ObjectiveTo summarize the clinicopathological features of microencapsulated/reticular schwannoma.MethodsTwo cases of microencapsulated/reticular schwannoma treated in West China Hospital of Sichuan University were retrospectively collected. The histological and immunohistochemical features were summarized, and the related literatures were reviewed.ResultsTwo cases of microcystic/reticular neurilemmoma diagnosed and treated in West China Hospital of Sichuan University were male. The clinical manifestations were “dull pain in the right lower abdomen” and “mass of left index finger”. Microscopically, the characteristic reticular or cribriform structures were found in all cases, and lymphocytic sheath was seen in 1 case. Immunohistochemical staining showed S-100, glial fibrillary acidic protein (GFAP) and calretinin positive. By February 2020, only 41 cases were reported in the literatures （there were no cases of this group),there was no significant gender difference, the median age was 55 years (11–93 years), and the median size of the lesion was 2.1 cm (0.4–13.0 cm). Most of them were found by accident, without obvious symptoms and signs, and no history of neurofibromatosis type 1 or 2. Microencapsulated/reticular schwannoma in this patients mainly occurs in the viscera, especially in the gastrointestinal tract (mostly in the the colorectal of lower gastrointestinal tract ), most of them were solitary nodular masses without capsule. Microscopically, the boundary was clear, and the characteristic microcapsules and reticular structures were seen. The tumor cells were diffusely expressing S-100 protein, and GFAP and calretinin were expressed in varying degrees. Electron microscopy showed the characteristics of Schwann cells.ConclusionsMicrocystic/reticular schwannoma is a rare subtype of schwannoma, which needs to be differ-entiated from multiple benign and malignant neoplasms. The diagnosis of the tumor is a challenge for the pathologist.
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.
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.
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.
Schwannoma originating in the retroperitoneal space is relatively rare. The author reported a case of retroperitoneal Schwannoma confirmed by surgery and pathology, presented the typical CT and MRI manifestations of Schwannoma——“target signs”, described the pathophysiological mechanism and image differential diagnosis of the disease, so as to strengthen readers’ understanding of the typical signs of Schwannoma and improve the correct diagnosis rate of the disease.
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.