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 observe the changes of retinal nerve fiber layer (RNFL) thickness in patients with Alzheimer's disease (AD). Methods Twenty eyes of 40 patients with mild and (or) moderate AD confirmed by clinical examination (AD group) were included in the study. There were 11 males and 9 females with an average age of (72.75±8.25) years. Age and gender-matched normal 20 objectives were in the normal control group. Among them, there were 11 males and 9 females with a mean age of (71.05±7.08) years. There was no significant difference in gender composition, age and intraocular pressure between the two groups (P＞0.05). There were significant differences in visual acuity, cup disc ratio and mini-mental state examination score (P＜0.05). All eyes underwent high-resolution optical coherence tomography (OCT) examination. With a diameter of 3.4 mm and a center on the center of the optic disc, circular fast scans on optic disc were performed to obtain an average disc RNFL thickness, signal threshold ＞6. Computer image analysis system was used to measure the RNFL thickness from superior, inferior, temporal and nasal quadrants, and the average RNFL thickness. The changes of RNFL thickness between the two groups and between different eyes of the same group were compared. Results Compared with the normal control group, the average (t=5.591), superior (t=8.169, 8.053) and inferior (t=12.596, 11.377) thickness of RNFL in both eyes in AD group were thinner, the differences were significant (P＜0.05); the temporal (t=1.966, 0.838）and nasal (t=2.071, 0.916) thickness of RNFL in both eyes of AD group were thinner, but the difference was not statistically significant (P＞0.05). There was no significant difference of the mean and different quadrant RNFL thickness between different eyes in AD group and normal control group (AD group: t=0.097, 0.821, 0.059, 0.020, 0.116; normal control group: t=0.791, 1.938, 1.806, 2.058, 1.005; P＞0.05). Conclusion The RNFL thickness around the optic disc in AD patients is thinner; This occurs first in superior and inferior quadrants of the optic disc.
Neurofibromatosis type 1 (NF1) is an autosomal dominant genetic disease caused by mutations in the NF1 gene. The disease is characterized by neurofibromatosis, which simultaneously affects multiple systems such as nerves, skin, and bone, and has complex clinical manifestations. Since the National Institutes of Health (NIH) established diagnostic criteria in 1988, the diagnosis and treatment of NF1 have progressed significantly. However, due to the complexity of the disease and the lack of effective treatments, the diagnosis and treatment of NF1 still face many challenges. Strengthening multidisciplinary collaboration, improving and popularizing disease diagnosis and treatment strategies, and developing more effective drugs and treatment methods are the keys to further improve the treatment level of NF1 diseases.
Objective To compare the differences of optic disc parameters and the thickness of circumpapillary retinal nerve fiber layer (CP-RNFL) in acute uveitis Vogt-Koyanagi-Harada (VKH) syndrome with different degrees of optic disc edema. Methods This is a retrospective study including 40 eyes of 23 VKH patients. The eyes were divided into 2 groups according to the results of fluorescein fundus angiography (FFA). The discs with hyperfluorescence but no leakage were in mild optic disc edema group (group A, 13 patients and 25 eyes), and the discs with hyperfluorescence and leakage were in severe optic disc edema group (group B, 10 patients and 15 eyes). The patients were significantly older in group B than in group A (t=−2.17, P＜0.05). The differences of gender, diseased time (t=−1.67) and corrected visual acuity (t=−0.76) between 2 groups were not significant (P＞0.05). Eighty eyes of 46 normal healthy subjects, matching group A and group B with age and gender, were divided into group C (26 subjects and 50 eyes) and group D (20 subjects and 30 eyes) respectively. All subjects underwent three dimensional optical coherence tomography examinations. The parameters included average thickness of entire circumpapillary retinal nerve fiber layer (CP-RNFL), thickness of nasal, superior, temporal and inferior quadrant of CP-RNFL, disc area, disc cup area, rim area, cup/disc (C/D) area ratio, C/D horizontal diameter ratio and C/D vertical diameter ratio. Results The disc area, disc cup area and rim area were bigger, the thickness of mean CP-RNFL and the nasal and temporal quadrants of CP-RNFL were thicker in group A than those in group C (P＜0.05). The disc area and rim area were bigger, the thickness of mean CP-RNFL and the nasal, superior, temporal and inferior quadrant of CP-RNFL were thicker, C/D area ratio and C/D diameter ratio were smaller in group B than those in group D (P＜0.05). The disc area was bigger, disc cup area, C/D area ratio and C/D diameter ratio were smaller, the thickness of mean CP-RNFL and the nasal , superior and inferior quadrants of CP-RNFL were thicker in group B than those in group A (P＜0.05). Conclusions Acute VKH uveitis with mild optic disc edema has thicker CP-RNFL in the nasal and temporal quadrants; with severe optic disc edema has thicker CP-RNFL in all 4 quadrants. Acute VKH uveitis also has smaller C/D area ratio and C/D diameter ratio.
Objective To determine the long-term changes in optic disc parameter and the thickness of circumpapillary retinal nerve fiber layer (CP-RNFL) and macular retina after acute primary angle closure. Methods Prospective clinical case-control study. A total of 26 patients (30 eyes) with acute primary angle-closure glaucoma (APACG) were in the APACG group, whose intraocular pressure were control after a single episode acute primary angle closure; 30 age-and sex-matched healthy subjects (30 eyes) in the control group. All subjects underwent three dimensional optical coherence tomography (3D-OCT) examination with 3D optic disk scanning or circle optic disk scanning and 6 mm×6 mm macular scanning. The parameters included average thickness of entire CP-RNFL, thickness of nasal, superior, temporal and inferior quadrant of CP-RNFL, disc area, disc cup area, rim area, cup/disc (C/D) area ratio, C/D horizontal diameter ratio and C/D vertical diameter ratio. The foveal retinal thickness, center retinal thickness (≤1 mm from the fovea), 4 quadrants of macular inner-ring ( > 1 mm but≤3 mm from the fovea) retinal thickness, 4 quadrants of macular outer-ring ( > 3 mm but≤6 mm from the fovea) retinal thickness, average thickness of macular retinal thickness and macular volume were measured and analyzed. Results The disc area, disc cup area, C/D area ratio, C/D horizontal diameter ratio and C/D vertical diameter ratio in APACG group were significantly bigger than the control group (t=3.22, 4.12, 3.90, 3.00, 3.23; P < 0.05), rim area was smaller than the control group (t=-2.63, P < 0.05). The average thickness (t=-6.68) and the thickness of superior (t=-5.90), temporal (t=-11.64) and inferior (t=-5.06) quadrants of CP-RNFL, center retinal thickness (t=-2.50), 4 quadrants of macular inner-ring retinal thickness (t=-4.91, -4.88, -2.83, -3.59), nasal (t=-2.13) and superior (t=-2.49) quadrants of macular outer-ring retinal thickness as well as average thickness of macular retinal thickness (t=-2.65) were significantly thinner than the control group (P < 0.05), and the macular volume (t=-2.69) was significantly smaller than the control group (P < 0.05). There was no statistically difference at nasal CP-RNFL (t=-0.11), foveal retinal thickness (t=-0.59), temporal (t=-0.67) and inferior (t=-1.02) quadrants of macular outer-ring retinal thickness between two groups (P > 0.05). Conclusions In comparison with the healthy subjects, the disc area, disc cup area, C/D area ratio, C/D horizontal diameter ratio, C/D vertical diameter ratio in APACG eyes were bigger, while rim area was smaller; the CP-RNFL and macular retinal thickness were thinner except nasal CP-RNFL, fovea, temporal and inferior quadrants of macular outer-ring retinal.
ObjectiveTo summarize the treatment strategies and clinical experiences of 5 cases of giant plexiform neurofibromas (PNF) involving the head, face, and neck. MethodsBetween April 2021 and May 2023, 5 patients with giant PNFs involving the head, face, and neck were treated, including 1 male and 4 females, aged 6-54 years (mean, 22.4 years). All tumors showed progressive enlargement, involving multiple regions such as the maxillofacial area, ear, and neck, significantly impacting facial appearance. Among them, 3 cases involved tumor infiltration into deep tissues, affecting development, while 4 cases were accompanied by hearing loss. Imaging studies revealed that all 5 tumors predominantly exhibited an invasive growth pattern, in which 2 and 1 also presenting superficial and displacing pattern, respectively. The surgical procedure followed a step-by-step precision treatment strategy based on aesthetic units, rather than simply aiming for maximal tumor resection in a single operation. Routine preoperative embolization of the tumor-feeding vessels was performed to reduce bleeding risk, followed by tumor resection combined with reconstructive surgery. Results All 5 patients underwent 1-3 preoperative embolization procedures, with no intraoperative hemorrhagic complications reported. Four patients required intraoperative blood transfusion. A total of 10 surgical procedures were performed across the 5 patients. One patient experienced early postoperative flap margin necrosis due to ligation for hemostasis; however, the incisions in the remaining patients healed without complications. All patients were followed up for a period ranging from 6 to 36 months, with a mean follow-up duration of 21.6 months. No significant tumor recurrence was observed during the follow-up period. Conclusion For patients with giant PNF involving the head, face, and neck, precision treatment strategy can effectively control surgical risks and improve the standard of aesthetic reconstruction. This approach enhances overall treatment outcomes by minimizing complications and optimizing functional and cosmetic results.
ObjectiveTo compare the differences of optic nerve head (ONH) parameters and the thickness of circumpapillary retinal nerve fiber layer (CP-RNFL) between acute Vogt-Koyanagi-Harada syndrome (VKH) and acute central serous chorioretinopathy (CSC) patients.MethodsRetrospective clinical case control analysis. This study included 38 eyes of 20 acute VKH patients (VKH group) and 37 eyes of 37 acute CSC patients (CSC group). Seventy five eyes of 57 normal healthy subjects, matching patients with age and gender, were collected as control group. The disc RPE angle, the thickness of average CP-RNFL, the nasal, superior, temporal and inferior quadrant CP-RNFL thickness, and ONH parameters including optic disc area, cup area, rim area, C/D area ratio, linear CD ratio (CDR), vertical CDR were measured by 3D-OCT. Analysis of variance was performed for comparison among three groups. Minimum significant difference t test was performed for comparison between two groups.ResultsThe differences of ONH parameters between VKH group and CSC group: 29 eyes of VKH group appeared retinal detachment next to disc, only 12 eyes appeared in CSC group. Twenty one eyes of VKH group appeared optic disc hyperemia while none in CSC group. The three groups’ disc RPE angles were (138.62±11.96)°, (154.09±5.85)° and (153.41±5.77)°. VKH group were significantly smaller than CSC group (t=－2.05, P=0.00) and control group (t=－1.68, P=0.00), while there was no significant difference between CSC group and control group (t=－1.88, P=0.72). The optic cup area and rim area were significantly bigger in VKH group than in CSC group (t=4.61, 2.71; P=0.00, 0.01), and the thickness of mean CP-RNFL, all quadrants of CP-RNFL were significantly thicker in VKH group than in CSC group (t=6.25, 4.40, 3.53, 5.48, 2.69; P=0.00, 0.00, 0.00, 0.00, 0.01).ConclusionCompared with the acute CSC, VKH patients are likely to appear retinal detachment next to disc, their disc RPE angles are smaller, their optic cup area and rim area are bigger, and their CP-RNFL thickness are thicker.
Objective To analyze the thickness of peripapillary retinal nerve fiber layer (pRNFL) and photoreceptor (PR) sublayer in Leber hereditary optic neuropathy (LHON) and G11778A mutation carriers. MethodsA cross sectional study. From September 2020 to October 2021, 68 LHON patients (136 eyes) (patient group) and 40 G11778A mutation carriers (80 eyes) of LHON patients' families (carrier group) were included in the study. All patients were found to have G11778A mutation by Genetic testing. Forty healthy volunteers with 80 eyes matched to the age and gender of the patient group were recruited as a normal control group. All eyes were examined by optical coherence tomography (OCT). The pRNFL thickness was automatically measured by the built-in software of the OCT device. The total retinal thickness (MT) and the thickness of the outer bundle layer (OPL), outer nuclear layer (ONL), external limiting membrane to retinal pigment epithelium (ELM-RPE) in macular OCT images were measured by Image J software. Linear mixed model was used to analyze and compare the thickness of pRNFL, macular fovea and four layers above the nasal and temporal paracentral retina in patients, carriers and normal controls. The correlation between pRNFL and macular retinal sublayer thickness and the course of disease was also analyzed. ResultsThe thickness of the upper and lower pRNFL, temporal pRNFL and average pRNFL of the patients were smaller than those of the carriers and the normal control group (P＜0.01), and the nasal pRNFL thickness of the patients was smaller than that of the carriers (P＜0.01). Fovea: compared with the normal control group, the thickness of MT and ONT in the patient group was decreased, ONL thickness decreased in carrier group, with the significant different (P＜0.05). Parafovea: compared with normal control group, the thickness of MT and temporal ONL decreased and temporal OPL increased in the patients group, with the significant different (P＜0.05). In the carrier group, the thickness of MT and temporal, nasal ONL decreased, and the thickness of nasal OPL increased, with the significant different (P＜0.05). Compared with the carrier group, the MT thickness of the patient group was decreased, and the nasal ONL and nasal ELM-RPE thickness were increased, with the significant different (P＜0.05). Correlation analysis results showed that the thinning of pRNFL in the superior, nasal, temporal and average (r=－0.22, －0.21, －0.25, －0.22), and the thickening of ELM-RPE in foveo-temporal (r=0.19) were correlated with the course of disease (P＜0.05). ConclusionsThe pRNFL of LHON patients with G11778A mutation becomes thinner and is related to the course of the disease. There were significant differences in the thickness of MT and PR sublayers between patients and carriers compared to the normal control group.
ObjectiveTo investigate the relationship between optic disc hemorrhage and localized retinal never fiber layer defects (RNFLDs) in norma l tension glaucoma.MethodsIn 83 patients with normal-tension glaucoma, the cumulative frequency and quadrantal distribution of optic disc hemorrhages were retrospectively analyzed. The neighboring relation between optic disc hemorrhages and RNFLDs in a same quadrant and the changes of correspondin gretinal never fiber layer (RNFL) after the occurrence of optic disc hemorrhages were observed by tridimensional photochromy of ocular fundus.Results(1) The occurrences and distribution of optic disc hemorrhages: 29of83(34.94%) patients (33 eyes) had totally 58 occurrences, including 39 in infer iotemporal area, 14 in superiotemporal area, and 5 in other area. (2) The relati onship of neighborhood between optic disc hemorrhages and RNFLDs: in the availab le tridimensional photochrome, 23 occurrences in 15 patients (16 eyes) were foun d with cuneiform RNFLDs in the same quadrant, in which 22 was near the border of cuneiform RNFLDs. (3) The changes of corresponding retinal never fiber layer (R NFL) after the occurrence of optic disc hemorrhages: the photochromes of 24 occurrences in 20 patients (21 eyes) were kept well in the initial and the 2-year follow-up periods, while the changes of RNFL were found in each region correspon ding to the 19 occurrences (in inferiotemporal or superiotemporal area) in the initial photochrome, including 7 cuneiform defects with various sizes, and 12 developed localized RNFLDs next to the initial hemorrhages in the optic disc. No obvious localized RNFL corresponding to the other 5 occurrences (1 in inferiotempo ral, 1 in superiotemporal, and 3 in other areas) were found in the follow up period.ConclusionOptic disc hemorrhages in normal-tension glaucoma occur mostly in inferiotemporal area, and secondly in superiotemporal area of optic disc, and the appearance of optic disc hemorrhages may suggest that the localized RNFLDS would develop in the associated regions.(Chin J Ocul Fundus Dis，2004，20：339-342)
Twenty adult healthy rabbits were divided into 2 groups at random. Under the identical experimental conditions, cut the trunk of the right facial nerve. Two weeks later. the caudal end of the divided facial nerve was anastomized with the cephalad end of the divided main trunk of the nerve to the masseter muscle. The experimental group was stimulated by music, twenty minutes of each time, three times a day. From the results it was noted that the rate of growth of the nerve in the experimental group was more rapid than that of the contril group.