memo - Magazine of European Medical Oncology

Kostron, H.

2012 memo - Magazine of European Medical Oncology

doi: 10.1007/s12254-011-0320-1

Slavc, I.

2012 memo - Magazine of European Medical Oncology

doi: 10.1007/s12254-011-0307-y

Brain tumours in children differ from brain tumours in adult patients with regard to epidemiology, localisation, clinical presentation, growth pattern, histology/molecular biology, therapy and follow-up care. CNS tumours are the most common type of solid tumour in children, accounting for 25% of all childhood neoplasms and surpassed only by leukemias. CNS tumours are the leading cause of cancer related deaths in children, accounting for 32% of all cancer deaths compared to 2% in adults. CNS tumours of childhood are very heterogeneous and therefore up to 90% of children with CNS tumours are treated according to international multicentre treatment protocols. The most frequent histology in children is low-grade glioma accounting for approximately 40% of all tumours, followed by the group of embryonal tumours including medulloblastoma, CNS primitive neuroectodermal tumour (CNS PNET), pineoblastoma and ATRT (18%). As 5- and 10-year survival rates of children with CNS tumours have increased, so has the concern over late effects of treatment. Due to the immaturity of the brain of children many long-term survivors have cognitive, endocrine, and neurologic deficits that lead to social handicaps and reduced quality of life.

Woehrer, A.

2012 memo - Magazine of European Medical Oncology

doi: 10.1007/s12254-011-0308-x

In childhood, brain tumours are the second most common group of neoplasms following leukemias, but constitute the most common cause of cancer-related death. The neuropathological spectrum of paediatric brain tumours differs markedly from that of adults (higher prevalence of embryonic brain tumours and low-grade gliomas). Recent scientific advances have helped to better characterise the heterogeneous groups of entities. Novel morphological characteristics have been described (e.g. ETANTR) and further insights into their molecular genetic basis have been gained. The latter has led to the identification of molecular markers, which are increasingly recognised in surgical neuropathology, as they contribute to diagnostic accuracy, provide prognostic information (e.g. IDH1 mutation) and are used for patient stratification. These new molecular insights are already having influenced therapeutic approaches and in the near future might constitute the most important guidelines for therapeutic decision making.

Dieckmann, K.U.

2012 memo - Magazine of European Medical Oncology

doi: 10.1007/s12254-011-0309-9

Brain tumours account for 25% of all childhood neoplasms and are the most frequent solid tumour in children and treated according to multidisciplinary treatment concepts with surgery and depending on tumour type also chemotherapy and radiotherapy. Developing brain tissue in younger children is more sensitive to high energy x-rays and long-term side effects are more pronounced; therefore, radiotherapy has to be planned and applied very carefully to reduce the risk of long term side effects such as neurocognitive-, endocrine-, and neurological deficits. 3-D planning with individually adopted beams guarantees an individual configuration of dose plans, intensity modulated radiotherapy (IMRT) allows dose painting with higher doses to the more active parts of the tumour diagnosed on proton emission tomography (PET-CT; PET-MRI). The development of modern techniques and energies is evolving at a steady pace. Proton therapy carries a lower risk of radiation-induced morbidity and in the nearer future an increasing number of children will benefit from proton beam irradiation.

Meister, B.

2012 memo - Magazine of European Medical Oncology

doi: 10.1007/s12254-011-0310-3

Low-grade glioma is the most common form of childhood brain tumour, comprising nearly 40% of all primary central nervous system tumours diagnosed in patients under 18 years of age. The role of surgery is limited with no affect on overall survival and its goal is to avoid shunt replacement in the case of obstructive hydrocephalus and to postpone radiation. Present recommendations are that patients less than 8 years of age be treated with chemotherapy as first line treatment, avoiding long-term cognitive and neuroendocrine sequelae seen with surgery and radiation therapy. A variety of different drug regimens have shown efficacy in patients with both newly diagnosed, progressive disease and those with recurrent lesions.

Azizi, A.A.

2012 memo - Magazine of European Medical Oncology

doi: 10.1007/s12254-011-0311-2

Over the last few decades, the paediatric neuro-oncologic community has gained a more comprehensive insight into paediatric low grade glioma. 10–20% of childhoods LGG associated with inherited disorders, mainly neurofibromatosis type 1 (NF1). The localisations of childhoods LGG are in decreasing order in the posterior fossa, the supratentorial midline (optic nerves, chiasm and hypothalamus), the cerebral hemispheres and the spinal cord. In many paediatric patients LGG is a disease curable by neurosurgical removal only. Survival data are favourable with a five year overall survival (OS) of 94.6% and progression free survival (PFS) of 69.4%. However, an important number of patients needs further chemotherapy or (in respect of patient age) radiotherapy. Treatment decisions will have to be made by an expert team of paediatric neuro-oncologists, neurosurgeons and radiation oncologists as part of international treatment protocols. The recent advances in biological studies on these tumours with identification of specific molecular changes may completely change our treatment approach in the future.

Benesch, M.

2012 memo - Magazine of European Medical Oncology

doi: 10.1007/s12254-011-0312-1

Risk-adapted treatment stratification is a major characteristic of therapy optimisation studies for the treatment of benign and malignant childhood brain tumours. Thus far a patient having unfavourable risk factors ultimately requires a more intensive treatment in contrast to a patient with a more favourable risk profile. Several neuro-oncological therapy optimisation studies for children with brain tumours were recently initiated for patients with intracranial germ-cell tumours, with relapsed or refractory high-grade gliomas and for standard-risk or low-risk medulloblastoma to include biological parameters. Since our knowledge about the molecular biology of brain tumours is rapidly growing, molecular-based risk stratification will be used also for other brain tumour subtypes in the near future.

Peyrl, A.; Chocholous, M.; Kieran, M.W.; Azizi, A.A.; Prucker, C.; Czech, T.; Dieckmann, K.; Schmook, M.; Haberler, C.; Leiss, U.; Slavc, I.

2012 memo - Magazine of European Medical Oncology

doi: 10.1007/s12254-011-0313-0

Children with relapsed malignant brain tumours have a poor prognosis despite intensive treatment including high-dose chemotherapy with stem cell rescue [1, 2]. Angiogenesis, the formation of new blood vessels, is an important component of normal physiological processes such as wound healing and development. Moreover, tumour growth and metastasis is closely related to formation of new blood vessels [3, 4]. Antiangiogenic therapy inhibits neovascularization, thereby inhibiting tumour progression indirectly [5]. The term metronomic chemotherapy refers to the chronic administration of chemotherapeutic agents at relatively low, minimally toxic doses, and without prolonged drug-free breaks. Beside the antiangiogenic effect, metronomic chemotherapy has been shown to modulate anti-tumoural immunity and has the ability to induce tumour dormancy [6]. The combination of agents used in our metronomic antiangiogenic therapy is based on a four-drug regimen (thalidomide, celecoxib, etoposide and cyclophosphamide) published by Kieran et al. in 2005 [7]. We augmented this regimen with bevacizumab, fenofibrate, and intrathecal therapy with the goal to improve progression-free survival for patients with recurrent or refractory CNS tumours for whom no curative therapy is available. We report on our experience with an antiangiogenic metronomic chemotherapy for paediatric patients with recurrent CNS tumours of different histologies.

Leiss, U.

2012 memo - Magazine of European Medical Oncology

doi: 10.1007/s12254-011-0314-z

Learning abilities and memory or attentional skills are still underestimated as treatment sequel in case for children and adolescents with brain tumours. As survival rates in pediatric neuro-oncology have risen over the past decades, tumour- and treatment related neurocognitive late effects remain problematic for survivors of pediatric brain tumours. Estimates of neurocognitive late effects range widely, as rates have been documented in 40 to 100% of survivors. Based on clinical experience and observations, (ethical) guiding principles concerning the way neuropsychological assessment is carried out were developed.

Buchroithner, J.; Nußbaumer, K.; Pichler, J.; Weis, S.

2012 memo - Magazine of European Medical Oncology

doi: 10.1007/s12254-011-0315-y

We present the case of a young woman who suffered from acute lymphatic leukaemia when she was 8 years old. She was treated with bone marrow transplantation and intrathecal chemotherapy. The girl recovered and was considered disease-free. The Ommaya-reservoir and catheter to the ventricle in the right frontal lobe were left in situ. Twenty years later, a tumour was diagnosed that was located around the catheter. Histology disclosed an anaplastic oligo-astrocytoma WHO III.

Nowosielski, M.; Kuppelwieser, B.; Hutterer, M.; Stockhammer, G.

2012 memo - Magazine of European Medical Oncology

doi: 10.1007/s12254-011-0316-x

Ependymomas are slow growing tumours emerging from cells lining the cerebral ventricles or the central canal of the spinal cord and constitute ~9–10% of all primary central nervous system tumours. Large variations exist in the proportion of patients with metastatic disease and the exact incidence of leptomeningeal dissemination is still unknown [1, 2]. Here, we present an unusual case of a WHO grade II ependymoma with leptomeningeal as well as extraneural metastases.

Freyschlag, C.F.; Kostron, H.; Thomé, C.; Seiz, M.

2012 memo - Magazine of European Medical Oncology

doi: 10.1007/s12254-011-0317-9

Rare entities always have to be kept in mind as a differential diagnosis in brain tumours of adults. Pilocytic astrocytomas are benign neoplasms which preponderantly occur in children and adolescents. They are a subtype of astrocytomas which are distinctly classified as WHO Grade I lesions [1]. These tumours commonly arise around the third ventricle and the cerebellar vermis. Therapy of choice is usually surgical resection with curative intention. If complete surgical removal is impossible there is no existing standardised adjuvant protocol published to date. Among the different adjuvant therapeutic strategies radiotherapy is the most common.