The costs of avoidable injury from childhood cancer: Litigate or mediate?

We write as experienced paediatric practitioners who have been involved in medico-legal proceedings where cases related to childhood cancer practice have featured frequently. We will use the service evaluation of Professor David A Walker's last 35 cases, where all but seven concerned children with tumours of the brain or spine to illustrate the concerns that families raise. We refer to the evidence from the HeadSmart programme (www.headsmart.org.uk), which seeks to accelerate diagnosis by raising awareness of the disease and symptoms. We use the experience of Dr Jonathan AG Punt to illustrate the legal issues that apply and explain the way that significant quantum calculations are applied to cases of this type. The current move by NHS Resolution to explore the expanded role of mediation will be discussed and the need for research to explore the precise way that mediation could be developed to offer an alternative approach to conflict resolution.

Treatment of recurrent central nervous system primitive neuroectodermal tumours in children and adolescents: results of a Children's Cancer and Leukaemia Group study.

BACKGROUND: The treatment of previously irradiated patients with recurrent central nervous system primitive neuroectodermal tumours (PNETs) is a considerable challenge. A study was undertaken to attempt to improve the outcome for such patients using a high dose chemotherapy (HDCT) based strategy. METHODS: Between 2000 and 2007, 40 patients with relapsed medulloblastoma (MB) and 5 with relapsed supratentorial PNETs (StPNETs) were accrued. All but one had received prior craniospinal radiotherapy. Patients were initially treated with cyclophosphamide (4 g/m(2)) together with surgery or local radiotherapy where appropriate. If complete or near complete remission was achieved, the patient proceeded to receive two sequential courses of HDCT with stem cell rescue. The first course consisted of thiotepa (900 mg/m(2)) and the second carboplatin (AUC 21). RESULTS: All five patients with StPNET died of tumour progression with a median OS of 0.4 years. Nineteen of the 40 patients with relapsed MB underwent surgery. Radiotherapy was administered to eight patients. All patients received at least one course of cyclophosphamide. Only 22 MB patients progressed to the HDCT phase; 10 patients received thiotepa only and 12 thiotepa and carboplatin. At a median follow-up of 7.4 years (Range 2.8-8.2 years), only three MB patients are still alive, one following a further relapse. Three and 5 year OS was 22.0% and 8.2%, respectively and 3 and 5 year EFS was 14.6% and 8.7%, respectively. CONCLUSION: This national study based on a strategy including a particular tandem HDCT regimen showed no benefit for previously irradiated patients with relapsed StPNET and very limited benefit for patients with relapsed medulloblastoma.

Primary postoperative chemotherapy without radiotherapy for intracranial ependymoma in children: the UKCCSG/SIOP prospective study.

BACKGROUND: Over half of childhood intracranial ependymomas occur in children younger than 5 years. As an adjuvant treatment, radiotherapy can be effective, but has the potential to damage the child's developing nervous system at a crucial time-with a resultant reduction in IQ and cognitive impairment, endocrinopathy, and risk of second malignancy. We aimed to assess the role of a primary chemotherapy strategy in avoiding or delaying radiotherapy in children younger than 3 years with intracranial ependymoma. METHODS: Between December, 1992, and April, 2003, we enrolled 89 children with ependymoma who were aged 3 years or younger at diagnosis, of whom nine had metastatic disease on pre-operative imaging. After maximal surgical resection, children received alternating blocks of myelosuppressive and non-myelosuppressive chemotherapy every 14 days for an intended duration of 1 year. Radiotherapy was withheld unless local imaging (ie, from the child's treatment centre) showed progressive disease. FINDINGS: 50 of the 80 patients with non-metastatic disease progressed, 34 of whom were irradiated for progression. The 5-year cumulative incidence of freedom from radiotherapy for the 80 non-metastatic patients was 42% (95% CI 32-53). With a median follow-up of 6 years (range 1.5-11.3), overall survival for the non-metastatic patients at 3 years was 79.3% (95% CI 68.5-86.8) and at 5 years 63.4% (51.2-73.4). The corresponding values for event-free survival were 47.6% (36.2-58.1) and 41.8% (30.7-52.6). There was no significant difference in event-free or overall survival between complete and incomplete surgical resection, nor did survival differ according to histological grade, age at diagnosis, or site of disease. In 47 of 59 (80%) patients who progressed, relapse resulted from local control only. The median time to progression for the 59 patients who progressed was 1.6 years (range 0.1-10.2 years). The median age at irradiation of the whole group was 3.6 years (range 1.5-11.9). For the 80 non-metastatic patients, the 23 who achieved the highest relative dose intensity of chemotherapy had the highest post-chemotherapy 5-year overall survival of 76% (95% CI 46.6-91.2), compared with 52% (33.3-68.1) for the 32 patients who achieved the lowest relative dose intensity of chemotherapy. INTERPRETATION: This protocol avoided or delayed radiotherapy in a substantial proportion of children younger than 3 years without compromising survival. These results suggest, therefore, that primary chemotherapy strategies have an important role in the treatment of very young children with intracranial ependymoma.

Surgical management of paediatric stroke.

Childhood cerebrovascular disease is characterised by a wide range of relatively rare conditions. The management of a selection of some of the more frequently encountered, complex conditions is reviewed. The key to achieving the optimal therapeutic strategy for the individual child is multidisciplinary team management within a specialist neurovascular team. Access to rehabilitation is crucial.

Neuroendoscopic third ventriculostomy for failed shunts.

BACKGROUND: Neuroendoscopic third ventriculostomy has increased in frequency for the management of hydrocephalus. The objective of this paper is to study the outcome in patients with hydrocephalus whose shunt subsequently failed and who were treated with neuroendoscopic third ventriculostomy (NTV). METHOD: The departmental prospectively acquired database, kept since 1994, was researched to identify those patients who underwent NTV, having presented with a failed shunt. Subsequent failure of the NTV occurs when further treatment for the hydrocephalus is required. RESULTS: There were 88 patients identified, 45(51%) male and 43(49%) female. Median age at time of NTV was 14 years (range 1 day to 69 years). Median time from last shunt to NTV was 8 years (1 week to 35 years). Follow-up was for a median of 3 years (1 month to 6 years) after their NTV. Overall 42 (48%) failed and 46 (52%) were successful. In those with noncommunicating causes the success rate was 73%. Median time to failure was 1 month (immediate to 5 years) Median age of failed patients at time of NTV was 7 years. Serious complications occurred in 5 (5.6%). CONCLUSION: NTV in patients having previously been shunted for their hydrocephalus is safe and as successful as in primary NTV. Failure can be expected to occur with greater frequency in communicating than noncommunicating types of hydrocephalus. The fact that they have a malfunctioning shunt in situ is not a contraindication to this procedure. In cases of infected shunts it is a useful adjunct to the treatment of the infection.

Intradural cerebellar chordoma in a child: a case report and review of the literature.

CASE REPORT: The authors describe an unusual case of an intradural cerebellar chordoma, the first such case to be reported in a child. A 9-year-old girl presented with headaches and papilloedema, and a discrete cerebellar mass was resected, which was shown histologically to be chordoma. There was no bony or dural association, and after radiologically proven complete resection it was elected that the child be followed expectantly. DISCUSSION: The case is compared with the few previously reported intradural chordomas, and insights into the origins and management of such cases are discussed.