Central nervous system neuroblastic tumor with FOXR2 activation presenting both neuronal and glial differentiation: a case report

伴 FOXR2 激活的中枢神经系统神经母细胞瘤同时呈现神经元和神经胶质分化 1 例

  • 影响因子:2.86
  • DOI:10.1007/s10014-020-00370-2
  • 作者列表:"Furuta, Takuya","Moritsubo, Mayuko","Muta, Hiroko","Koga, Motohisa","Komaki, Satoru","Nakamura, Hideo","Morioka, Motohiro","Ohshima, Koichi","Sugita, Yasuo
  • 发表时间:2020-06-13

A subset of central nervous system neuroblastomas (CNS NB), rare primary embryonal CNS tumors, has been encompassed in CNS NB with FOXR2 activation (CNS NB- FOXR2 ) and usually shows the primitive neuronal architecture and occasional neurocytic differentiation. Here, we report a rare case of 3-year-old female with uncommon morphology of CNS embryonal tumor with FOXR2 activation presenting bidirectional differentiation to neurocytic small primitive cells and astrocytic spindle cells both of which are positive for synaptophysin and GFAP. Ultrastructural study also showed that there were presynaptic structure and intermediate filament in the tumor cells, suggesting glioneuronal differentiation. This case indicates the possibility of CNS neuroblastic tumor to differentiate neuronal and glial lineages.


中枢神经系统神经母细胞瘤 (CNS NB) 的一个子集,罕见的原发性胚胎性 CNS 肿瘤,已包含在 CNS NB 中,伴 FOXR2 激活 (CNS NB- FOXR2) 通常显示原始神经元结构和偶尔的神经细胞分化。在这里,我们报告一例罕见的 3 岁女性患者,其罕见形态为 CNS 胚胎性肿瘤,FOXR2 激活呈现双向分化为神经细胞小原始细胞和星形细胞梭形细胞,两者均阳性。突触素和 GFAP。超微结构研究还表明,肿瘤细胞内存在突触前结构和中间丝,提示胶质神经分化。该病例表明 CNS 神经母细胞瘤有可能区分神经元和神经胶质谱系。



作者列表:["Yakoub-Agha I","Chabannon C","Bader P","Basak GW","Bonig H","Ciceri F","Corbacioglu S","Duarte RF","Einsele H","Hudecek M","Kersten MJ","Köhl U","Kuball J","Mielke S","Mohty M","Murray J","Nagler A","Robinson S","Saccardi R","Sanchez-Guijo F","Snowden JA","Srour M","Styczynski J","Urbano-Ispizua A","Hayden PJ","Kröger N"]

METHODS::Chimeric antigen receptor (CAR) T cells are a novel class of anti-cancer therapy in which autologous or allogeneic T cells are engineered to express a CAR targeting a membrane antigen. In Europe, tisagenlecleucel (Kymriah™) is approved for the treatment of refractory/relapsed acute lymphoblastic leukemia in children and young adults as well as relapsed/refractory diffuse large B-cell lymphoma, while axicabtagene ciloleucel (Yescarta™) is approved for the treatment of relapsed/refractory high-grade B-cell lymphoma and primary mediastinal B-cell lymphoma. Both agents are genetically engineered autologous T cells targeting CD19. These practical recommendations, prepared under the auspices of the European Society of Blood and Marrow Transplantation, relate to patient care and supply chain management under the following headings: patient eligibility, screening laboratory tests and imaging and work-up prior to leukapheresis, how to perform leukapheresis, bridging therapy, lymphodepleting conditioning, product receipt and thawing, infusion of CAR T cells, short-term complications including cytokine release syndrome and immune effector cell-associated neurotoxicity syndrome, antibiotic prophylaxis, medium-term complications including cytopenias and B-cell aplasia, nursing and psychological support for patients, long-term follow-up, post-authorization safety surveillance, and regulatory issues. These recommendations are not prescriptive and are intended as guidance in the use of this novel therapeutic class.

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来源期刊:Oncology reports
作者列表:["Li C","Xu Y","Xin P","Zheng Y","Zhu X"]

METHODS:The aim of the present study was to explore the possible mechanisms of phosphatase and tensin homolog (PTEN) in the pathogenesis of Burkitt's lymphoma, and provide novel information that can be used in the targeted treatment of this disease. PTEN lentiviral overexpression vector and short‑hairpin PTEN silencing vectors were constructed. The effect of PTEN on the growth and proliferation of CA46 and RAJI cells was analyzed using a Cell Counting Kit‑8 assay. Apoptosis was detected by Hoechst 33342 and propidium iodide double staining. Flow cytometry was used to analyze the cell cycle. A Transwell chamber was used to detect cell migration and invasion abilities. Western blot analysis was used to detect related protein changes. The mechanism of the effect of PTEN on the biological characteristics of Burkitt's lymphoma cells was subsequently analyzed. The results revealed that PTEN inhibited the proliferation of CA46 and RAJI cells by downregulating the expression of p‑AKT, It was indicated that the upregulation of proapoptotic proteins (including Bad and Bax) induced apoptosis, regulated cyclin (including P53, P21, CDK4, CDK6, cyclin D3 and cyclin H) to inhibit cell cycle progression, and mediated epithelial‑mesenchymal transition‑like cell markers (including E‑cadherin, N‑cadherin, β‑catenin, TCF‑8, vimentin, Slug and Snail) to inhibit cell migration and invasion. In conclusion, the tumor‑suppressor gene PTEN inhibited the phosphoinositide 3‑kinase/protein kinase B (PI3K/AKT) signaling pathway and inhibited the proliferation and migration of Burkitt's lymphoma cells, induced apoptosis and cell cycle arrest, thus playing a crucial role in the pathogenesis of Burkitt's lymphoma.

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作者列表:["Yan G","Lei H","He M","Gong R","Wang Y","He X","Li G","Pang P","Li X","Yu S","Du W","Yuan Y"]

METHODS:Melatonin (Mel) has been shown to involve in many essential cell functions via modulating many signaling pathways. We for the first time investigated that Mel exerted anti-tumor activities in Hodgkin lymphoma (HL) via inhibiting cell proliferation and promoting cell apoptosis. Further study revealed that Mel treatment increased expression of LC3-II and decreased p62 proteins with the enhanced production of autolysosome, indicating it induced activation of autophagy. Nevertheless, Mel treatment together with autophagy inhibitors 3-MA or CQ exacerbated the damage effect of Mel in HL cells, which means autophagy plays a protective role in this process. Furthermore, we found Mel treatment increased the expression of G protein-coupled receptors MT2 and retinoic acid-related orphan receptors (RORs), eg. RORA, RORB and RORC. While RORC has the highest increase in Mel treated HL cells. In addition, RORC overexpression induced autophagy activation. Therefore, Mel showed tumor-suppressive role due to an increased level of RORC induced autophagy in HL.