Serotonin release during percutaneous radiofrequency ablation in a patient with symptomatic liver metastases of a neuroendocrine tumor.

In a patient with symptomatic liver metastases of a neuroendocrine tumor larger than 10 cm in diameter percutaneous radiofrequency ablation was performed. The ablation resulted in a significant decrease in tumor size and a good long-term improvement of symptoms. Plasma serotonin 48 hours after the ablation was approximately 10-fold lower than before. However, sequential determination of plasma serotonin during the radiofrequency ablation revealed a two-fold increase of plasma serotonin induced by the procedure. There was also an approximately three-fold increase of 5-hydroxyindol acetic acid in urine in the 24 hours following the ablation. The data show that ablation procedures in neuroendocrine tumors may induce hormone release which may be critical in patients with severe clinical symptoms.

Superior effects of high-dose enzyme replacement therapy in type 1 Gaucher disease on bone marrow involvement and chitotriosidase levels: a 2-center retrospective analysis.

Dosing of enzyme replacement therapy (ERT) for Gaucher disease type 1 is still a subject of debate and varies from 15 to 130 U/kg/mo, making a huge economic difference of 70,000 US dollars to 380,000 US dollars(euro55,000-300,000) per patient per year. To investigate whether this difference in dosing ultimately translates into a different response, we retrospectively compared long-term outcome of ERT at 2 large European treatment centers, Academic Medical Center, Amsterdam, The Netherlands (n = 49, median dose, 15-30 U/kg/4 wks) and Heinrich-Heine University, Duesseldorf, Germany (n = 57, median dose, 80 U/kg/4 wks). These adult cohorts had a similar genetic background. All follow-up parameters were matched separately at baseline, to avoid bias with respect to disease severity. Improvement in hemoglobin, platelet count, and hepatosplenomegaly was not significantly different between both cohorts, whereas plasma chitotriosidase and bone marrow involvement by magnetic resonance imaging improved more quickly and was more pronounced in the higher-dosed group. Major bone complications rarely occurred in both groups. In conclusion, different dosing regimens of ERT do not affect outcome of hematologic and visceral parameters, but higher dosing leads to accelerated decrease of chitotriosidase and better objective bone response in adult type 1 Gaucher disease.