[Nevoid basal cell carcinoma syndrome]. / Naevoid Basalzellkarzinom-syndrom.

Nevoid Basal Cell Carcinoma Syndrome (NBCCS) is an autosomal-dominant disorder characterized by multiple basal cell carcinomas, jaw cysts, palmar/plantar pits, calcification of the falx cerebri, and spine and rib anomalies. The combination of clinical, imaging, and histological findings is helpful in identifying NBCCS patients. Imaging plays a crucial role in evaluation of these patients. We present a wide variety of clinical and radiological findings characteristic of this disease.

Wilms' tumor in adults: results of the Society of Pediatric Oncology (SIOP) 93-01/Society for Pediatric Oncology and Hematology (GPOH) Study.

PURPOSE: In the Society of Pediatric Oncology (SIOP) 93-01 study, 30 patients older than 16 years were found to have Wilms' tumor. They were treated according to the pediatric protocol and were analyzed for clinical presentation, stage distribution, and prognosis. PATIENTS AND METHODS: Patient age ranged from 16 to 62 years (median, 25.4 years). Tumor stages were defined according to SIOP, and treatment was risk-adapted according to SIOP 93-01/Society for Pediatric Oncology and Hematology (GPOH) protocol. The patients were evaluated with regard to response, toxicity, and prognosis. Specimens of all tumors were centrally reviewed. RESULTS: Ten patients (33%) had metastatic disease at the time of diagnosis (liver, four patients; lung, three patients; liver and lung, three patients). The local stage distribution showed a predominance of higher stages (stage I, eight patients; stage IIN-, three patients; stage IIN+, four patients; stage III, 15 patients). Histologic studies revealed intermediate-risk in 23 of 30 tumors; two tumors were classified as high-risk; and three tumors were clear-cell sarcomas. Two of 30 patients showed a nephroblastoma and a renal cell carcinoma simultaneously in the same kidney. A complete remission was achieved in 24 patients; four patients relapsed after complete remission; and three of them reached a second remission with further treatment. Event-free survival was 57%, with an overall survival of 83% (median observation time, 4 years). CONCLUSION: Adults can be cured in a high percentage by a multimodal treatment according to pediatric protocols. Toxicity is higher than in children, but acceptable in view of the high remission rate.

Inter- and intraindividual variability in ketamine dosage in repetitive invasive procedures in children with malignancies.

Midazolam/ketamine sedation has been used successfully in children undergoing painful invasive procedures. The authors prospectively assessed inter- and intra-individual variability in ketamine dosage for sedation in repetitive invasive procedures in children with malignancies. A total of 92 invasive procedures (58 lumbar punctures, 34 bone marrow biopsies; range: 2-9 procedures/patient) were performed on 25 children (median age: 12 years). Intravenous sedation consisted of 0.1 mg midazolam/kg and 0.5-1.0 mg ketamine/kg. Incremental dosages of ketamine (0.33 mg/kg) were given if necessary to achieve or maintain deep sedation. Primary outcome measure was the inter- and intraindividual ketamine dosage required to achieve adequate sedation; secondary outcome measures were the number of procedures with adequate sedation (Ramsay score of > 4), the number of adverse side effects, and the need for therapeutic interventions. All 92 invasive procedures were completed with satisfactory sedation levels in 88 procedures (95.7%). There was a great inter- and intraindividual variability in ketamine dosage required to achieve or maintain adequate sedation. In 12% of procedures side effects were seen, which required no or only minor interventions. Due to great inter- and intraindividual differences, ketamine dosage should be titrated toward the desired level of sedation. Thus, ketamine can be adjusted to the individual's need while achieving adequate sedation.

Sedation with midazolam and ketamine for invasive procedures in children with malignancies and hematological disorders: a prospective study with reference to the sympathomimetic properties of ketamine.

Different pharmacological agents have been used for sedation in children undergoing invasive procedures. The authors prospectively evaluated the efficacy, the occurrence of adverse side effects, and cardiovascular parameters in midazolam and ketamine sedation for invasive procedures in children with malignancies and hematological disorders. A total of 183 invasive procedures were performed on 63 children (mean age 9.2 +/- 5.2 years). Intravenous sedation consisted of 0.1 mg midazolam/kg and 1.0 mg ketamine/kg. Incremental dosages of ketamine (0.33 mg/kg) were given if necessary to maintain deep sedation. Systolic and diastolic blood pressure, heart rate, and oxygen saturation were recorded. All 183 invasive procedures were successfully completed with satisfactory sedation levels in 170 procedures (92.9%; 95% CI:88.2-96.2%). In 33 procedures (18%; 95% CI: 12.8-24.4%) sedation was associated with side effects, the most common being oxygen desaturation. One patient experienced a transient episode of laryngospasm. There was a significant increase in both systolic and diastolic blood pressure and heart rate after ketamine medication (p <.01). Procedure and recovery time were correlated to ketamine dosage (p <.01). The combination of midazolam and ketamine is efficacious in achieving deep sedation for painful invasive procedures. Considering the possibility of potentially serious respiratory complications it should be performed only by physicians who are trained in advanced airway management and life support. As opposed to many other sedative drugs with cardio-depressant properties, ketamine causes a rise in both systolic and diastolic blood pressure, and heart rate.

Ganglianeuroma: radiological and metabolic features in 4 children.

Ganglioneuroma constitutes a benign and surgically treatable tumor. The authors studied 4 patients with histopathologically proven ganglioneuroma focusing on radiological and metabolic features. The results confirm previous investigations that have shown metabolic activity in ganglioneuroma and characteristic patterns in imaging studies. Although for definite diagnosis tissue investigation is required, certain clinical and radiological features are suggestive of ganglioneuroma.