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.
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 re-understand the nerve sheath tumors of the breast, to improve its diagnosis and cure rate.MethodSearched the relevant literatures of nerve sheath tumors of the breast, to analyze and summarize the origin, etiology, clinical manifestations, imaging characteristics, pathological characteristics, treatment, and prognosis of this disease.ResultsNerve sheath tumors of the breast was a very rare disease, which originated in the neuromembrane Snowwang cells. its specific cause was unknown and clinical manifestations were not specific, and other breast diseases were difficult to identify, such as leaf ybrilloma, mammary vascular epidermal cytoma, breast fibroids, and so on. Imaging data could provide some reference value, but the gold standard relied on pathology and immunohistochemical examination. Surgery could cure benign nerve sheath tumors of the breast, but there was a possibility of malignant changes that required follow-up after surgery. Malignant neuroblastoma was mainly surgically removed, supplemented by chemotherapy, which could effectively prevent the recurrence of tumor and distant metastasis. The prevention of nerve sheath tumors of the breast could be referred to breast cancer screening.ConclusionsDuring clinical practice, we need to understand the diagnosis and treatment of nerve sheath tumors of the breast to achieve early detection, early diagnosis, and early treatment, as well as improve the diagnosis rate and cure rate of the disease, in order to protect women’s physical and mental health.
Objective To explore the clinical features, surgical treatment, and effectiveness of neurofibromas associated with neurofibromatosis type 1 (NF1). Methods A clinical data of 41 patients with NF1 admitted between December 2018 and April 2024 was retrospectively analyzed. There were 15 males and 26 females, with an average age of 27.5 years (range, 5-61 years). Only one type of neurofibroma existed in 3 patients and the rest of the patients had more than two types of neurofibromas. Fourteen patients had total resection of multiple cutaneous neurofibromas (CNF). Eighteen patients of diffuse neurofibromas underwent total, near-total, or subtotal resection. Among the 13 patients of localized nodular neurofibromas, 9 of benign tumors underwent total sub-capsular resection and 4 of malignant peripheral nerve sheath tumor (MPNST) underwent maginal resection, and only 1 underwent postoperative radiotherapy and chemotherapy. Among the 15 patients of plexiform neurofibromas (PNF), 5 patients underwent both superficial and deep PNF resection, 2 underwent the superficial PNF resection, and 8 underwent the large nodular lesions in the deep PNF resection. There were 8 MPNST, of which 7 cases underwent total sub-capsular resection and large tumor capsule resection under neurophysiological monitoring, and 1 case with the tumor located on the top of the head underwent wide resection and skin grafting. One patient underwent proton knife therapy after surgery, 2 patients did not receive radiotherapy, and the remaining patients received conventional radiotherapy. Results All patients were followed up after surgery, and the follow-up time was 3-66 months, with an average of 25.0 months. Patients with CNF recovered satisfactorily after surgery, and there was no recurrence during follow-up. Patients with diffuse neurofibromas relieved preoperative symptoms after surgery. Three patients with diffuse neurofibromas located in the head and face recurred during follow-up. The patients with benign localized nodular neurofibromas recovered well after surgery, and only 1 patient had transient regional neuralgia after surgery. Among the patients with MPNST, 2 patients died of recurrence and lung metastasis, while the remaining 2 patients had no recurrence and metastasis during follow-up. All preoperative symptoms disappeared in patients with benign PNF, and no tumor recurrence was observed during follow-up. Two patients with PNF located in the brachial plexus had difficulty in shoulder abduction after surgery, 1 patient with PNF located in vagus developed hoarseness after surgery. Among the 8 patients with MPNST in PNF, 1 died of lung metastases and 1 died of systemic failure. The remaining 6 patients were in stable condition during follow-up, and no tumor recurrence or metastasis was observed. Conclusion According to the clinical features of neurofibromas in patients with NF1, choosing appropriate surgical approaches can obtain good effectiveness. Because of the difficulty of completely resection, diffuse neurofibromas, especially those located in the head and face, are prone to recurrence after surgery. MPNST has the worst prognosis, high incidence of recurrence/metastasis, and short survival period. Total resection combined with radiotherapy can decrease local recurrence.
Objective To investigate the clinical features, treatment methods, and prognostic influence factors of patients with malignant peripheral nerve sheath tumor (MPNST). MethodsA retrospective analysis was conducted on 96 MPNST patients treated between January 1, 2015 and December 31, 2021. There were 46 males and 50 females, aged between 15 and 87 years (mean, 48.2 years). The tumors were located in the trunk in 50 cases, extremities in 39 cases, and head and neck in 7 cases. The maximum tumor diameter was <5 cm in 49 cases, ≥5 cm in 32 cases, with 15 cases missing data. Tumor depth was deep in 77 cases and superficial in 19 cases. The Fédération Nationale des Centres de Lutte Contre le Cancer (FNCLCC) histological grading was G1 in 9 cases, G2 in 12 cases, and G3 in 34 cases, with 41 cases missing data. There were 37 recurrent MPNST cases, 32 cases with neurofibromatosis type 1 (NF1), and 26 cases in stage Ⅳ. Postoperative adjuvant radiotherapy was administered to 25 patients, perioperative chemotherapy to 45 patients, and anlotinib-targeted therapy to 30 patients. R0 resection was achieved in 73 cases. Patients were divided into groups based on the presence or absence of NF1, and baseline data between the two groups were compared. Kaplan-Meier curves were generated to assess disease-free survival (DFS) and overall survival (OS) based on various factors (age, gender, presence of NF1, recurrent MPNST, stage Ⅳ MPNST, FNCLCC grade, R0 resection, tumor location, tumor size, tumor depth, perioperative chemotherapy, postoperative adjuvant radiotherapy, and anlotinib-targeted therapy), and differences between survival curves were analyzed using the Log-Rank test. Multivariate COX proportional hazards regression was used to identify independent prognostic factors for MPNST. Results Patients with NF1 had a significantly higher proportion of superficial tumors and lower FNCLCC grade compared to those without NF1 (P<0.05); no significant difference was found for other variables (P<0.05). Kaplan-Meier analysis showed that recurrent MPNST, stage Ⅳ MPNST, FNCLCC grade, R0 resection, perioperative chemotherapy, and anlotinib-targeted therapy were factors influencing 1-year DFS (P<0.05), while stage Ⅳ MPNST, FNCLCC grade, and perioperative chemotherapy were factors affecting 3-year OS (P<0.05). Multivariate COX proportional hazards regression analysis revealed that recurrent MPNST and high-grade FNCLCC (G3) were independent prognostic factors for 1-year DFS (P<0.05), while stage Ⅳ MPNST, superficial tumor depth, age over 60 years, postoperative adjuvant radiotherapy, and anlotinib-targeted therapy were independent prognostic factors for 3-year OS (P<0.05). Conclusion MPNST patients with NF1 tend to have more superficial tumors and lower FNCLCC grades. FNCLCC grade, R0 resection, and adjuvant therapies, including radiotherapy and anlotinib-targeted therapy, are closely associated with MPNST prognosis. Complete surgical resection should be prioritized in clinical management, along with adjuvant treatments such as radiotherapy and targeted therapy of anlotinib to improve patient outcomes.
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.
摘要:目的: 探讨磁共振波谱(MRS)对鉴别桥小脑角神经鞘瘤与脑膜瘤的价值。 方法 :对8例神经鞘瘤和8例脑膜瘤病例进行MRI平扫和增强扫描,并采用点分辨波谱序列(PRESS,TR/TE=2000/136ms)进行单体素波谱分析。从事磁共振诊断专业的医师根据肿瘤的MRI及MRS表现特征进行鉴别诊断。 结果 :尽管多数神经鞘瘤(5/8)和脑膜瘤(6/8)具有典型的MRI表现特征并仅经MRI即可得到正确鉴别,但部分病例(5/16)为不典型表现者,如脑膜瘤呈长T1长T2信号并伴有囊变,以及神经鞘瘤呈等信号、均匀强化且不伴有囊变或内听道扩大者,鉴别诊断困难。MRS显示脑膜瘤的胆碱/肌酸比值(Cho/Cr)(2.74±1.47)略高于神经鞘瘤(2.70±1.21),但差异无统计学意义。丙氨酸(Ala)在脑膜瘤中的出现率(4/8)显著高于神经鞘瘤(0/8)(Plt;0.05),谷氨酸盐/谷氨酰胺(Glx)在脑膜瘤中的出现率(5/8)也高于神经鞘瘤(2/8);而肌醇(Ins)在神经鞘瘤中的出现率(4/8)高于脑膜瘤(2/8)。结合MRS表现,在MRI上尚难鉴别的病例可被明确诊断,并与最终病理结果相符。 结论 : MRI有助于多数桥小脑角神经鞘瘤与脑膜瘤的鉴别诊断,但对不典型病例有一定局限性;MRS对不典型病例的鉴别具有重要的参考价值。Abstract: Objective: To explore the value of magnetic resonance spectroscopy (MRS) in the differentiation of neurinoma and meningioma in the cerebellopontine angle. Methods : 16 cases, including 8 cases of meningiomas and 8 cases of neurinomas, underwent MRI (plan and gadoliniumenhanced scan) and singlevoxel proton MRS (PRESS series, TR/TE=2000/136ms). MRI and MRS results were reviewed and differential diagnoses were made by professional radiologists. Results : Although most cases (11/16) were typical and clearly classified on MRI, atypical cases were also observed, such as meningioma of long T1/T2 signal intensity with small cystic foci, and neurinoma of isointensity and homogenous enhancement without cystic change or enlargement of internal acoustic meatus. The choline to creatine ratio (Cho/Cr) of meningioma (2.74±1.47) was higher than that of neurinoma (2.70±1.21), however the difference was not significant (Pgt;0.05). Alanine was observed in 4/8 meningiomas but none of neurinomas (Plt;0.05), and glutamine/glutamate was more frequently visualized in meningiomas (5/8) than in neurinomas (2/8), whilemyoinositol was more frequently observed in neurinomas (4/8) than in meningiomas (2/8). With MRS, cases difficult to differentiate were clearly and correctly classified. Conclusion : Although most CPA neurinomas and meningiomas can be diagnosed by MRI, MRS is useful for the differential diagnosis of atypical cases.