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Sclerosing angiomatoid nodular transformation (SANT) of the spleen is a morphologically distinctive lesion. Although the clinical course of SANT is benign, its reactive or neoplastic nature remains to be clarified. Furthermore, some investigators have suggested that SANT is related to IgG4 sclerosing lesion or inflammatory pseudotumor with stromal cells positive for Epstein-Barr virus (EBV). In this study, we assessed 22 cases of SANT derived from adult women. Clinical data and follow-up information were obtained by chart review. Immunohistochemical studies for IgG4, IgG, and CD21 stains and in situ hybridization to detect EBV-encoded small RNAs were performed. We also assessed genomic DNA extracted from paraffin-embedded tissue for human androgen-receptor α gene analysis using conventional and methylation-specific polymerase chain reaction methods. The median patient age was 41.5 years (range, 25 to 82 y). Most (77%) patients presented with a single mass that was detected incidentally (59%). The mean size of the lesions was 3.8 cm (range, 1.0 to 9.0 cm). Clinical symptoms correlated with multiple lesions (P=0.043) but not lesional size (P=0.637) or location in the spleen (hilum vs. periphery, P=0.696). None of the cases had evidence of IgG4-related disease or recurred after splenectomy. The mean number of IgG4 cells was 27.7 (range, 4 to 125), and the mean IgG4/IgG ratio was 16.4% (range, 1.6% to 55.7%) with only 2 cases being >40%. Cases with higher IgG4 cells did not correlate with inflammatory pseudotumor-like morphology. No lesions were positive for EBV-encoded small RNAs, and almost all cases with informative results (n=19) showed a polyclonal pattern. We conclude that SANT is a polyclonal, reactive lesion rather than a neoplasm.

A 14-year-old boy developed progressive bilateral leg weakness for 5 months. Physical examinations showed calf muscle atrophy (figure, A) and hyperreflexia. Spinal lesion of slow progression was suspected.

A 1-day-old infant developed heart murmur with tachypnea. Echocardiography showed multiple biventricular cardiac rhabdomyomas (figure, A). Brain sonography showed multiple cerebral cortical tubers (figure, B) and subependymal nodules (figure, D and E), confirming tuberous sclerosis. Bilateral renal cysts were present (figure, C).

Background: For decades, peginterferon and ribavirin (PegIFN/RBV) have been the standard-of-care for chronic hepatitis C virus (CHC) infection. However, the actual cost-effectiveness of this therapy remains unclear. We purposed to explore the real-world cost effectiveness for subgroups of treatment-naïve CHC patients with PegIFN/RBV therapy in a large real-world cohort using a whole population database. Methods: A total of 1809 treatment-naïve chronic hepatitis C virus (HCV) patients (829 HCV genotype 1 [G1] and 980 HCV G2) treated with PegIFN/RBV therapies were linked to the National Health Insurance Research Database, covering the entire population of Taiwan from 1998 to 2013 to collect the total medical-care expenses of outpatient (antiviral agents, nonantiviral agents, laboratory, and consultation costs) and inpatient (medication, logistic, laboratory, and intervention costs) visits. The costs per treatment and the cost per sustained virological response (SVR) achieved were calculated. Results: The average medical-care cost was USD $4823 (±$2984) per treatment and $6105 (±$3778) per SVR achieved. With SVR rates of 68.6% and 87.8%, the cost/SVR was significantly higher in G1 than those in G2 patients, respectively ($8285 vs $4663, P

The case presented by Tsai et al.1 described a 62-year-old woman with an intraparenchymatous hemorrhage and an angiographic abnormality diagnosed as vein of Galen malformation. The angiographic image showed an early filling of a dilated vein of Galen and occlusion of straight sinus, but no dilation of choroidal vessels, which would be expected in a vein of Galen malformation. Instead, …

An 8-month-old boy with cutaneous vascular malformations and dermal melanocytosis (Mongolian spots, figure, A) on the face and trunk was diagnosed with phacomatosis pigmentovascularis type 2. He had normal neurodevelopment, but progressive macrocephaly (figure, B). Linear brain ultrasonography showed extensive venous angiomatosis in the prominent subarachnoid space (figure, C and D). MRI revealed cortical sulcal widening, prominent leptomeningeal vessels in an enlarged subarachnoid space (figure, E and F), and communicating hydrocephalus (figure, F). Neurologic involvement in phacomatosis pigmentovascularis is uncommon except in Sturge-Weber and Klippel-Trenaunay syndromes.(1,2) Communicating hydrocephalus due to subarachnoid angiomatosis may be underdiagnosed in phacomatosis pigmentovascularis, and should be considered in case of progressive macrocephaly.