Hypothalamic tumor involvement and familial disposition for obesity are risk factors for the development of severe obesity in patients with craniopharyngioma. As weight gain starts early after diagnosis and severe obesity causes a reduction in QoL, early therapeutic efforts should be considered in patients at risk. To confirm our results the prospective multicenter study Kraniopharyngeom 2000 on children and adolescents with craniopharyngioma was initiated (www.kraniopharyngeom.com).

The prognosis of mediastinal teratoma is excellent after complete or microscopically incomplete resection. In children with malignant GCT, the prognosis is favorable with a therapeutic strategy of delayed resection after preoperative chemotherapy. In most children, the diagnosis can be based on elevated tumor markers and imaging. Biopsy is indicated in nonsecreting GCT.

A relationship exists between tumours and malformations both generally and in particular combinations. This is also valid for minor errors of morphogenesis suggesting that embryonic tumours are an expression of aberrant intra-uterine morphogenesis. We speculated that these minor aberrations might also manifest in other morphological defects, especially in minor anomalies and malformations of the ribs. We reviewed chest roentgenographs of 1000 children with malignancies for rib anomalies and compared them to 200 patients with mainly infectious diseases. We found 242 rib anomalies in 218 children with tumours (21.8%) and 11 (5.5%) in children without malignancy. This difference was statistically highly significant (P less than 0.001). A high incidence of cervical ribs was found in neuroblastoma (33%), brain tumour (27.4%), leukaemia (26.8%), soft tissue sarcoma (24.5%), Wilms tumour (23.5%) and Ewing sarcoma (17.1%). Only neuroblastoma showed a high incidence of rib bifurcation (4.5%). The increased incidence of these mesenchymal defects in children with malignancies may be another clue for an altered morphogenesis in tumour origin. In neuroblastoma the rib anomaly may be another expression of neurocristopathy as proposed for the association of congenital heart disease and neuroblastoma.

The incidence of shunt infections and possible risk factors was investigated by chart analysis. From 1986 to 1989 350 shunt procedures were performed including 273 ventriculoperitoneal shunts and 75 ventriculoatrial shunts. Twenty-eight infectious episodes (8%) occurred in 25 patients during a median follow-up time of 20 months. For 204 patients the follow-up time could be prolonged until September 1992. In these patients no infectious episodes occurred in the extended observation period. In 24 cases (85.7%) a causative organism could be isolated. The infecting organisms were gram-positive cocci in 22 cases (78.6%) and gram-negative bacilli in two cases. The main signs and symptoms were fever, shunt malfunction and meningeal irritation, and with VP-shunts only, abdominal pain. Twenty-four infectious episodes were treated with antibiotics and immediate removal of the shunt. The remaining were managed with antibiotics only. The risk for shunt infection did not correlate with age or sex of patients, nor with the etiology of hydrocephalus, type of shunt implanted or perioperative antibiotic prophylaxis. However, a trend showing a higher risk for shunt infections with prolonged operation time was noticed. The infection rate was 13.6% for an operation lasting more than 90 minutes versus 5.2% for procedures of less than 30 minutes duration.

Purpose:To look for the presence and age-dependence of late structural alterations of otherwise normal-appearing cerebral gray and white matter after radiation and chemotherapy in adult survivors of acute lymphoblastic leukemia (ALL) during childhood. Materials and Methods:In a group of 13 adult survivors 17-37 years old, who had been treated by total brain radiation (18 -24 Gy) and chemotherapy 16 -28 years ago, prospective MR examinations including diffusion tensor imaging (DTI) were performed. Evaluation included volumetry, calculation of mean diffusivity (MD) and fractional anisotropy (FA), and comparison of results to an age-matched control group.Results: DTI showed significantly reduced FA values in the temporal lobes (difference of 0.069 units, P Ͻ 0.001), hippocampi (difference of 0.033 units, P Ͻ 0.001), and thalami (difference of 0.046 units, P ϭ 0.001), which were accompanied by significant white matter volume loss (difference of 92 cm 3 , P Ͻ 0.001). Significant elevations of MD were limited to the temporal white matter (difference of 42 ϫ 10 Ϫ6 mm 2 /s, P ϭ 0.005). Global and frontal white matter MD correlated negatively to increasing age of the survivors (P Ͻ 0.01). Conclusion:With regard to structural white matter alterations, adult long-term survivors of childhood ALL, who had received total brain radiation and chemotherapy, apparently show the same overall age dependence as controls. Follow-up studies are needed for confirmation.

The German cooperative study group for childhood acute lymphoblastic leukemia (COALL-92) was designed to examine the clinical effectiveness of thioguanine (TG) versus mercaptopurine (MP) in maintenance treatment of childhood acute lymphoblastic leukemia (ALL) in a randomized multicenter trial. TG and MP are prodrugs and have to be converted intracellularly to 6-thioguanine nucleotides (TGNs) for cytostatic activity. TG is converted into TGN in fewer steps and has been shown to be more cytotoxic in equimolar doses in vitro compared with 6-MP. Therefore, a higher effectiveness of TG in maintenance treatment was postulated. Of 521 patients enrolled into the protocol, 474 were randomized to receive either MP or TG during maintenance therapy in a daily oral dose. After a median observation time of 6.6 years, the probability of event-free survival was 79% ؎ 3% for the MP group (238 children) and 78% ؎ 3% in the TG group (236 patients). In spite of TGN levels, exceeding those of the MP group 7 times, treatment with TG did not improve the outcome but was more complicated to handle due to a specific toxicity profile of prolonged myelosuppression with marked thrombocytopenia.

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Modern treatment of childhood acute lymphoblastic leukaemia (ALL) has dramatically improved the prognosis for children with this disease. Therapeutic approaches consist of multimodal chemotherapy and radiotherapy with significant long-term side-effects. We report on 4 children out of a group of 120 newly diagnosed patients with ALL, who survived the disease for more than 2 years and developed a cerebral haemorrhage after chemotherapy and fractionated cranial irradiation. Following a period of 2-12 years the four children presented with acute neurological signs and symptoms. i.e. seizures, ataxia and hemiparesis. CT and MRI revealed intracerebral mass lesions, interpreted as haemorrhage. After neurosurgery the patients neurological state improved. Histological examination confirmed the suspected diagnosis of bleeding cavernous haemangioma or capillary telangiectases. There are two possibilities to explain these rare alterations: they may be pre-existent to the disease and therapy or they may be caused by irradiation. CONCLUSION Acute neurological symptoms in patients treated for ALL may be caused by spontaneous cerebral haemorrhaging of cavernous haemangiomas or capillary telangiectases induced by chemotherapy and/or radiotherapy.