNNB1 and APC mutations. The results were correlated with clinicopathologic variables, principally outcome. Children with medulloblastomas that showed a nucleopositive beta-catenin immunophenotype (27 of 109; 25%) had significantly better overall (OS) and event-free (EFS) survivals than children with tumors that showed either membranous/cytoplasmic beta-catenin immunoreactivity or no immunoreactivity (P = .0015 and P = .0026, respectively). For beta-catenin nucleopositive and nucleonegative medulloblastomas, 5-year OS was 92.3% (95% CI, 82% to 100%) versus 65.3% (95% CI, 54.8 to 75.7%), and 5-year EFS was 88.9% (95% CI, 77% to 100%) versus 59.5% (95% CI, 48.8 to 70.2%), respectively. Mutations in CTNNB1 were found exclusively among medulloblastomas that demonstrated nuclear beta-catenin immunoreactivity, but no evidence of APC mutation was found in these cases. All children with beta-catenin nucleopositive large cell/anaplastic medulloblastomas and beta-catenin nucleopositive medulloblastomas presenting with metastatic disease are alive at least 5 years postdiagnosis. Nuclear accumulation of beta-catenin appears to be a marker of favorable outcome in medulloblastoma, and should be investigated further in large group-wide trials.

Paediatric medulloblastoma associated with poor prognosis and short volume doubling time

CorrespondencePaediatric medulloblastoma associated with poor prognosis and short volume doubling timeS Bacon, J Clinkard and R E TaylorS BaconDepartments of 1Medical Physics and 2Clinical Oncology, Cookridge Hospital, Leeds, LS16 6QB, UKSearch for more papers by this author, J ClinkardDepartments of 1Medical Physics and 2Clinical Oncology, Cookridge Hospital, Leeds, LS16 6QB, UKSearch for more papers by this author and R E TaylorDepartments of 1Medical Physics and 2Clinical Oncology, Cookridge Hospital, Leeds, LS16 6QB, UKSearch for more papers by this authorPublished Online:28 Jan 2014https://doi.org/10.1259/bjr/29536247SectionsPDF/EPUBFull Text ToolsAdd to favoritesDownload CitationsTrack Citations ShareShare onFacebookTwitterLinked InEmail About"Paediatric medulloblastoma associated with poor prognosis and short volume doubling time." The British Journal of Radiology, 78(935), pp. 1059–1060 References 1 Taylor RE. UKCCSG Radiotherapy and Brain Tumour groups. Medulloblastoma/PNET and craniospinal radiotherapy (CSRT). Report of a workshop held in Leeds 30.6.99. Clin Oncol 2001;13:58–64. Google Scholar2 Taylor RE, Lucraft H, Bailey CC, Robinson KJ, Weston CL, Ellison D, et al. Impact of radiotherapy parameters on outcome in the International Society of Paediatric Oncology (SIOP)/United Kingdom Children's Cancer Study Group (UKCCSG) PNET-3 study of pre-radiotherapy chemotherapy for M0-1. Int J Radiat Oncol Biol Phys 2004;58:1184–93. Crossref Medline ISI, Google Scholar3 Ash D, Barrett A, Hicks A, Squire C. Re-audit of radiotherapy waiting times 2003. Clin Oncol 2004;16:387–94. Crossref ISI, Google Scholar4 Ito S, Hoshino T, Prados MD, Edwards SB. Cell kinetics of medulloblastomas. Cancer 1992;70:671–8. Crossref Medline ISI, Google Scholar5 Yamashita T, Kuwubara T. Estimation of rate of growth of malignant brain tumors by computed tomography scanning. Surg Neurol 1983;20:464–70. Crossref Medline, Google Scholar Previous article Next article FiguresReferencesRelatedDetailsCited byNatural history of medulloblastoma in a child with neurofibromatosis type IAsian Journal of Neurosurgery, Vol. 13, No. 3A medulloblastoma showing an unusually long doubling time: reflection of its singular nature6 January 2016 | Child's Nervous System, Vol. 32, No. 6Imaging characteristics of pilomyxoid astrocytomas in comparison with pilocytic astrocytomasEuropean Journal of Radiology, Vol. 79, No. 2Atypical teratoid/rhabdoid tumors of the central nervous system: imaging and clinical findings in 16 childrenClinical Radiology, Vol. 64, No. 3Radiologia pediatricaPediatric Radiology Volume 78, Issue 935November 2005Pages: 973-1064 © The British Institute of Radiology History ReceivedJune 13,2005AcceptedJune 15,2005Published onlineJanuary 28,2014 Metrics Download PDF

The treatment of stages I-IV favorable histology Wilms' tumor.

The prognosis for children with Wilms’ tumor (WT) has improved dramatically. During the last three decades, the National Wilms Tumor Study Group (NWTSG), the International Society of Pediatric Oncology (SIOP), and the United Kingdom Children’s Cancer Study Group (UKCCSG) conducted sequential studies of treatments for children with WT. The results will be reviewed in the light of long-term follow-up. Areas for future investigation will be identified.

A survey of current practice with regard to oral care for children being treated for cancer

The aim of the study was to establish current UK oral care practice for children with cancer. A telephone survey of all 22 United Kingdom Children's Cancer Study Group (UKCCSG) centres was undertaken. Nineteen (86%) of the centres reported using guidelines/protocols for mouth care. The use of routine preventive oral care therapies showed the greatest variation between centres. Four centres (18%) did not use any prophylactic oral care therapy other than basic oral hygiene, whereas seven (32%) routinely used a combination of three or more agents. Chlorhexidine was the most frequently administered prophylactic therapy (17/22 centres, 77%), followed by nystatin (11/22 centres, 50%). There was little variation in advice given to parents/patients on basic oral hygiene. Regarding dental check-ups, 9/22 centres (41%) recommended children to attend a hospital-linked dental clinic. Only at 8/22 centres (36%) did children undergo a dental check-up before commencing cancer treatment. The survey identified significant variation in preventive oral care therapies and dental check-ups at the UKCCSG centres. Attention needs to be given to establishing evidence based, effective strategies.

Long-term follow-up of survivors of childhood cancer in the UK.

Childhood cancer is rare, but there are now good survival prospects and in the UK approximately 1 in 1,000 young adults is a survivor of childhood cancer. There are many adverse health outcomes associated with the treatment of childhood cancer often arising several years after completion of treatment. The aim of this study was to quantify the long-term clinical follow-up practices concerning survivors of childhood cancer. A cross-sectional postal survey of 22 treatment centres of the United Kingdom Children's Cancer Study Group (UKCCSG) clinicians was carried out as well as a cross-sectional postal survey of general practitioners of most adult survivors of childhood cancer in Britain. Subsequent to 5 years after the end of treatment: 52% of UKCCSG clinicians follow-up all survivors for life, while 45% discharge some patients. Of those clinicians discharging: over 50% discharged benign, stage I or tumors treated with surgery alone, in contrast 16% reported discharging all or most patients; almost all (97%) clinicians discharged to a general practitioner. Only 14% of clinicians reported nurses undertook a specialist role. Sixty-five percent of the 10,979 general practitioners reported that their patient was not on regular hospital follow-up. There are wide variations in the extent to which survivors of childhood cancer are discharged from hospital follow-up. There is a need for regularly updated national guidelines concerning the levels of follow-up required for specific groups of survivors defined principally by the treatment they received.

Stature of young people with malignant bone tumors.

Little is known about the aetiology of primary bone tumours. There have been conflicting reports relating to stature in young people with bone cancer. We analysed height data at diagnosis for 364 patients with osteosarcoma and 356 patients with Ewing sarcoma registered on clinical trials run by the Medical Research Council (MRC) and the United Kingdom Children's Cancer Study Group (UKCCSG). Height at diagnosis for each patient was standardised for age and sex compared to national reference data with a standard deviation score (SDS) calculated for each subject. Those with osteosarcoma were significantly taller than the general population (mean height SDS 0.2, P=0.001). Patients with osteosarcoma of the femur were significantly taller than patients with other primary sites (mean height SDS 0.45 vs. -0.06, P=0.0001). Overall those with Ewing sarcoma were not significantly taller than the general population (mean height SDS 0.09, P=0.1), but children presenting under 15 years were taller (SDS 0.2, P=0.004) whilst older patients were not (SDS -0.07, P=0.4). In both osteosarcoma and Ewing sarcoma the mean age at diagnosis for females was significantly younger than for males. This study suggests that tall stature and an earlier pubertal growth spurt may be important factors in the aetiology of both osteosarcoma and Ewing sarcoma.

Recruiting children into cancer trials--role of the United Kingdom Children's Cancer Study Group (UKCCSG).

The UK Children's Cancer Study Group (UKCCSG), established in 1977, provides a highly organised structure for both service provision and research, and represents the model to which the adult cancer community is currently aspiring. Since childhood cancer is so rare, it is both essential and feasible for the vast majority of children to be referred into the network of specialist centres, and also for the maximum number of children to be recruited into national and international clinical trials. Over the last 30–40 years there have been major advances in treatment, such that now approximately 70% of children diagnosed with cancer will be cured of their disease. The conduct of clinical trials in this patient population does, however, raise a number of specific issues and these are discussed in the paper.

An overview of sperm cryopreservation services for adolescent cancer patients in the United Kingdom

For young men, a consequence of surviving childhood malignancy can be iatrogenic infertility. Current health policies focus on the elimination of ‘post code lotteries’ in cancer services. The extent to which sperm cryopreservation services for young men at risk of infertility from cancer treatment are provided and standardised within the United Kingdom and Ireland, was therefore the subject of this research. This paper draws on data from a three-stage study to describe sperm cryopreservation services for adolescent males, identifying current provision of sperm cryopreservation at the majority of United Kingdom Children's Cancer Study Group (UKCCSG) centres. In particular, the ways in which services are managed and written information provided to patients and their families was focused upon. Nurses from 18 of 22 UKCCSG centres responded to a questionnaire, six nurses from the replying centres participated in further, focused interviews. Results suggested that, during a 1 year period, approximately 118 adolescent males within the United Kingdom and Ireland could potentially have benefited from cryopreservation services. Of the responding centres, 15 offered a cryopreservation service. However, the majority ( n=14) lacked consistency and co-ordination in their service provision. The provision of written information to this patient group was limited and analysis revealed all was of a poor quality. Findings from the study led the researchers to conclude that all young men at risk of iatrogenic sterility from cancer treatment could benefit from the production and systematic application of national guidelines and that standardising sperm cryopreservation services would fit with current health policy.

Outcome after relapse in an unselected cohort of children and adolescents with Ewing sarcoma.

Survival after relapse in patients with Ewing sarcoma is very poor and this retrospective study attempts to identify of prognostic factors predicting survival after relapse. A total of 191 patients with localised Ewing sarcoma were registered in the ET-2 trial of the United Kingdom Children's Cancer Study Group (UKCCSG). All patients received standardised primary treatment with chemotherapy and surgery and or radiotherapy as local modality treatment. Sixty-four patients who relapsed are included in this report. Treatment at relapse was variable and included chemotherapy, surgery, radiotherapy and high dose therapy (HDT) or megatherapy with peripheral stem cell transplantation (PBSCT) or autologous bone marrow transplantation (ABMT) in various combinations. A subgroup of patients had only non-specific symptomatic treatment at relapse. Both univariate and multivariate methods were used to investigate variables affecting survival after relapse. The overall actuarial median survival from relapse for all patients was 14 months (95% CI 11-16 months). Univariate analysis showed that males had a longer survival (median, 16 months vs. 11 months); patients who relapsed while on treatment did worse (median, 3 months vs. 16 months) and patients who had a longer disease-free interval (DFI) prior to relapse had a better outcome (DFI <1 year, median survival = 3 months; DFI 1-2 years, survival = 8 months; DFI > 2 years, median survival = 24 months, P < 0.001). Multivariate analysis confirmed that duration of first remission was the only factor associated with longer survival after relapse. These data suggest that although aggressive therapy may delay disease progression after relapse for some children, the course of the disease after relapse is usually fatal. International co-operative studies are needed to evaluate new strategies.

Sacrococcygeal teratomas: the UK Children's Cancer Study Group's experience. I. Neonatal.

The aim of this study was to review the United Kingdom Children's Cancer Study Group (UKCCSG) experience of sacrococcygeal teratomas (SCT) including histological presentation, response to surgery and chemotherapy, and long term effects of the tumour and treatment. This paper presents the results for those children diagnosed during the neonatal period. Children aged up to 4 weeks with biopsy proven localised or metastatic sacrococcygeal germ cell tumours were eligible. From 1st January 1989 to 31st December 1997 (9 years), 15 UKCCSG centres registered 51 neonates with SCT into GC 8901. Surgery alone was performed in all and the prognosis was good - except for 1 baby who died from massive haemorrhage at the initial operation and 1 who died from the complications of prematurity. Seven of the 51 children (14%) who had teratomas in the neonatal period (5 mature, two immature) had yolk sac tumour (YST) recurrence at: 4, 12, 15, 20, 20, 28 and 32 months of age. These children received chemotherapy in the form of etoposide/bleomycin/carboplatin (JEB) and are alive and well at review. These results emphasise the need for oncological follow-up of SCT and the good response to JEB chemotherapy of malignant teratomas and YST.

ChlVPP chemotherapy in children with stage IV Hodgkin's disease: results of the UKCCSG HD 8201 and HD 9201 studies.

We reviewed the results of two consecutive United Kingdom Childrens' Cancer Study Group (UKCCSG) studies of children with stage IV Hodgkin's Disease (HD) treated between January 1982 and December 1999. Among 697 children with HD, 67 were diagnosed to be stage IV. The median age at diagnosis was 12.7 years (range 4.4-16.2). Thirty-five (52%) were boys. Thirty-nine patients (58%) had B symptoms at diagnosis. All were treated with 6-8 cycles of ChlVPP chemotherapy regimen (Chlorambucil, Vinblastine, Procarbazine and prednisolone) and only 12 had radiotherapy. The overall survival (OS) at 5 and 10 years was 80.8% and 77.2%, respectively, whilst the event-free survival (EFS) at the same time intervals was 55.2% and 48.8% respectively. Twenty-eight patients (41.79%) relapsed/progressed, 18 (64%) survived after further chemotherapy with or without high-dose therapy and stem cell rescue. Twelve patients died, seven of HD, three from infections and one from secondary acute myeloblastic leukaemia (AML). Although the EFS in this study was lower than other studies, 64% of relapsed patients were salvaged with second-line therapy. It is also anticipated that survivors treated with this non-anthracycline-containing regimen will have less long-term toxicity.