Activating mutations in RAS and receptor tyrosine kinases such as KIT and FLT3 are hypothesized to cooperate with chimeric transcription factors in the pathogenesis of acute myeloid leukemia (AML). To test this hypothesis, we genotyped 150 pediatric AML samples for mutations in KIT (exons 8, 17), NRAS and KRAS (exons 1, 2) and FLT3/ITD. This is the largest cohort of pediatric AML patients reported thus far screened for all four mutations. Of the children with AML, 40% had a mutation in KIT (11.3%), RAS (18%) or FLT3/ITD (11.1%), and 70% of cases of core-binding factor (CBF) leukemia were associated with a mutation of KIT or RAS. Mutations in RAS or FLT3/ITD were frequently found in association with a normal karyotype. Patients with a FLT3/ITD mutation had a significantly worse clinical outcome. However, the presence of a KIT or RAS mutation did not significantly influence clinical outcome. We demonstrate that KIT exon 8 mutations result in constitutive ligand-independent kinase activation that can be inhibited by clinically relevant concentrations of imatinib. Our results demonstrate that abnormalities of signal transduction pathways are frequent in pediatric AML. Future clinical studies are needed to determine whether selective targeting of these abnormalities will improve treatment results.

These data do not support the prognostic utility of PET-CT in pretreatment and interim treatment settings. A positive PET-CT after the completion of therapy identifies a patient subset with an inferior PFS and a trend toward inferior OS.

We examined 29 patients with intra-and extrahepatic masses and 10 normal volunteers with duplex and color Doppler ultrasonography. Portal blood flow adjacent to the masses was categorized as anterograde, retrograde, or nondetectable and was correlated with lesion size, character, and location.

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Serous borderline tumor (SBT) of the testis is a rare, ovarian epithelial-type tumor of the testis. We present a case of SBT after radical orchiectomy in a 59-year-old man who had 3-month progressive enlargement of his right hemi-scrotum, negative testicular tumor markers and scrotal ultrasound equivocal for malignancy. Magnetic resonance imaging (MRI) of the pelvis was obtained to aid with evaluation and showed marked hyperintensity on T2-weighted images with frond-like nodular peripheral enhancement within right testis mass. These distinct MRI findings can help clinicians differentiate SBT from other testis tumors. Relapse has not been observed in 8 years of follow-up.

Purpose:Preservation of renal parenchyma is a major goal when performing a partial nephrectomy. IRIS anatomical visualization software generates a segmented 3D model, allowing improved visualization of the tumor and surrounding structures. We hypothesize that using IRIS intraoperatively during partial nephrectomy on complex tumors increases the precision of surgical procedures and therefore may result in more tissue preservation.Methods:We identified 74 non-IRIS and 19 IRIS patients who underwent partial nephrectomy, with nephrometry scores of 9, 10, and 11. Propensity scores were used to match 18 pairs of patients on nephrometry score, age, and tumor volume. Pre- and postoperative imaging (MRI/CT) was obtained. Volumes of the preoperative tumor and preoperative whole kidney were obtained to calculate predicted postoperative whole kidney volume and then compared to actual postoperative whole kidney volume.Results:Mean differences between predicted and actual postoperative whole kidney volumes were 19.2 cm3 (SD=20.2) and 32 cm3 (SD=16.1, P = .0074) for IRIS and non-IRIS groups, respectively. The mean improvement in precision for the IRIS procedure was 12.8 cm3 (95% confidence interval, 2.5 to Inf; P = .02). There was no significant change in mean glomerular filtration rate from baseline to 6 months postoperatively between IRIS and non-IRIS groups (−6.39, SD=15.8 vs −9.54, SD=13.3; P = .5). No significant differences in complication rates (0 vs 1, P = .2), worsening glomerular filtration rate staging (5 vs 4, P = 1), and >25% decrease in glomerular filtration rate (3 vs 4, P = 1) were found between IRIS and non-IRIS groups.Conclusions:We demonstrated that using IRIS intraoperatively when performing partial nephrectomy on complex tumors is associated with improved surgical precision.

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