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Background: Infantile Ewing Sarcoma (ES) is an extremely rare disease with comparable outcomes on contemporary protocols. There is a paucity of data from India. Methods: Infants with proven ES diagnosed between January 2012 and December 2020 were retrospectively analyzed. The staging was performed using positron emission tomography-computerized tomography scan. Treated infants were administered weight-based chemotherapy. The local control was with surgery and/or radiotherapy. Results: There were 10 infants with ES with a male-to-female ratio of 2.3:1. The majority had extraosseous (90 %, n = 9) or axial primary (50 %, n = 5). Five patients with localized disease received treatment. Two died during induction chemotherapy (sepsis-1, cause unknown-1), and 3 are alive with no evidence of disease at a median follow-up of 8 years (range: 4–9). Two episodes of complicated febrile neutropenia were reported in 5 treated infants. Conclusions: ES in infants is rare and predominantly extraosseous with axial primary. Treated infants with non-metastatic disease tolerated chemotherapy well with good outcomes.

Adult hippocampal neurogenesis is a lifelong process that involves the integration of newborn neurons into the hippocampal network, and plays a role in cognitive function and the modulation of mood-related behavior. Here, we sought to address the impact of chemogenetic activation of adult hippocampal progenitors on distinct stages of progenitor development, including quiescent stem cell activation, progenitor turnover, differentiation and morphological maturation. We find that hM3Dq-DREADD-mediated activation of nestin-positive adult hippocampal progenitors recruits quiescent stem cells, enhances progenitor proliferation, increases doublecortin-positive newborn neuron number, accompanied by an acceleration of differentiation and morphological maturation, associated with increased dendritic complexity. Behavioral analysis indicated anxiolytic behavioral responses in transgenic mice subjected to chemogenetic activation of adult hippocampal progenitors at timepoints when newborn neurons are predicted to integrate into the mature hippocampal network. Furthermore, we noted an enhanced fear memory extinction on a contextual fear memory learning task in transgenic mice subjected to chemogenetic activation of adult hippocampal progenitors. Our findings indicate that hM3Dq-DREAD-mediated chemogenetic activation of adult hippocampal progenitors impacts distinct aspects of hippocampal neurogenesis, associated with the regulation of anxiety-like behavior and fear memory extinction.

Wilms tumour is the commonest renal tumour in children with peak age group of 2–5 years [1,2]. It commonly presents as an incidentally detected abdominal mass; however fever, hematuria and hypertension may also be seen [1]. Extra-renal Wilms tumour is extremely rare, with an incidence of 0.5%–1% [3,4]. Wilms tumour, most commonly metastasizes to the lungs, followed by lymph nodes and liver, however, metastasis to the bones and spinal cord at diagnosis is extremely rare [1,2,5–10]. We hereby, report an extremely rare presentation of suprarenal Wilms tumour with intraspinal metastases. Our case highlights the high degree of vigilance as well as timely intervention with multimodality therapy required to improve the outcomes in the setting of advanced metastatic disease; notwithstanding the potentially irreversible neurological weakness in the absence of optimal intervention.

In eukaryotic cells, inefficient splicing is surprisingly common and leads to the degradation of transcripts with retained introns. How pre-mRNAs are committed to nuclear decay is unknown. Here, we uncover a mechanism by which specific intron-containing transcripts are targeted for nuclear degradation in fission yeast. Sequence elements within these “decay-promoting” introns co-transcriptionally recruit the exosome specificity factor Mmi1, which induces degradation of the unspliced precursor and leads to a reduction in the levels of the spliced mRNA. This mechanism negatively regulates levels of the RNA helicase DDX5/Dbp2 to promote cell survival in response to stress. In contrast, fast removal of decay-promoting introns by co-transcriptional splicing precludes Mmi1 recruitment and relieves negative expression regulation. We propose that decay-promoting introns facilitate the regulation of gene expression. Based on the identification of multiple additional Mmi1 targets, including mRNAs, long non-coding RNAs, and sn/snoRNAs, we suggest a general role in RNA regulation for Mmi1 through transcript degradation.