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Teratoid Wilms' tumor is a rare variant of Wilms' tumor (WT) that has been reported exclusively in pediatric patients. The present paper describes a teratoid WT in a 50-year-old Korean man with a giant right renal mass. Radical nephrectomy was performed under the impression that the mass was a renal cell carcinoma. Grossly, the removed kidney contained a giant well-encapsulated mass measuring 24 × 18 × 10 cm with cystic changes, necrosis, and hemorrhage. On microscopy triphasic patterns of WT and prominent heterologous components were seen, including rhabdomyoblasts, neuroepithelial tissue, fat, cartilage, and various types of mature epithelium. This is the first reported case of a teratoid WT occurring in an adult kidney. [ABSTRACT FROM AUTHOR]

The article focuses on the study on the prognostic significance of c-Met protein expression in glioblastomas. The study determines whether expression of c-Met protein in glioblastomas was linked with overall survival and biologic components representing tumor invasiveness in patients with glioblastomas. An analysis of glioblastomas and the association between c-Met expression and clinicobiologic features showing tumor invasiveness is accounted.

Intracerebral granular cell tumors (GCTs) are a rare finding. We report here on a case of cerebral GCT in a 47-year-old man who suffered with severe headache. The tumor appeared as a relatively well-defined, enhancing mass at the periventricular white matter of the left occipital lobe of the brain. Histologically, the tumor was entirely composed of granular cells. Some of the tumor cells showed peripherally accentuated cytoplasmic granules with central clearing, which produced a unique “targetoid” appearance. The granular cells of the current case were positive for neuron-specific enolase (NSE), S-100 protein, GFAP, vimentin, CD 68, lysozyme, and α-1-antitrypsin. These wide immunoexpressions were not observed for the previously reported cerebral GCTs. Interestingly, this case showed “targetoid” or “reversed targetoid” immunoreactive patterns in NSE, CD68, GFAP, and vimentin. Despite these wide immunoexpressions and the lack of any association with astrocytoma, the histogenesis of cerebral GCTs is still suggested to be of a glial origin, based on the tumor location, the GFAP positivity and the ultrastructural findings, of which the latter showed intermediate filaments, and these are reminiscent of neoplastic astrocytes. [ABSTRACT FROM AUTHOR]

Human mesenchymal stem cells (hMSCs) can be readily isolated from bone marrow and differentiate into multiple tissues, making them a promising target for future cell and gene therapy applications. The low frequency of hMSCs in bone marrow necessitates their isolation and expansion in vitro prior to clinical use, but due to senescence-associated growth arrest during culture, limited cell numbers can be generated. The lifespan of hMSCs has been extended by ectopic expression of human telomerase reverse transcriptase (hTERT) using retroviral vectors. Since malignant transformation was observed in hMSCs and retroviral vectors cause insertional mutagenesis, we ectopically expressed hTERT using lentiviral gene transfer. Single-cell-derived hMSC clones expressing hTERT did not show malignant transformation in vitro and in vivo after extended culture periods. There were no changes observed in the expression of tumour suppressor genes and karyotype. Cultured hMSCs lack telomerase activity, but it was significantly increased by ectopic expression of hTERT. HTERT expression prevented hMSC senescence and the cells showed significantly higher and unlimited proliferation capacity. Even after an extended culture period, hMSCs expressing hTERT preserved their stem cells character as shown by osteogenic, adipogenic and chondrogenic differentiation. In summary, extending the lifespan of human mesenchymal stem cells by ectopic expression of hTERT using lentiviral gene transfer may be an attractive and safe way to generate appropriate cell numbers for cell and gene therapy applications. [ABSTRACT FROM AUTHOR]

There is a difference in the susceptibility to inflammation between the umbilical vein (UV) and the umbilical arteries (UAs). This led us to hypothesize that there is an intrinsic difference in the pro-inflammatory response between UA and UV. Real-time quantitative RT-PCR and microarray analysis revealed higher expression of interleukin (IL)-1β and IL-8 mRNA in the UV and differential expression of 567 genes between the UA and UV associated with distinct biological processes, including the immune response. Differential expression of human leukocyte antigen (HLA)-DRA mRNA between the UA and UV was due to unexpected HLA-DR+ cells migrating via the umbilical vessels into Wharton's jelly, more frequently in the UV. A significant proportion of these cells co-expressed CD45 and type I pro-collagen, and acquired CD163 or α-smooth muscle actin immunoreactivity in Wharton's jelly. Migrating cells were also found in the chorionic and stem villous vessels. Furthermore, the extent of migration increased with progression of gestation, but diminished in intrauterine growth restriction (IUGR). The observations herein strongly suggest that circulating foetal fibrocytes, routing via umbilical and placental vessels, are a reservoir for key cellular subsets in the placenta. This study reports fibrocytes in the human umbilical cord and placenta for the first time, and a novel role for both circulating foetal cells and the umbilical vessels in placental development, which is deranged in IUGR. [ABSTRACT FROM AUTHOR]

Aim: To determine the incidence and clinical aspects of allergic proctitis (AP) in infants with symptoms that mimic Hirschsprung's disease (HD). Methods: One hundred and five patients less than 6 months of age, who underwent barium enema, anorectal manometry and rectal suction biopsy due to suspicion of HD, were enrolled. Comparison of the patient characteristics associated with each disease was based on the results of the triple testing. The sensitivity and specificity of the three tests, for the diagnosis of HD, were evaluated. Results: The mean age of enrolled patients was 2.1 ± 0.9 months. Based on the three tests, 39 patients (37.1%) were diagnosed with HD, seven patients (6.7%) with AP, and 53 (50.5%) had normal results. Of the 54 patients with transitional zone and a reversed rectosigmoid index on the barium enema, four (7.4%) were patients with AP. The mean age of the AP patients (3.1 ± 1.5 months old) was older than the HD children (1.4 ± 0.9 months old). The sensitivity of the three tests for HD was 97.4%, 87.2% and 92.3% and the specificity was: 74.2%, 78.8% and 100%, respectively. Conclusions: In the infants with severe abdominal distention, the incidence of AP mimicking HD was relatively high. Therefore, consideration of AP should be part of the differential diagnosis in infants with severe abdominal distention or findings that mimic HD. For differentiation of these disorders, a rectal suction biopsy is very useful. [ABSTRACT FROM AUTHOR]

Nerve sheath myxoma (NSM) is a rare, benign tumor of predominantly cutaneous location. They usually arise from small cutaneous nerves in the head, neck, and extremities, but exceptionally they arise from spinal nerve roots. Only three cases of intraspinal NSM have been reported. Herein is describe two additional cases of spinal NSM. The patients were 64-year-old and 31-year-old men who presented with low back pain. Spine magnetic resonance imaging showed small intradural extramedullary masses at the L2–3 level. Both tumors had typical histological features of myxoid-type NSM. The tumors had a strong immunoreactivity for vimentin, S-100 protein, and neuron-specific enolase and focal expression of epithelial membrane antigen and phosphorylated neurofilament. Ultrastructural observation of tumor cells with perineurial, fibroblast-like, and Schwann-cell differentiation suggests an origin from nerve sheath precursor cells. [ABSTRACT FROM AUTHOR]

Comorbidity of normal pressure hydrocephalus (NPH) and Alzheimer's disease (AD) is not uncommon. However, few studies have reported the clinical courses of these patients in depth. A 73-year-old woman was confirmed to have AD by a biopsy performed during a shunt operation for NPH after a head trauma. She was followed for 4 years using serial neuropsychological tests and positron emission tomography (PET). Her clinical symptoms remained improved for 2.5 years and then declined. The 1-year minus the presurgical PET scan highlighted the bilateral frontal area, basal ganglia, and thalamus, which may reflect brain regions associated with the improvement of hydrocephalic dementia. On the other hand, the 1-year minus the 4-year scan highlighted the bilateral temporoparietal area and the posterior cingulate gyrus, which may reflect brain regions associated with the aggravation of AD. This subtraction method may be useful for monitoring the clinical course in patients with NPH and AD. [ABSTRACT FROM AUTHOR]

Chordoid glioma has been recently described as a slow-growing neoplasm with chordoid appearance, occurring exclusively in the regions of the third ventricle and hypothalamus of middle-aged women. We experienced a case of a 48-year-old woman with a suprasellar tumor composed of chordoid glioma and Rathke's cleft cyst, which was confirmed by histopathological, immunohistochemical and electron microscopic examinations. Histologically, chordoid glioma comprised the major part of the tumor, and the prominent Rathke's cleft cysts were distributed focally in the same tumor tissue without any transitions. Chordoid glioma was immunoreactive for glial fibrillary acidic protein, S-100 protein and vimentin, and focally positive for epithelial membrane antigen and CD34, while cytokeratin highlighted epithelial cells lining Rathke's cleft cysts. Ultrastructural examination of the chordoid glioma revealed short cytoplasmic processes, intermediate filaments, intercellular junctions of zonular adherens type, basal lamina, secretory granules and pinocytic vesicles. The ultrastructural observations of the current case are similar to those of the subcommisural organ, although cell body zonation or microvilli were not evident. The coexistence of chordoid glioma and Rathke's cleft cyst has not been reported previously and may represent a collision tumor. [ABSTRACT FROM AUTHOR]

Sung C O, Suh Y-L & Hong S-C (2011) Histopathology 59, 308-317 CD34 and microtubule-associated protein 2 expression in dysembryoplastic neuroepithelial tumours with an emphasis on dual expression in non-specific types Aims: Three histological variants of dysembryoplastic neuroepithelial tumour (DNT) have been described, namely, simple, complex and non-specific. However, the concept of non-specific variants of DNT remains controversial, because they cannot be accurately distinguished by histological findings alone from ordinary gliomas. The aim was to characterize further the non-specific histological forms of DNT. Methods and results: Forty-one DNTs classified as three histological forms were investigated with CD34 and microtubule-associated protein 2 (MAP2) immunohistochemistry. CD34 immunoreactivity was more frequently observed in non-specific DNT types (16/18 cases; 88.9%) than in classic types (6/23 cases; 26.1%) ( P < 0.001). Peritumoral CD34 expression of non-neoplastic cells was significantly associated with CD34-positive tumours (20/22 cases; 90.9%) than with CD34-negative tumours (3/19 cases; 15.8%) ( P < 0.001). MAP2 positivity in oligodendroglia-like cells or glial elements was significantly different between classic types and non-specific types ( P = 0.025). CD34 and MAP2 immunoreactivities were significantly more frequent in non-specific types (83.3%) than in simple (10%) and complex forms (30.8%) ( P < 0.001). Conclusions: Non-specific DNTs are glioneuronal tumours that have a heterogeneous population of cells with more immature neuronal and glial phenotypes. Furthermore, with regard to practical implications, combined analysis of CD34 and MAP2 is useful in distinguishing DNTs from particularly diagnostically challenging mimics. [ABSTRACT FROM AUTHOR]