目的 总结1例腹膜后神经鞘瘤合并胃神经鞘瘤的临床诊疗方法。 方法 2010年12月收治1例女性患者，因呕血行CT检查发现胃体前壁及右肾上腺区占位入院，行胃楔形切除术及右肾上腺肿瘤切除术治疗。 结果 术后病理证实为腹膜后神经鞘瘤合并胃神经鞘瘤，随访半年无复发。 结论 腹膜后神经鞘瘤合并胃神经鞘瘤病例罕见且诊断困难，影像学检查缺乏特异性，可依靠术后病理检查确诊；外科手术完整切除肿瘤是有效的治疗方法，预后较好。
Objective To explore the role and clinical significance of cell-cycle dependent kinase 1 (CDK1) and its upstream and downstream molecules in the development of malignant peripheral nerve sheath tumor (MPNST) through the analysis of clinical tissue samples. Methods A total of 56 tumor samples from MPNST patients (“Tianjin” dataset) who underwent surgical resection, confirmed by histology and pathology between September 2011 and March 2020, along with 17 normal tissue samples, were selected as the research subjects. MPNST-related hub genes were identified through transcriptome sequencing, bioinformatics analysis, immunohistochemistry staining, and survival analysis, and their expression levels and prognostic associations were analyzed. Results Transcriptome sequencing and bioinformatics analysis revealed that upregulated genes in MPNST were predominantly enriched in cell cycle-related pathways, with CDK1 occupying a central position among all differentially expressed genes. Further differential analysis demonstrated that CDK1 mRNA expression in sarcoma tissues was significantly higher than in normal tissues [based on searching the cancer genome atlas (TCGA) dataset, P<0.05]. In MPNST tissues, CDK1 mRNA expression was not only significantly higher than in normal tissues (based on Tianjin, GSE141438 datasets, P<0.05), but also significantly higher than in neurofibromatosis (NF) and plexiform neurofibromas (PNF) (based on GSE66743 and GSE145064 datasets, P<0.05). Immunohistochemical staining results indicated that the expression rate of CDK1 protein in MPNST tissues was 40.31%. Survival analysis results demonstrated that CDK1 expression was associated with poor prognosis. The survival time of MPNST patients with high CDK1 mRNA expression was significantly lower than that of the low expression group (P<0.05), and the overall survival trend of patients with positive CDK1 protein expression was worse than that of patients with negative CDK1 expression. Additionally, differential analysis of CDK family genes (CDK1-8) revealed that only CDK1 was significantly upregulated in MPNST, NF, and PNF. Conclusion Increased expression of CDK1 is associated with poor prognosis in MPNST patients. Compared to other CDK family members, CDK1 exhibits a unique expression pattern, suggesting its potential as a therapeutic target for MPNST.
ObjectiveTo investigate the sonographic characteristics and diagnostic value of color Doppler sonography for patients with renal schwannoma. MethodsTen patients with pathologically confirmed renal schwannoma dimensional ultrasonography treated between January 2008 and May 2014 were included in this study. We analyzed and summarized their color flow distribution characteristics. ResultsThe ultrasound of the 10 patients showed substantial hypoechoic mass with clear boundary and complete capsule; color Doppler flow imaging displayed mass within the probe and a small amount of dotted blood flow information or no obvious blood flow information. Four patients were suspected to have schwannoma before surgery by ultrasound diagnosis, while the remaining 6 cases only showed benign lesions without clear diagnosis. All the patients underwent surgery subsequently under laparoscope, and resection of the tumor was performed with the integrity of normal kidney tissue retained. Postoperative recovery was good, and follow-up did not detect any recurrence. ConclusionColor Doppler ultrasound has a certain specificity on the diagnosis of renal schwannoma. Combined with other examination methods, it can not only give the clinicians a good suggestion, but avoid laparotomy.
Schwannoma originating from the common bile duct is rare. We presented a patient who was diagnosed with biliary cystadenoma preoperatively and pathologically confirmed as a choledochal Schwannoma, analyzed the CT and MRI imaging manifestations, and illustrated its anatomical and pathological basis, and to improve the understanding of clinicians and radiologists for choledochal Schwannoma.
ObjectiveTo investigate the procedure and effectiveness of posterior approach for operation of atlantoaxial subdural extramedullary nerve sheath tumors.MethodsBetween January 2012 and March 2017, 9 patients with atlantoaxial subdural extramedullary nerve sheath tumors were treated, including 7 males and 2 females, aged 25-62 years (mean, 45.4 years). There were 8 cases of neurinoma and 1 case of neurofibroma. The tumors were located at C1 in 1 case and C1, 2 in 8 cases. The disease duration ranged from 5 to 120 months, with an average of 45.9 months. The neural function was rated as grade D in 8 cases and grade E in 1 case according to the American Spinal Injury Association (ASIA) grading system. The Japanese Orthopaedic Association (JOA) score was 12.8±2.5. All patients underwent posterior cervical surgery. The laminae were replanted and fixed in 2 cases. The atlantoaxial or occipitocervical axis was not fixed in all patients.ResultsThe operation time was 90-343 minutes, with an average of 179.2 minutes. The intraoperative blood loss was 50-1 000 mL, with an average of 335.6 mL. No relevant complication occurred after operation. All patients were followed up 6-21 months (mean, 11.1 months). The postoperative X-ray films showed the good stability of the cervical spine. All patients had complete tumor resection and no recurrence. The replanted laminae achieved fusion and the internal fixation was firm. According to ASIA grading system, 3 patients of preoperative ASIA grade D had upgraded to grade E at 3 months after operation, while the remaining patients had no change in grading. The JOA score was 15.1±1.4 at 6 months after operation, which was significantly improved when compared with that before operation (t=4.221, P=0.003).ConclusionThe atlantoaxial subdural extramedullary nerve sheath tumor (including the ventral tumor) can be removed completely via posterior approach. The axis lamina can be replanted and fixed with the small titanium plate or lamina screw when necessary, and the atlantoaxial or occipitocervical fixation was not needed.
Objective To summarize the latest developments in neurosurgical treatments for neurofibromatosis type 1 (NF1) and explore therapeutic strategies to provide comprehensive treatment guidelines for clinicians. Methods The recent domestic and international literature and clinical cases in the field of NF1 were reviewed. The main types of neurological complications associated with NF1 and their treatments were thorough summarized and the future research directions in neurosurgery was analyzed. Results NF1 frequently results in complex and diverse lesions in the central and peripheral nervous systems, particularly low-grade gliomas in the brain and spinal canal and paraspinal neurofibromas. Treatment decisions should be made by a multidisciplinary team. Symptomatic plexiform neurofibromas and tumors with malignant imaging evidence require neurosurgical intervention. The goals of surgery include reducing tumor size, alleviating pain, and improving appearance. Postoperative functional rehabilitation exercises, long-term multidisciplinary follow-up, and psychosocial interventions are crucial for improving the quality of life for patients. Advanced imaging guidance systems and artificial intelligence technologies can help increase tumor resection rates and reduce recurrence. Conclusion Neurosurgical intervention is the primary treatment for symptomatic plexiform neurofibromas and malignant peripheral nerve sheath tumors when medical treatment is ineffective and the lesions progress rapidly. Preoperative multidisciplinary assessment, intraoperative electrophysiological monitoring, and advanced surgical assistance devices significantly enhance surgical efficacy and safety. Future research should continue to explore new surgical techniques and improve postoperative management strategies to achieve more precise and personalized treatment for NF1 patients.
Objective To summarize the research progress of gastric schwannoma (GS). Methods GS related researches and case reports were collected to make a review on general clinical manifestations, pathology characteristics, auxiliary examinations, differential diagnosis, and treatment. Results GS is rare in clinical reports, and it is lack of tissue specificity. Diagnosis is rely mainly on the expressions of S-100 protein and glial fibrillary acidic protein (GFAP). All kinds of auxiliary examinations, such as gastrointestinal angiography, ultrasound, endoscope, CT, an so on, could not independently as a diagnostic basis. Identification between GS and other gastric tumors is difficult, and GS is not sensitive to radiation and chemotherapy, the main treatment is surgery. Conclusions GS is a kind of rare tumor which occurs in gastric mucosa, immunohistochemistry is the most main examination for the diagnosis of GS. The surgical excision is the main treatment for GS, but the safety and efficacy of combination therapy of laparoscopy and endoscopy remains to be study.