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This is the eighth official document of the SIOP Working Committee on Psychosocial Issues in Pediatric Oncology, instituted in 1991. It deals with a topic discussed and approved by the SIOP Committee; namely, "Recognition, prevention, and remediation of burnout in health care professionals participating in the care of children with cancer." It is addressed to the Pediatric Oncology community and outlines: 1) the general definition of burnout as mental and physical exhaustion, indifference, sense of failure as a professional, and sense of failure as a person; 2) the causes of burnout from the nature of the work itself, the work environment, and the characteristics of the individual; 3) the prevention of burnout, changing the detrimental aspects of one's work environment and modifying one's own behavior; and accepting methods to remediate burnout when it occurs., (Copyright 2000 Wiley-Liss, Inc.)

This is the seventh official document of the SIOP Working Committee on Psychosocial Issues in Pediatric Oncology, instituted in 1991. This document develops an additional topic discussed and approved by the SIOP Committee, "Assistance to siblings of children with cancer. " It is addressed to the pediatric oncology community and outlines general principles for helping siblings throughout phases of treatment: at diagnosis, during treatment, in the event of relapse, during bone marrow transplantation, after completion of therapy, during palliative care and the terminal phase., (Copyright 1999 Wiley-Liss, Inc.)

This, the sixth official document of the SIOP Working Committee on psychosocial issues in pediatric oncology, develops another important and especially difficult topic: assistance for terminally ill children with cancer. This is provided for the pediatric oncology community as a useful set of guidelines. It should be always possible for a declining child to die without unnecessary physical pain, fear, or anxiety. It is essential that he or she receive adequate medical, spiritual, and psychological support, and that the child at no point feels abandoned. Palliative care, in the terminal phase of cancer, should be tailored to the different needs and desires of the child and the family, with the goal of providing the best possible quality of life for the days that remain.

Childhood lymphoblastic leukemia (ALL) is usually assumed to have been permanently eradicated in patients in long-term remission, but occasionally can recur after many years. To learn more about the problem, we studied a group of children whose leukemia had been in remission for 10 or more years before relapse and tried to determine whether they had true recurrences or second malignancies. We studied children treated on Medical Research Council ALL protocols between 1970 and 1984 and followed up by the Clinical Trial Service Unit in Oxford. Detailed clinical and laboratory data was collected from the centers concerned on all who were reported to have had a recurrence of their leukemia after 10 or more years from the time of achieving first complete remission (CR1). To prove that the relapse was a true recurrence rather than a second or secondary leukemia, DNA extracted from archived marrow smears was subjected to polymerase chain reaction (PCR) analysis for the presence of an identical Ig heavy chain (IgH) or T-cell receptor (TCR) gene rearrangement at initial diagnosis and subsequent relapse. A total of 1,134 of 2,746 children had survived 10 years or more (range, 10 to 24 years) in CR1 and of those, 12 (approximately 1%) had subsequently relapsed. Relapse blast cells were shown to express the common ALL antigen (CD 10) in all cases and an identical clonal IgH or TCR gene rearrangement was found on PCR analysis of DNA from diagnosis and relapse in all eight cases where DNA extraction was successful. A further program of therapy was successful in inducing a second CR in all patients, four of whom have succumbed to a second relapse after 12 to 27 months. The remaining eight are in continuing CR2 at a follow-up of 12 to 108 months (median, 52) from relapse. Although the risk of relapse of childhood ALL after 10 years in remission appears to be small (around 1%), it persists. This raises questions about how blasts can survive quiescent for so long and when we can truly be confident of cure, if ever.

This, the fifth official document of the SIOP Working Committee on Psychosocial Issues in Pediatric Oncology, develops another important topic: the Therapeutic Alliance between families and staff. This is addressed to the Pediatric Oncology Community as Guidelines that could be followed. Every parent, medical staff member, and psychosocial professional involved in the care of the child should be responsible for cooperating in the child's best interest. Everyone must work together toward the common goal of curing the cancer and minimizing its medical and psychosocial side-effects.

This is the fourth official document of the SIOP Working Committee on psychosocial issues in pediatric oncology constituted in 1991. This document develops another topic discussed and approved by the SIOP Committee: "communication of the diagnosis" is addressed to the pediatric oncology community as guidelines that could be followed. The highly stressful nature of the diagnostic period must be acknowledged, and communication involving the staff and all family members should cover both medical and psychosocial issues. A well-planned and extensive initial session should be followed by continuing discussions. The goal is a knowledgeable family that can talk openly with its members and with the staff.

This is the third official document of the SIOP Working Committee on Psychosocial issues in pediatric oncology constituted in 1991. This document develops another topic already discussed and approved by the SIOP committee. The topic: "Care of long-term survivors" is addressed to the pediatric oncology community as guidelines that could be followed for considering this issue in a more appropriate way.

Little is known about Clostridium difficile infection in children with cancer but a presumed outbreak has previously been described. The carriage rate before admission to hospital and morbidity is reported to be high, especially in younger children. The prevalence of C difficile infection on a paediatric oncology ward was monitored from June 1991 to May 1992. Twenty eight (13%) of 214 children were found to be infected. Though the temporal distribution suggested an outbreak, polyacrylamide gel electrophoresis identified several different types. Unlike previous reports, infection appeared to be possibly endogenous or possibly environmental in origin rather than due to cross infection; the morbidity was low and age was not a determinant for infection. The duration of hospital stay and the primary diagnosis were found to be determinants for infections, those with lymphoid malignancies being at the highest risk. The diagnostic category at greatest risk were those most intensively treated, with protracted neutropenia and prolonged antibiotic exposure. Early identification of cases and prompt institution of simple control measures will prevent cross infection. It is therefore important that diarrhoea is not accepted as a normal symptom of cancer chemotherapy and stool specimens are sent for full bacteriological and viral investigation.

The haematological toxicity of the induction phase of chemotherapy for acute lymphoblastic leukaemia (ALL) was compared in two cohorts of patients. The principal difference between these two cohorts was the mode of administration of the anthracycline, daunorubicin (DNR). Both groups received four-drug induction chemotherapy, which produced a high remission rate. Those receiving DNR on days 1 and 2 experienced a profound but shorter period of neutropenia and more severe thrombocytopenia than those who received the DNR weekly. The pattern of hospitalisation and support facilities in the individual unit may determine which regimen is to be preferred. These observations are relevant for the newly diagnosed patient in whom an anthracycline is retained in the induction therapy for ALL.

Background: Several reports have been published of anemia due to infection by parvovirus in patients receiving chemotherapy for leukemia. However, parvovirus B19 has not been previously identified as a cause of chronic anemia in children with solid tumors., Methods: A child with rhabdomyosarcoma developed chronic anemia, with need for regular blood transfusions during chemotherapy. An infectious etiology was sought., Results: B19 viremia and an absent antibody response was present during the period of chronic anemia during chemotherapy. After completion of chemotherapy, the viremia cleared, IgM and IgG antibody to B19 virus were detected, and the anemia resolved., Conclusions: Parvovirus must be considered as a cause of anemia in patients undergoing chemotherapy for solid tumors as well as leukemia.

This review charts the evolution of therapy for childhood acute lymphoblastic leukaemia (ALL) in the United Kingdom. The present chemotherapeutic regimen is the result of experience gained from carefully planned randomised cooperative studies carried out during the last two decades. In common with the experience of the West German and American groups, the best results have been in those treated with post remission intensification blocks. With current chemotherapy protocols, almost 70% of children with ALL in U.K. can be cured but there may be a medical cost of such a cure, in terms of both acute and long term toxicity. This was especially true when central nervous system (CNS) therapy with cranial irradiation was used. Therefore present regimens are examining chemotherapeutic options for CNS disease control and the efficacy of additional post remission intensification. Failure of chemotherapy is most often seen in those children with a presenting white cell count of more than 50 x 10(9)/l, very young children and/or the presence of certain chromosomal rearrangements (e.g. t4: 11, t9: 22). At present the optimum therapeutic option for such high risk patients and for the majority of those in second remission, is an allogenic bone marrow transplant if an HLA-matched sibling is available. Modern day therapy is both complicated and costly and will be beyond the resources available for most children with ALL in developing countries. A significant decrease in worldwide mortality due to ALL will only occur if either the disease can be prevented or a simpler cure devised.

The duration of symptoms before diagnosis (lag time) was defined for 184 of 236 children diagnosed as having a malignancy at the Royal Hospital for Sick Children, Edinburgh for the time period January 1982 until December 1990. The natural logarithm of the lag time was correlated with age, gender, diagnostic group, white cell count in acute leukaemia, clinical stage of disease in solid tumours, and event free survival. Age was significantly associated with lag time, older children presenting later. In the diagnostic groups, mean lag time ranged from 2.8 weeks in nephroblastoma to 13.3 weeks for brain tumours. Diagnostic group was predictive for lag time after adjustment for age, with for example, a significantly longer lag time for those with brain tumours. However lag time was not predictive of event free survival and it is likely that lag time has other major determinants. When compared with previous studies, there also appears to be a regional variation in lag time for diagnostic groups. It seems likely that this is a reflection of geographical difference in the structure of health systems and is therefore yet another important determinant.

Currently intravenous ceftazidime with or without an aminoglycoside or alternatively ciprofloxacin are the recommended antibiotics of choice in Pseudomonas aeruginosa meningitis. A case of atraumatic, spontaneous Ps. aeruginosa meningitis in a child with acute lymphoblastic leukaemia is described. Despite the organism demonstrating in vitro sensitivity to ceftazidime, netilmicin and ciprofloxacin, intravenous therapy with these drugs failed to sterilise the cerebrospinal fluid (CSF). Both netilmicin and ciprofloxacin failed to attain therapeutic levels in the CSF. Intrathecal aminoglycoside therapy via an intraventricular reservoir was successful in eradicating the infection. In children with meningitis due to Ps. aeruginosa where intravenous therapy is unsuccessful despite in vitro sensitivity to recommended antibiotics; intraventricular medications should be commenced as soon as possible.

We report the experience of the European Neuroblastoma Study Group (ENSG) with central nervous system (CNS) involvement of neuroblastoma. Among this series of intensively treated patients, CNS neuroblastoma was diagnosed by computerised tomography (CT) scanning, rather than by autopsy. Cranial disease occurred in 5% of ENSG patients. Of 11 patients with intracranial disease, 4 had disease in the posterior fossa, a site rarely reported previously. Furthermore, 5 cases had CNS metastases at a time when there was no detectable disease elsewhere, rather than as part of extensive relapse. The pattern of disease we observed, at least for those with parenchymal disease, is in keeping with arterial spread. Although CT scanning is the optimal modality for identifying CNS disease, 2 cases had normal head CT scans prior to the onset of CNS disease. As most patients had symptoms of raised intracranial pressure (RICP) at the time the CNS disease was diagnosed, there does not seem to be any indication for routine CT scanning of the head at diagnosis, but this should be performed as soon as any symptoms or signs appear. With patients living longer with their disease, vigilance must be maintained during follow-up.

Germline mutations within a defined region of the p53 gene have recently been found in families with the Li-Fraumeni syndrome (LFS). In the present study this region of p53 was sequenced in affected individuals from 8 families with LFS. In only 2 of them were such mutations detected. Our findings suggest that the p53 mutation could be the primary lesion in some but not all families with LFS, and confirm that there is a "hot spot" for these mutations at the CpG dinucleotide moiety of codon 248. Assigning risks and counselling families on the basis of presence of p53 mutations should be approached with caution.