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The study aimed to investigate the clinical implications and natural history of primary intraparenchymal lesions in patients with neurofibromatosis type 2. Radiological findings of 15 neurofibromatosis type 2 cases were retrospectively collected. Twenty-seven primary intraparenchymal lesions were observed in 7 out of 15 patients (47%). Cortical/subcortical T2 hyperintense lesions and enlarged Virchow–Robin spaces were the most common findings in five and four patients, respectively. During the follow-up period (median 84 months), one new primary intraparenchymal lesion was identified and increased lesions were observed in two cases on contrast-enhanced MRI. Surgical resection was performed in one case pathologically diagnosed with atypical meningioma. Twenty-five other lesions without contrast enhancement presented no apparent growth during follow-up. Although most primary intraparenchymal lesions are benign, a subset of cases would present newly developed or increased lesions on contrast-enhanced MRI. Careful monitoring is necessary for such cases, and pathological confirmation should be considered.

Background High-density Lipoprotein (HDL) attenuates endothelial cell apoptosis induced by different cell-death stimuli such as oxidation or growth factor deprivation. HDL is the main plasma carrier of the bioactive lipid sphingosine 1-phosphate (S1P), which it is a signaling molecule that promotes cell survival in response to several apoptotic stimuli. In HDL, S1P is bound to Apolipoprotein M (ApoM), a Lipocalin that is only present in around 5% of the HDL particles. The goal of this study is to characterize ApoM-bound S1P role in endothelial apoptosis protection and the signaling pathways involved. Methods Human umbilical vein endothelial cells (HUVEC) cultures were switched to serum/grow factor deprivation medium to induce apoptosis and the effect caused by the addition of ApoM and S1P analyzed. Results The addition of HDL+ApoM or recombinant ApoM-bound S1P promoted cell viability and blocked apoptosis, whereas HDL-ApoM had no protective effect. Remarkably, S1P exerted a more potent anti-apoptotic effect when carried by ApoM as compared to albumin, or when added as free molecule. Mechanistically, cooperation between S1P1 and S1P3 was required for the HDL/ApoM/S1P-mediated anti-apoptotic ability. Furthermore, AKT and ERK phosphorylation was also necessary to achieve the anti-apoptotic effect of the HDL/ApoM/S1P complex. Conclusions Altogether, our results indicate that ApoM and S1P are key elements of the anti-apoptotic activity of HDL and promote optimal endothelial function. Electronic supplementary material The online version of this article (doi:10.1186/s12944-017-0429-2) contains supplementary material, which is available to authorized users.

Background: Vitreoretinal lymphoma (VRL) is a life- and sight-threatening disorder. The aim of this study was to analyze the usefulness of the cell block method for diagnosis of VRL. Methods: Sixteen eyes in 12 patients with VRL, and 4 eyes in 4 patients with idiopathic uveitis presenting with vitreous opacity were enrolled in this study. Both undiluted vitreous and diluted fluids were isolated during micro-incision vitrectomy. Cell block specimens were prepared in 19 eyes from diluted fluid containing shredding vitreous. These specimens were then submitted for HE staining as well as immunocytological analyses with antibodies against the B-cell marker CD20, the T-cell marker CD3, and cell proliferation marker Ki67. Conventional smear cytology was applied in 14 eyes with VRL using undiluted vitreous samples. The diagnosis of VRL was made based on the results of cytology, concentrations of interleukin (IL)-10 and IL-6 in undiluted vitreous, and immunoglobulin heavy chain gene rearrangement analysis. Results: Atypical lymphoid cells were identified in 14 out of 15 cell block specimens of VRL (positive rate: 93.3 %), but in 5 out of 14 eyes in conventional smear cytology (positive rate: 35.7 %). Atypical lymphoid cells showed immunoreactivity for CD20 and Ki67. Seven cell block specimens were smear cytology-negative and cell block-positive. The cell block method showed no atypical lymphoid cells in any patient with idiopathic uveitis. Conclusions: Cell block specimens using diluted vitreous fluid demonstrated a high diagnostic sensitivity and a low pseudo-positive rate for the cytological diagnosis of VRL. The cell block method contributed to clear differentiation between VRL and idiopathic uveitis with vitreous opacity., 背景：眼内リンパ腫（VRL）は視力・生命予後に影響を及ぼす疾患である。本研究ではVRLの診断における細胞塊（セルブロック）標本の有用性を検討することを目的とした。症例と方法：北海道大学病院眼科および他院を受診したVRL12例16眼、および硝子体混濁を呈した特発性ぶどう膜炎4例4眼を対象とした。小切開硝子体手術により、無希釈硝子体液および硝子体灌流液を採取した。19眼で、硝子体灌流液を用いたセルブロック標本を作製した。これらの標本を用いて特殊染色、およびB細胞マーカーである抗CD20抗体の免疫細胞化学的検討を行った。14眼では無希釈硝子体液を用いた塗抹細胞診を併せて行った。VRLの診断は、細胞診、無希釈硝子体液のIL-10, -6濃度、灌流液沈殿物から遺伝子再構成部位のモノクローナリティについて検討して行った。結果：VRLの15眼中14眼でセルブロック標本にて悪性リンパ腫細胞が検出された（陽性率：93.3％）。一方、塗抹細胞診では5眼で悪性細胞が検出された（陽性率：35.7％）。塗抹細胞診で陰性、セルブロックで陽性を示したIOL症例は7例8眼であった。特発性ぶどう膜炎では、悪性細胞が検出された症例はなかった（陽性率：0％）。VRL細胞はCD20陽性を示したが、特発性ぶどう膜炎ではCD20陽性細胞は検出されなかった。結語：硝子体灌流液を用いたセルブロック細胞診は、これまでの塗抹細胞診よりVRLの陽性率が高く、偽陽性も無かった。セルブロックは、VRLとぶどう膜炎との鑑別にも有用であることが示唆された。