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Lipoblastoma is a rare benign mesenchymal neoplasm that typically occurs at various sites in infants and children but may also occur in adults. Thus, differential diagnoses are often performed. To understand this tumor type, the present study described clinicopathological features, diagnosis and differential diagnosis of different morphological lipoblastomas. A single-institution retrospective review of 36 lipoblastoma cases diagnosed between 2015 and 2021 was performed. Formalin-fixed paraffin-embedded tissue was used for S-100, CD34, P16 and desmin immunohistochemistry analysis, along with rapid fluorescence in situ hybridization (FISH) detection with pleiomorphic adenoma gene 1 (PLAG1). The 36 cases included 14 females and 22 males [age range, 7 days to 33 years (median, 16.5 years); 28 patients were aged ≤3 years] and the tumors were located in the trunk (n=16), limbs (n=12), head and neck (n=6), and perineum (n=2). Histologically, lipoblastomas were divided into classic (n=15), lipoma-like (n=13) and myxoid (n=8) subtypes. They comprised lobules of mature adipose tissue of varying size and a fine capillary network surrounded by mucinous stroma. Single- or multivesicular lipoblasts positive for S-100 (29/36, 81%) were observed, with occasional mature adipocytes. Peripheral vessels and cytoplasm of primitive mesenchymal cells were diffusely positive for CD34 (36/36, 100%), whereas primitive mesenchymal cells and striated muscle tissue were positive for desmin (26/36, 72%). Most tumor cells were negative while only few were positive for P16 (8/36, 22%). FISH revealed PLAG1 breakage and rearrangement in 24/32 (75%) patients. In total, 28 patients were followed up post-operatively (range, 2-84 months; median, 41 months; 3 patients relapsed and 8 were lost to follow-up). In conclusion, diagnosis of a typical lipoblastoma is not difficult and PLAG1 breakage detection is key for the diagnosis. [ABSTRACT FROM AUTHOR]

Objective: To summarize application effect and clinical experience of multimodal intraoperative neurophysiological monitoring (IONM) technology in the surgery of neurofibromatosis type 1 (NF1) related peripheral nerve tumors., Methods: A retrospective study was conducted on NF1 patients, who admitted between January 2019 and December 2023 and treated with peripheral nerve tumor resection surgery assisted by multimodal IONM technology. There were 49 males and 45 females. The age ranged from 5 to 78 years, with an average of 33.7 years. Tumor morphological classification included 71 cases of nodular type, 13 cases of diffuse type, and 10 cases of mixed type. Target tumors were distributed in craniofacial region (47 cases), neck (11 cases), trunk (12 cases), and limbs (24 cases). Preoperatively, 44 cases had no obvious neurological symptoms, while the remaining patients had neurological symptoms, including 15 cases of visual impairment, 5 cases of hearing impairment, 16 cases of somatic movement disorders, and 31 cases of somatic sensory disorders, of which 7 cases had more than one symptom. IONM plans were selected based on the relevant nerves and adjacent important structures of the target tumor, including visual evoked potential (17 cases), somatosensory evoked potential (44 cases), motor evoked potential (88 cases), and electromyogram (94 cases)., Results: All surgeries were successfully completed. Ninety-three patients underwent total/near total resection and 1 patient underwent palliative resection. Pathological examination showed 80 cases of neurofibroma and 14 cases of malignant peripheral nerve sheath tumors. Complications included 2 cases of hematoma and 3 cases of incision infection. All patients were followed up 3-61 months (median, 15 months). During follow-up, no significant changes in neurological symptoms or tumor recurrence were found. Among the patients with preoperative visual impairment, there were 14 cases with no improvement in symptoms and 1 with improvement after surgery. Among the patients with somatic movement disorders, there were 11 cases with no improvement in symptoms, 3 cases with improvement, 2 cases with aggravation, 4 newly onset cases, and 1 case with significant impact on daily life after surgery. Among the patients with somatic sensory disorders, there were 17 cases with no improvement in symptoms, 14 cases with improvement, and 13 newly onset cases. The patients with hearing impairment showed improvement after surgery., Conclusion: The clinical manifestations of NF1 related peripheral nerve tumors are complex. Multimodal IONM technology can provide real-time detection of nerve provocation and damage. Surgical treatment with multimodal IONM technology is safe and can reduce complications.

Background: Percutaneous balloon compression (PBC) has become one of the most common and effective minimally invasive treatments for trigeminal neuralgia (TN). However, the initial and long-term pain outcomes, as well as the complication rates of PBC for patients with TN with concomitant continuous pain (CCP) have yet to be specifically documented., Objective: In this clinical study, we aimed to evaluate and compare the results of PBC in treating TN with and without CCP., Study Design: Retrospective study., Methods: This research retrospectively analyzed the pain outcomes and complications of 57 patients with TN with CCP and 118 patients with TN without CCP who had undergone PBC at our institution from January 2019 through June 2022. Procedures were performed by one senior neurosurgeon in a single center. The postdischarge follow-up and the collection of clinical data, including immediate and long-term pain relief, time to recurrence, and complications, were completed through phone contact by an independent neurosurgeon blind to the patients' information. Then, the results of the 2 groups were compared; demographic and clinical data were evaluated for possible predictive factors for poor pain outcomes., Results: In this study, PBC immediately resulted in complete pain relief in 70.2% of patients with CCP and significant pain relief in 84.2% of patients with CCP. For patients without CCP, the rates were 73.7% for complete pain relief and 85.6% for significant pain relief. After a minimum 6-month follow-up period, the rates decreased to 52.6% for complete pain relief and 73.7% for significant pain relief in patients with CCP, compared to 54.2% and 75.4% in those without CCP. The initial and long-term pain control rates in patients without CCP were slightly higher than those with CCP, but the differences were not statistically significant (P = 0.878, P = 0.968, respectively). The incidences of postoperative complications were similar between patients with and without CCP (21.1% vs 22.0%, P = 0.883), whereas the remission rate of complications in patients with CCP was significantly lower than that in patients without CCP (25.0% vs 69.2%, P = 0.011). A longer symptoms duration and having a history of neurodestructive procedures were predictive factors for poor outcomes following PBC., Limitations: The study was performed in a single-center. The nature of this research is retrospective instead of prospective and randomized, with the inability to control completely for variables. Additionally, the follow-up duration was not long enough to observe recurrence in some patients., Conclusions: This is the first specifically reported experience treating TN with CCP with PBC. PBC can result in significant relief of both episodic and constant pain from TN with CCP. Patients with a longer duration of pain and prior neurodestructive procedures have a higher risk of poor outcomes. The presence of CCP is not associated with pain outcomes and should not be considered a contraindication to PBC.

The clinical prognosis of malignant gliomas is poor and PCDH9 down-regulation is strongly associated with its poor prognosis. But the mechanism of PCDH9 down-regulation is unknown. Abnormal miRNAs profiles regulate tumor phenotypes through inhibiting their target genes and miRNAs could inhibit target genes more efficiently by binding to both the promoter and 3'UTR of target genes. In this study, to search the dual inhibitory miRNAs which suppress PCDH9 expression in gliomas, we performed an integrative analysis of databases including miRDB, TargetScan, microPIR and miRCancer. We identified three candidate miRNAs which were predicted to bind both the promoter and 3'UTR of PCDH9 and up-regulated in gliomas. Then, we validated miR-215-5p up-regulation and PCDH9 down-regulation in glioma samples and demonstrated that miR-215-5p could inhibit the mRNA and protein levels of PCDH9 in glioma cell lines by targeting its promoter and 3' UTR at the same time. Moreover, miR-215-5p could increase glioma cell proliferation, clone formation, in-vitro migration and reduce apoptosis via inhibiting PCDH9 expression. Our study provides evidence for a novel dual inhibition of PCDH9 by miR-215-5p in gliomas and suggests that miR-215-5p might be a therapeutic target for the treatment of gliomas.