Progressive conduction disorders are noted during long-term follow-up in Fallot patients who received transannular patch but also in those who received no patch or a pulmonary homograft. It suggests that volume overloading related to the transannular patch but also pressure overloading and myocardial injury related to surgery contribute to their development.
We report the clinical history of 2 female patients with Ewing sarcoma and microscopic ovarian infiltration. In both cases, the initial workup found no metastasis. However, the examination of cryopreserved ovarian tissues revealed the presence of CD99 positive tumor cells with rearrangement of EWS gene confirmed by FISH. Both children were treated as patients with localized tumor and are currently in remission. These reports underline that, in Ewing sarcoma patients, retransplantation of cryopreserved ovarian tissue is not harmless and could lead to cancer relapse. These observations question also on the significance of ovarian dissemination on Ewing sarcoma prognosis and therapy.
Background
As dermatofibrosarcoma protuberans (DFSP) are rare with no prospective series within paediatric sarcoma trials, the European Paediatric Soft Tissue Sarcoma Study Group (EpSSG) examined the clinical data and outcomes of DFSP enrolled in a multinational study of non‐rhabdomyosarcoma soft tissue sarcomas (NRSTS).
Patients and methods
Forty‐six patients with confirmed DFSP were enrolled into the EpSSG NRSTS 2005 study. All had surgical resection and none had any further therapy at diagnosis.
Results
The median age at diagnosis was 6.9 years (range 0.4‐17.5). All patients had localised disease, and the majority had small <5 cm tumours (93%), and 76% had Intergroup Rhabdomyosarcoma Study (IRS) I tumours. All patients had up front surgery, 32 requiring two operations. There were 11 patients with IRS II tumours, of which only two went on to have a local recurrence. After a median follow up of 49.0 months (range 4.2‐130.9), all patients were alive at the time of this report, with 5‐year event‐free survival of 92.6% (CI 78.8‐97.6) with a 100% overall survival.
Conclusion
This report demonstrates the ability to run prospective paediatric studies in NRSTS in multiple European countries, with reasonable numbers of DFSP patients, with few events and no deaths, and hence excellent outcomes.
IntroductionDiffuse intrinsic pontine glioma is a rare disease with a high mortality. Our primary aim was to determine the incidence of this disease in Belgium. Secondly, we wanted to compare the treatment approach of Belgian pediatric oncology centres, to investigate possibilities for improvement.
MethodsWe retrospectively collected and analysed data on DIPG-patients diagnosed between 1994 and 2018 and recorded in the Belgian Cancer Registry.We included patients ≤ 18 years who were followed in one of the eight Belgian pediatric oncology centres.
ResultsWe included 100 patients over a period of 25 years with a median age at diagnosis of 7 years. We observed an increase in diagnoses with an incidence of 3.1 per 1,000,000 persons (aged 0-≤18) per year over the last 5 years compared to an overall incidence of 1.8. Forty-ve patients (51.7%) were biopsied at diagnosis. In ten (22%), this was study-related. H3 K27M-mutation was present in 75% of biopsied patients. Fifty-one patients (59.3%) received chemotherapy, without a signi cant survival bene t. Eleven patients (21.2%) were included in a clinical trial. Biopsy rate and the use of chemotherapy differed widely between centres. Mean OS and PFS were 10.49 and 4.87 months respectively. We observed an improved survival over time.
ConclusionsOver the past 25 years, we observed an increase of new DIPG-diagnoses. Outcome in our cohort is comparable with literature ndings. We demonstrate an important heterogeneity in treatment approach between different centres and limited inclusion in clinical trials. Therefore, collaboration between centres and inclusion of patients in clinical trials is much needed.
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