• Accurate staging is important for treatment planning and assessing prognosis • Whole-body MRI-DWI could be a good radiation-free alternative to FDG-PET/CT • Interobserver agreement of whole-body MRI-DWI is good • Agreement between whole-body MRI and the FDG-PET/CT reference standard is good • Most discrepancies were caused by suboptimal accuracy of size measurements on MRI.

A premature neonate had a catheter-associated bloodstream infection due to Staphylococcus lugdunensis. The MIC of oxacillin for the strain was >256 g/ml, and the mecA gene of S. lugdunensis was detected by PCR. The infection was resolved after removal of the line and treatment with vancomycin for 2 weeks. CASE REPORTA baby boy was delivered by caesarean section at 29 weeks of gestation with a birth weight of 980 g. Initial problems included grade III hyaline membrane disease, patent ductus arteriosus, and pulmonary hemorrhage. On day 8 of life, a long line was inserted into the left cubital fossa. On day 23, the baby developed sepsis. He had recurrent apnea and bradycardia requiring him to receive assisted ventilation. The level of Creactive protein was elevated, at 125.5 mg/liter. The total white cell count was 14 ϫ 10 9 /liter, and the immature neutrophil-tototal neutrophil ratio was 0.04. Thrombocytopenia (platelet count, 67 ϫ 10 9 /liter) was also present. Based on empirical evidence, the baby was started on cloxacillin and gentamicin. Two species of staphylococci were isolated from blood cultures taken before antibiotic administration. Both strains were resistant to cloxacillin. Treatment was changed to vancomycin after the baby had been on cloxacillin and gentamicin for 3 days. The long line was removed on the fifth day of infection, when one of the isolated organisms was confirmed to be Staphylococcus lugdunensis; the other isolate was reported to be a coagulasenegative staphylococcus. The culture of the long line tip also yielded S. lugdunensis. The chest X ray showed right upper lobe pneumonia. The results of a cranial ultrasound and a two-dimensional echocardiogram were normal. The baby showed good response to treatment with vancomycin. The level of C-reactive protein decreased to 7.3 mg/liter (on day 13 of vancomycin treatment), and the platelet count increased to 141 ϫ 10 9 /liter (on day 9 of vancomycin treatment). A repeat blood culture was still positive 48 h after vancomycin treatment was started but was negative by day 7. Vancomycin treatment was continued for a total of 2 weeks, with good clinical response. This is the first case report in the English-language literature of an infection due to an S. lugdunensis isolate carrying the mecA gene. Kawaguchi et al. detected mecA in one of two strains of S. lugdunensis in 1996 during a survey of staphylococcal isolates, but no description of clinical infection was given (5). As far as we are aware, this is also the first documentation of S. lugdunensis causing bloodstream infection in a neonate.As noted above, there were two isolates from the first blood culture. Both were characterized as staphylococci by Gram stain, colonial morphology, and a positive catalase reaction. The first isolate was a coagulase-negative staphylococcus with a typical negative reaction in both the latex agglutination test for clumping factor and protein A (BACTi Staph; Remel, Lenexa, Kans.) and the tube coagulase test with rabbit plasma. The second isolate had slightly yellow...

Children with hemophagocytic lymphohistiocytosis (HLH) are at an increased risk of critical illness. In this study, we described the clinical characteristics of critically ill children with HLH and identify factors associated with poor clinical outcomes. Children who were diagnosed with HLH with emergent admission to Children's Intensive Care Unit (CICU) between January 1, 2000 and October 31, 2015 were included. The primary outcome was CICU mortality. Over the 15-year period, there were 14 critically ill patients with HLH with 23 CICU admissions. Median age at HLH diagnosis was 8.2 years (interquartile range [IQR], 2.9 to 11.3). Overall CICU mortality was 8 of 23 CICU admissions (34.8%). Factors that were associated with CICU mortality in critically ill children with HLH identified in this study include: a worse median pediatric index of mortality 2 score (4.7% in survivors [IQR, 2.9% to 11.6%] vs. 2.4% [IQR, 1.2% to 4.3%]; P=0.031); higher median peak serum lactate level (mmol/L) within 24 hours of admission (5.6 [IQR, 2.7 to 17.4] vs. 1.6 [IQR, 1.2 to 2.8]; P=0.032); the need for mechanical ventilation (100% vs. 46.7%; P=0.019); inotropic support (87.5% vs. 20.0%; P=0.006); renal replacement therapy (50% vs. 0%; P=0.008); and blood product transfusion episodes (24.5 [IQR, 14.3 to 46.8] vs. 3.0 [IQR, 1.0 to 9.0]; P=0.002). Further studies are required to validate the factors that are associated with poor outcomes in critically ill children with HLH.

Assessment of cancer predisposition syndromes (CPS) in childhood tumours is challenging to paediatric oncologists due to inconsistent recognizable clinical phenotypes and family histories, especially in cohorts with unknown prevalence of germline mutations. Screening checklists were developed to facilitate CPS detection in paediatric patients; however, their clinical value have yet been validated. Our study aims to assess the utility of clinical screening checklists validated by genetic sequencing in an Asian cohort of childhood tumours. We evaluated 102 patients under age 18 years recruited over a period of 31 months. Patient records were reviewed against two published checklists and germline mutations in 100 cancer-associated genes were profiled through a combination of whole-exome sequencing and multiplex ligation-dependent probe amplification on blood-derived genomic DNA. Pathogenic germline mutations were identified in ten (10%) patients across six known cancer predisposition genes: TP53, DICER1, NF1, FH, SDHD and VHL. Fifty-four (53%) patients screened positive on both checklists, including all ten pathogenic germline carriers. TP53 was most frequently mutated, affecting five children with adrenocortical carcinoma, sarcomas and diffuse astrocytoma. Disparity in prevalence of germline mutations across tumour types suggested variable genetic susceptibility and implied potential contribution of novel susceptibility genes. Only five (50%) children with pathogenic germline mutations had a family history of cancer. We conclude that CPS screening checklists are adequately sensitive to detect at-risk children and are relevant for clinical application. In addition, our study showed that 10% of Asian paediatric solid tumours have a heritable component, consistent with other populations.

Pathological diagnosis of clear cell sarcoma of the kidney (CCSK) is challenging as it resembles blastemal Wilms tumor (WT) and other pediatric sarcomas, and does not have any distinctive immunophenotype. The YWHAE-FAM22 translocation t(10;17)(q22;p13) has been reported in a subset of CCSK. This translocation also occurs in high-grade endometrial sarcoma, in which it is associated with cyclin D1 overexpression. Hence we seek to determine YWHAE-FAM22 translocation status and cyclin D1 immunoreactivity in a series of local CCSK cases. Of 8 CCSK cases from 7 patients identified, no CCSK had the YWHAE-FAM22 fusion transcript by reverse transcriptase-polymerase chain reaction. Novel karyotypes were identified for 2 cases: 1 had t(2;13)(q13;q22) and the other t(3:17)(q29;p11.2). Excluding a case with poor tissue section antigenicity, 7 of 7 CCSKs (100%) showed diffuse and strong nuclear cyclin D1 staining. Cyclin D1 immunohistochemistry was also performed on tissue microarrays of other pediatric renal tumors: blastemal areas of 18 WT cases were negative; 6 rhabdoid tumors and 1 metanephric adenoma showed patchy and weak staining; 3 mesoblastic nephromas and 18 of 29 neuroblastomas had positive staining. Cyclin D1 immunohistochemistry helps distinguish CCSK from blastemal WT and metanephric adenoma and rhabdoid tumors, but not from neuroblastomas and mesoblastic nephromas. Cyclin D1 overexpression in CCSK is not contingent on YWHAE-FAM22 translocation, and cyclin D1 inhibition may potentially be explored as a targeted therapeutic strategy in CCSK.

The overall 5-year survival rate is comparable to international data in most developed countries. However, most patients presented with advanced disease, making the rate of globe preservation lower than in some developed countries. Better education of the public and healthcare professionals may increase awareness and enable early detection of the disease.

A 13-year-old boy underwent allogeneic hematopoietic stem cell transplantation (HSCT) for underlying acute lymphoblastic leukemia and achieved neutrophil engraftment 28 days after HSCT. He developed ichthyosis 6 weeks after HSCT and then keratotic follicular papules, palmoplantar keratoderma, and a seborrheic dermatitis-like eruption 18 weeks after HSCT. From skin biopsies he was diagnosed with eczematoid graft-versus host disease (GVHD), which showed spongiosis with scattered necrotic keratinocytes. He responded to oral and topical steroids and an increase in cyclosporine dose. Although uncommon, eczematoid GVHD must be considered in children who have undergone HSCT and then develop an atypical eczematous eruption, especially in the absence of a history of atopy.

No abstract