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Objective: The T2-FLAIR mismatch sign is a useful imaging sign in clinical magnetic resonance imaging studies for detecting isocitrate dehydrogenase (IDH)-mutant 1p/19q non-codeleted astrocytomas. However, the association between the mismatch sign and pathologic findings is poorly understood. Therefore, the aim of this study was to elucidate the relationship of histopathologic and radiologic features with the mismatch sign in IDH-mutant 1p/19q non-codeleted astrocytomas. Methods: We divided 17 IDH-mutant 1p/19q non-codeleted patients into 2 groups according to mismatch sign presence (WITH, n = 9; WITHOUT, n = 8) and retrospectively analyzed their pathologic findings and apparent diffusion coefficient (ADC) values. We also compared these findings between the tumor Core (central area) and Rim (marginal area). Results: In the pathologic analysis, Core of the WITH group contained numerous microcysts whereas Rim had abundant neuroglial fibrils and cellularity. In contrast, Core of the WITHOUT group had highly concentrated neuroglial fibrils. In ADC analysis, Core of the WITH group had significantly higher ADC values compared with Rim (P < 0.001). However, there was no significant difference between Core and Rim in the WITHOUT group (P = 0.12). The WITH group had a significantly higher Core/Rim ratio of ADC values compared with the WITHOUT group (P < 0.001). Conclusions: This study provides evidence that a region-dependent microstructural difference could reflect the mismatch sign in IDH-mutant 1p/19q non-codeleted astrocytomas. Core of the mismatch sign characteristically had microcystic changes accompanied by higher ADC values, whereas Rim had abundant neuroglial fibrils and cellularity accompanied by lower ADC values.

Purpose To report cases that showed partition-like, dark areas in the cone mosaic on adaptive optics scanning laser ophthalmoscopy (AO-SLO) images in eyes with inner nuclear layer (INL) microcystic changes. Methods Eyes with INL microcystic changes were imaged by prototype AO-SLO. Results An eye with Leber hereditary optic neuropathy, an eye with traumatic optic neuropathy, and an eye with retinitis pigmentosa that showed microcystic lesions in the INL were imaged by AO-SLO. The images revealed characteristic, dark, partition-like lesions in the cone mosaic of all the eyes in areas where microcystic changes in the INL were shown by spectral domain optical coherence tomography. The AO-SLO findings in eyes with optic neuropathy were quite similar in shape and size to those seen in eyes with retinitis pigmentosa. Conclusion We report cases that manifest dark, partition-like areas in the cone mosaic on AO-SLO images. Microcystic lesions in the INL may affect the images of the cone mosaic.

Subependymomas are slowly growing glial tumors, corresponding to WHO grade I. Few descriptions of the cytologic features of this neoplasm are available. This study describes the cytologic features of three subependymomas, as well as their differential diagnosis based on cytology. Three men, aged 52, 56, and 63 years, presented with headache. Magnetic resonance imaging revealed a nodular intraventricular mass in all three patients. Intraoperative squash cytology specimens from the three intraventricular tumors showed nodular clusters with microcystic changes. Nuclei were round to oval in shape, but showed no evidence of severe nuclear atypia or mitoses. Histological examination showed features of subependymoma. Squash cytology findings, including nodular clusters, mild cellular atypia, microcystic changes, and mucoid material, are useful in the rapid intraoperative diagnosis of subependymoma.

The angiomatous variant of the pleomorphic xantho-astrocytoma (PXA) is a morphological subtype of PXA that is characterized by the presence of large numbers of tiny blood vessels with variable wall thicknesses. A case of an angiomatous variant of PXA occurring in the temporal lobe of a 43-year-old man is presented. The patient had a 20-year history of seizures. Considering the clinical course and intra-operative electro-encephalography findings, it appears that this tumor was the epileptic focus in this patient and that it had been present for 20 years. Histopathologically, various degenerative changes, such as microcystic changes, calcification and hyaline thickening of vessel walls were seen in the tumor. In particular, obvious vascularity in the tumor and secondary vascular degenera-tion should be related to gradual growth and benign clinical course of an angiomatous variant of PXA.

There are numerous causes of bilateral hyperechoic kidneys. Congenital disorders of glycosylation (CDGs) are a rapidly growing family of inherited disorders due to defects in the synthesis of the glycans of glycoproteins or other glycoconjugates.To describe renal sonographic abnormalities in CDG type I in infants and children.A retrospective study of renal US in 12 infants and children: 8 CDG-Ia (6 multivisceral forms, 2 neurological forms), 2 CDG-Ib, and 2 CDG-Ix, with detailed functional renal tests in 6. Histology of the kidneys of one 35-week fetus with CDG-Ia was available.Renal US was normal in the two children with the neurological form of CDG-Ia. All patients with the multivisceral form of CDG-Ia or with CDG-Ib showed increased cortical echogenicity, and/or abnormal pyramids (small +/- hyperechoic). The two patients with CDG-Ix showed predominant involvement of the medulla, with inverted corticomedullary differentiation in one. Kidney size was normal in all but two patients. The fetal kidneys exhibited diffuse microcysts arising from the distal tubules.Hyperechoic kidneys are common in CDG-I patients, contrasting with grossly preserved renal function. The US pattern seems to differ slightly according to the type of CDG-I, and is consistent with microcystic changes of the renal parenchyma, which occur prenatally, and may be due to ciliary dysfunction secondary to altered glycosylation of tubular glycoproteins. CDG-I, which remains largely underdiagnosed at present, should be added to the causes of hyperechoic kidneys in children, especially in cases of multivisceral involvement, after ruling out other more frequent causes.

Die Verfasser fuhren einen Fall malignen symptomatologischen Hochdrucks bei einem achtjahrigen Knaben vor. Es werden neben der linken Nierenarteriendrosselung auch mikrocystische Nierenmarkveranderungen festgestellt. In diesem Zusammenhang wird an Hand des Schrifttums die Koexistenz der beiden Krankheiten analysiert. Der ungewohnliche klinische Verlauf wird betont, und zwar das Zusammentreffen von massiver Proteinurie mit den Hochstwerten des arteriellen Blutdrucks. Es werden auch Erscheinungen besprochen, die mit der auftretenden Hypokaliamie zusammenhangen, und deren Ursache der sekundare Aldosteronismus und hartnackiges Erbrechen sind. Die Ergebnisse der Laboruntersuchungen und Blutmessungen im Laufe des ersten Jahres nach der Operation liefern den Beweis einer volligen Ausheilung.