Plexiform angiomyxoid myofibroblastic tumor of the stomach is a unique mesenchymal tumor that we first described in 2007.The tumor is very rare,and to date,only 18 cases confirmed by immunohistochemistry have been rep...Plexiform angiomyxoid myofibroblastic tumor of the stomach is a unique mesenchymal tumor that we first described in 2007.The tumor is very rare,and to date,only 18 cases confirmed by immunohistochemistry have been reported in the literature.The patients' ages ranged from 7 to 75 years(mean,43 years),and the male-to-female ratio was approximately 1:1.Representative clinical symptoms are ulceration,associated upper gastrointestinal bleeding(hematemesis),and anemia.The tumors are located at the antrum in all cases,and grossly,the tumor is whitish to brownish or reddish,and forms a lobulated submucosal or transmural mass.Microscopically,the tumor is characterized by a plexiform growth pattern,the proliferation of cytologically bland spindle cells,and a myxoid stroma that is rich in small vessels and positive for Alcian blue stain.Immunohistochemically,the tumor cells are positive for α-smooth muscle actin and negative for KIT and CD34.Differential diagnoses include gastrointestinal stromal tumor and other mesenchymal tumors of the gastrointestinal tract.Some authors proposed that this tumor should be designated as "plexiform fibromyxoma",but this designation might cause confusion.The tumor is probably benign and thus far,neither recurrence nor metastasis has been reported.展开更多
BACKGROUND Plexiform fibromyxoma is a rare, special type of mesenchymal tumor. The most common presenting symptoms are anemia, hematemesis, and hematochezia, without sex or age predilection. The reported cases have ma...BACKGROUND Plexiform fibromyxoma is a rare, special type of mesenchymal tumor. The most common presenting symptoms are anemia, hematemesis, and hematochezia, without sex or age predilection. The reported cases have mainly occurred in the gastric antrum and pylorus region, with some cases in the duodenum. CASE SUMMARY We report here a case of plexiform fibromyxoma in the upper segment of the jejunum, which was continuously followed up for 3 years after surgical removal. Plexiform fibromyxoma showed multinodular or plexiform growth. The cells in the tumor node were spindle-shaped but few in number and mitotic figures. Small blood vessels and mucous matrix were found among the tumor cells. Immunohistochemistry revealed that the plexiform fibromyxoma cells were positive for smooth muscle actin, focally positive for CD10, and negative for cytokeratin, CD117, DOG-1(discovered on GIST-1) desmin, S-100, epithelial membrane antigen, and CD34. Ki-67 labeling index was < 5%. Plexiform fibromyxoma showed benign biological behavior. After 3 years of consecutive postoperative follow-up, no obvious signs of metastasis or recurrence were found by imaging examination. CONCLUSION Plexiform fibromyxoma is a rare type of mesenchymal tumor. The diagnosis mainly depends on pathological examination, and it should be distinguished from other gastrointestinal mesenchymal tumors.展开更多
Neurofibromatosis type 1(NF1)is one of the most common genetic disorders that predisposes patients to benign and malignant tumors of the peripheral nervous system.Plexiform and cutaneous neurofibromas are NF1-associat...Neurofibromatosis type 1(NF1)is one of the most common genetic disorders that predisposes patients to benign and malignant tumors of the peripheral nervous system.Plexiform and cutaneous neurofibromas are NF1-associated benign tumors.Despite their benign nature,they can cause tremendous morbidity in patients with NF1.Therapeutic drug options are limited to the MEK inhibitor,selumetinib,which is the only approved drug for pediatric patients with plexiform neurofibromas.Antifibrotic strategies have substantial therapeutic potential for NF1-associated neurofibromas.This review discusses the fibrotic features of plexiform and cutaneous neurofi-bromas focusing on the pathological composition of the extracellular matrix.It also highlights the core pathways implicated in the biochemical and biophysical regulation of the extracellular matrix remodeling in tumor imitation and progression.Finally,this review provides a brief outlook on how exploring novel vulnerabilities residing in the aberrant extracellular matrix and their underlying pathways can benefit the treatment of NF1-associated neurofibromas.展开更多
AIM:To assess the performance of macular ganglion cell-inner plexiform layer thickness(mGCIPLT)and 10-2 visual field(VF)parameters in detecting early glaucoma and evaluating the severity of advanced glaucoma.METHODS:T...AIM:To assess the performance of macular ganglion cell-inner plexiform layer thickness(mGCIPLT)and 10-2 visual field(VF)parameters in detecting early glaucoma and evaluating the severity of advanced glaucoma.METHODS:Totally 127 eyes from 89 participants(36 eyes of 19 healthy participants,45 eyes of 31 early glaucoma patients and 46 eyes of 39 advanced glaucoma patients)were included.The relationships between the optical coherence tomography(OCT)-derived parameters and VF sensitivity were determined.Patients with early glaucoma were divided into eyes with or without central 10°of the VF damages(CVFDs),and the diagnostic performances of OCT-derived parameters were assessed.RESULTS:In early glaucoma,the mGCIPLT was significantly correlated with 10-2 VF pattern standard deviation(PSD;with average mGCIPLT:β=-0.046,95%CI,-0.067 to-0.024,P<0.001).In advanced glaucoma,the mGCIPLT was related to the 24-2 VF mean deviation(MD;with average mGCIPLT:β=0.397,95%CI,0.199 to 0.595,P<0.001),10-2 VF MD(with average mGCIPLT:β=0.762,95%CI,0.485 to 1.038,P<0.001)and 24-2 VF PSD(with average mGCIPLT:β=0.244,95%CI,0.124 to 0.364,P<0.001).Except for the minimum and superotemporal mGCIPLT,the decrease of mGCIPLT in early glaucomatous eyes with CVFDs was more severe than that of early glaucomatous eyes without CVFDs.The area under the curve(AUC)of the average mGCIPLT(AUC=0.949,95%CI,0.868 to 0.982)was greater than that of the average circumpapillary retinal nerve fiber layer thickness(cpRNFLT;AUC=0.827,95%CI,0.674 to 0.918)and rim area(AUC=0.799,95%CI,0.610 to 0.907)in early glaucomatous eyes with CVFDs versus normal eyes.CONCLUSION:The 10-2 VF and mGCIPLT parameters are complementary to 24-2 VF,cpRNFLT and ONH parameters,especially in detecting early glaucoma with CVFDs and evaluating the severity of advanced glaucoma in group level.展开更多
The integrity of retinal ganglion cells is tightly associated with diabetic macular degeneration that leads to damage and death of retinal ganglion cells,affecting vision.The major clinical treatments for diabetic mac...The integrity of retinal ganglion cells is tightly associated with diabetic macular degeneration that leads to damage and death of retinal ganglion cells,affecting vision.The major clinical treatments for diabetic macular edema are anti-vascular endothelial growth factor drugs and laser photocoagulation.However,although the macular thickness can be normalized with each of these two therapies used alone,the vision does not improve in many patients.This might result from the incomplete recovery of retinal ganglion cell injury.Therefore,a prospective,non-randomized,controlled clinical trial was designed to investigate the effect of anti-vascular endothelial growth factor drugs combined with laser photocoagulation on the integrity of retinal ganglion cells in patients with diabetic macular edema and its relationship with vision recovery.In this trial,150 patients with diabetic macular edema will be equally divided into three groups according to therapeutic methods,followed by treatment with anti-vascular endothelial growth factor drugs,laser photocoagulation therapy,and their combination.All patients will be followed up for 12 months.The primary outcome measure is retinal ganglion cell-inner plexiform layer thickness at 12 months after treatment.The secondary outcome measures include retinal ganglion cell-inner plexiform layer thickness before and 1,3,6,and 9 months after treatment,retinal nerve fiber layer thickness,best-corrected visual acuity,macular area thickness,and choroidal thickness before and 1,3,6,9,and 12 months after treatment.Safety measure is the incidence of adverse events at 1,3,6,9,and 12 months after treatment.The study protocol hopes to validate the better efficacy and safety of the combined treatment in patients with diabetic macula compared with the other two monotherapies alone during the 12-month follow-up period.The trial is designed to focus on clarifying the time-effect relationship between imaging measures related to the integrity of retinal ganglion cells and best-corrected visual acuity.展开更多
AIM:To compare the macular ganglion cell-inner plexiform layer(GCIPL)thickness,retinal nerve fiber layer(RNFL)thickness,optic nerve head(ONH)parameters,and retinal vessel density(VD)measured by spectral-domain optical...AIM:To compare the macular ganglion cell-inner plexiform layer(GCIPL)thickness,retinal nerve fiber layer(RNFL)thickness,optic nerve head(ONH)parameters,and retinal vessel density(VD)measured by spectral-domain optical coherence tomography(SD-OCT)and analyze the correlations between them in the early,moderate,severe primary angle-closure glaucoma(PACG)and normal eyes.METHODS:Totally 70 PACG eyes and 20 normal eyes were recruited for this retrospective analysis.PACG eyes were further separated into early,moderate,or severe PACG eyes using the Enhanced Glaucoma Staging System(GSS2).The GCIPL thickness,RNFL thickness,ONH parameters,and retinal VD were measured by SD-OCT,differences among the groups and correlations within the same group were calculated.RESULTS:The inferior and superotemporal sectors of the GCIPL thickness,rim area of ONH,average and inferior sector of the retinal VD were significantly reduced(all P<0.05)in the early PACG eyes compared to the normal and the optic disc area,cup to disc ratio(C/D),and cup volume were significantly higher(all P<0.05);but the RNFL was not significant changes in early and moderate PACG.In severe group,the GCIPL and RNFL thickness were obvious thinning with retinal VD were decreasing as well as C/D and cup volume increasing than other three groups(all P<0.01).In the early PACG subgroup,there were significant positive correlations between retinal VD and GCIPL thickness(except superonasal and inferonasal sectors,r=0.573 to 0.641,all P<0.05),superior sectors of RNFL thickness(r=0.055,P=0.049).More obvious significant positive correlations were existed in moderate PACG eyes between retinal VD and superior sectors of RNFL thickness(r=0.650,P=0.022),and temporal sectors of RNFL thickness(r=0.740,P=0.006).In the severe PACG eyes,neither GCIPL nor RNFL thickness was associated with retinal VD.CONCLUSION:The ONH damage and retinal VD loss appears earlier than RNFL thickness loss in PACG eyes.As the PACG disease progressed from the early to the moderate stage,the correlations between展开更多
A 66-year-old man was diagnosed with a gastric submucosal tumor. Endoscopic ultrasound(EUS) revealed an iso/hypoechoic mass in the third layer. No malignant cells were detected in a histological examination. Yearly fo...A 66-year-old man was diagnosed with a gastric submucosal tumor. Endoscopic ultrasound(EUS) revealed an iso/hypoechoic mass in the third layer. No malignant cells were detected in a histological examination. Yearly follow-up endoscopy and EUS showed the slow growth of the tumor. Endoscopic submucosal dissection(ESD) was performed and a glistening tumor was resected. The lesion showed a multinodular plexiform growth pattern consisting of spindle cells with an abundant fibromyxoid stroma that was rich in small vessels. The tumor was diagnosed as plexiform fibromyxoma(PF) by immunohistochemistry. Although difficulties are associated with reaching a diagnosis preoperatively, chronological changes on EUS may contribute to the diagnosis of PF. ESD may also be useful in the diagnosis and treatment of PF.展开更多
AIM: To determine the discriminating performance of the macular ganglion cell-inner plexiform layer(GCIPL) parameters between all the consecutive stages of glaucoma(from healthy to moderate-to-severe glaucoma), and to...AIM: To determine the discriminating performance of the macular ganglion cell-inner plexiform layer(GCIPL) parameters between all the consecutive stages of glaucoma(from healthy to moderate-to-severe glaucoma), and to compare it with the discriminating performances of the peripapillary retinal nerve fiber layer(RNFL) parameters and optic nerve head(ONH) parameters.METHODS: Totally 147 eyes(40 healthy, 40 glaucoma suspects, 40 early glaucoma, and 27 moderate-to-severe glaucoma) of 133 subjects were included. Optical coherence tomography(OCT) was obtained using Cirrus HD-OCT 5000. The diagnostic performances of GC-IPL, RNFL, and ONH parameters were evaluated by determining the area under the curve(AUC) of the receiver operating characteristics. RESULTS: All GC-IPL parameters discriminated glaucoma suspect patients from subjects with healthy eyes and moderate-to-severe glaucoma from early glaucoma patients(P<0.017, for all). Also, minimum, inferotemporal and inferonasal GC-IPL parameters discriminated early glaucoma patients from glaucoma suspects, whereas no RNFL or ONH parameter could discriminate between the two. The best parameters to discriminate glaucoma suspects from subjects with healthy eyes were superonasal GC-IPL, superior RNFL and average c/d ratio(AUC=0.746, 0.810 and 0.746, respectively). Discriminating performances of all the parameters for early glaucoma vs glaucoma suspect comparison were lower than that of the other consecutive group comparisons, with the bestGC-IPL parameters being minimum and inferotemporal(AUC=0.669 and 0.662, respectively). Moreover, minimum GC-IPL, average RNFL, and rim area(AUC=0.900, 0.858, 0.768, respectively) were the best parameters for discriminating moderate-to-severe glaucoma patients from early glaucoma patients.CONCLUSION: GC-IPL parameters can discriminate glaucoma suspect patients from subjects with healthy eyes, and also all the consecutive stages of glaucoma from each other(from glaucoma suspect to moderate-tosevere glaucoma). Further, the discriminating performa展开更多
Plexiform schwannoma is an extremely rare variant of schwannoma, accounting for approximately 5% of cases. Due to the rarity and lack of typical symptoms, signs and radiological images, a definite diagnosis of plexifo...Plexiform schwannoma is an extremely rare variant of schwannoma, accounting for approximately 5% of cases. Due to the rarity and lack of typical symptoms, signs and radiological images, a definite diagnosis of plexiform schwannoma may not be made by clinicians prior to biopsy. In the present study, we report the first case(to our knowledge) of perianal plexiform schwannoma arising from the overlapped skin of the ischioanal fossa, and we propose an intratumorally nonenhanced circumferential capsule dividing the tumour into multiple homogeneously enhanced nodules as a magnetic resonance imaging feature to aid in the differential diagnosis of plexiform schwannoma from ancient schwannoma, cavernous haemangioma, liposarcoma and plexiform neurofibroma.展开更多
Plexiform angiomyxoid myofibroblastic tumor(PAMT) is a rare benign mesenchymal tumor of stomach. Rarity of this kind of tumors and scarce review articles may cause underrecognition of this entity and pose a real diagn...Plexiform angiomyxoid myofibroblastic tumor(PAMT) is a rare benign mesenchymal tumor of stomach. Rarity of this kind of tumors and scarce review articles may cause underrecognition of this entity and pose a real diagnostic challenge to gastroenterologists, pathologists and surgeons when encountering such patients and differentiating PAMT from other gastric intramural tumors. We report a case of 28-year-old woman, who presented with epigastric pain after meals, iron-deficiency anaemia and weight loss. Upper gastrointestinal endoscopy revealed submucosal tumorlike elevated lesion in the anterior wall of the antrum with intact overlying mucosa. Endoscopic ultrasound showed a 3-cm hypoechoic homogenous mass, originating from the third layer of the gastric wall. Endoscopic ultrasound-guided fine needle aspiration was not informative. Endoscopic buttonhole biopsy was performed to obtain specimens. Following this, the unexpected prolapse of the tumor occurred into the lumen of the stomach, causing gastric outlet obstruction- the biopsy was obtained. Pathomorphological features suggested the diagnosis of PAMT. Gastric resection of the Billroth I type was performed. Diagnosis was confirmed by histological analysis of the surgical specimen.展开更多
AIM: To evaluate the patterns of macular ganglion cell-inner plexiform layer(GCIPL) loss in normal tension glaucoma(NTG) and primary open angle glaucoma(POAG) in a detailed, disease severity-matched way;and to assess ...AIM: To evaluate the patterns of macular ganglion cell-inner plexiform layer(GCIPL) loss in normal tension glaucoma(NTG) and primary open angle glaucoma(POAG) in a detailed, disease severity-matched way;and to assess the diagnostic capabilities of GCIPL thickness parameters in discriminating NTG or POAG from normal subjects.METHODS: A total of 157 eyes of 157 subjects, including 57 normal eyes, 51 eyes with POAG and 49 eyes with NTG were enrolled and strictly matched in age, refraction, and disease severity between POAG and NTG groups. The average, minimum, superotemporal, superior, superonasal, inferonasal, inferior, and inferotemporal GCIPL thickness, and the average, superior, temporal, inferior, and nasal retinal nerve fiber layer(RNFL) thickness were obtained by Cirrus optical coherence tomography(OCT). The diagnostic capabilities of OCT parameters were assessed by area under receiver operating characteristic(AUROC) curves. RESULTS: Among all the OCT thickness parameters, no statistical significant difference between NTG group and POAG group was found(all P>0.05). In discriminating NTG or POAG from normal subjects, the average and inferior RNFL thickness, and the minimum GCIPL thickness had better diagnostic capabilities. There was no significant difference in AUROC curve between the best GCIPL thickness parameter(minimum GCIPL) and the best RNFL thickness parameter in discriminating NTG(inferior RNFL;P=0.076) and indiscriminating POAG(average RNFL;P=0.913) from normal eyes.CONCLUSION: Localized GCIPL loss, especially in the inferior and inferotemporal sectors, is more common in NTG than in POAG. Among all the GCIPL thickness parameters, the minimum GCIPL thickness has the best diagnostic performance in differentiating NTG or POAG from normal subjects, which is comparable to that of the average and inferior RNFL thickness.展开更多
Plexiform fibromyxoma is a very rare mesenchymal tumor of the stomach, found almost exclusively in the antrum/pylorus region. The most common presenting symptoms are anemia, hematemesis, nausea and unintentional weigh...Plexiform fibromyxoma is a very rare mesenchymal tumor of the stomach, found almost exclusively in the antrum/pylorus region. The most common presenting symptoms are anemia, hematemesis, nausea and unintentional weight loss, without sex or age predilection. We describe here two cases of plexiform fibromyxoma, involving a 16-year-old female and a 34-year-old male. Both patients underwent complete resection(R0) by distal gastrectomy and retrocolic gastrojejunostomy(according to Billroth 2); for both, the postoperative course was uneventful. Histology showed multiple intramural and subserosal nodules with characteristic plexiform growth, featuring bland spindle cells situated in an abundant myxoid stroma with low mitotic activity. Immunohistochemistry showed α-smooth muscle actin-positive spindle cells, focal positivity for CD10, and negative staining for KIT, DOG1, CD34, S100, β-catenin, STAT-6 and anaplastic lymphoma kinase. One of the cases showed focal positivity for h-caldesmon and desmin. Upon followup, no sign of disease was found. In the differential diagnosis of plexiform fibromyxoma, it is important to exclude the more common gastrointestinal stromal tumors as they have greater potential for aggressivebehavior. Other lesions, like neuronal and vascular tumors, inflammatory fibroid polyps, abdominal desmoid-type fibromatosis, solitary fibrous tumors and smooth muscle tumors, must also be excluded.展开更多
文摘Plexiform angiomyxoid myofibroblastic tumor of the stomach is a unique mesenchymal tumor that we first described in 2007.The tumor is very rare,and to date,only 18 cases confirmed by immunohistochemistry have been reported in the literature.The patients' ages ranged from 7 to 75 years(mean,43 years),and the male-to-female ratio was approximately 1:1.Representative clinical symptoms are ulceration,associated upper gastrointestinal bleeding(hematemesis),and anemia.The tumors are located at the antrum in all cases,and grossly,the tumor is whitish to brownish or reddish,and forms a lobulated submucosal or transmural mass.Microscopically,the tumor is characterized by a plexiform growth pattern,the proliferation of cytologically bland spindle cells,and a myxoid stroma that is rich in small vessels and positive for Alcian blue stain.Immunohistochemically,the tumor cells are positive for α-smooth muscle actin and negative for KIT and CD34.Differential diagnoses include gastrointestinal stromal tumor and other mesenchymal tumors of the gastrointestinal tract.Some authors proposed that this tumor should be designated as "plexiform fibromyxoma",but this designation might cause confusion.The tumor is probably benign and thus far,neither recurrence nor metastasis has been reported.
文摘BACKGROUND Plexiform fibromyxoma is a rare, special type of mesenchymal tumor. The most common presenting symptoms are anemia, hematemesis, and hematochezia, without sex or age predilection. The reported cases have mainly occurred in the gastric antrum and pylorus region, with some cases in the duodenum. CASE SUMMARY We report here a case of plexiform fibromyxoma in the upper segment of the jejunum, which was continuously followed up for 3 years after surgical removal. Plexiform fibromyxoma showed multinodular or plexiform growth. The cells in the tumor node were spindle-shaped but few in number and mitotic figures. Small blood vessels and mucous matrix were found among the tumor cells. Immunohistochemistry revealed that the plexiform fibromyxoma cells were positive for smooth muscle actin, focally positive for CD10, and negative for cytokeratin, CD117, DOG-1(discovered on GIST-1) desmin, S-100, epithelial membrane antigen, and CD34. Ki-67 labeling index was < 5%. Plexiform fibromyxoma showed benign biological behavior. After 3 years of consecutive postoperative follow-up, no obvious signs of metastasis or recurrence were found by imaging examination. CONCLUSION Plexiform fibromyxoma is a rare type of mesenchymal tumor. The diagnosis mainly depends on pathological examination, and it should be distinguished from other gastrointestinal mesenchymal tumors.
文摘Neurofibromatosis type 1(NF1)is one of the most common genetic disorders that predisposes patients to benign and malignant tumors of the peripheral nervous system.Plexiform and cutaneous neurofibromas are NF1-associated benign tumors.Despite their benign nature,they can cause tremendous morbidity in patients with NF1.Therapeutic drug options are limited to the MEK inhibitor,selumetinib,which is the only approved drug for pediatric patients with plexiform neurofibromas.Antifibrotic strategies have substantial therapeutic potential for NF1-associated neurofibromas.This review discusses the fibrotic features of plexiform and cutaneous neurofi-bromas focusing on the pathological composition of the extracellular matrix.It also highlights the core pathways implicated in the biochemical and biophysical regulation of the extracellular matrix remodeling in tumor imitation and progression.Finally,this review provides a brief outlook on how exploring novel vulnerabilities residing in the aberrant extracellular matrix and their underlying pathways can benefit the treatment of NF1-associated neurofibromas.
基金National Natural Science Foundation of China(No.81860170).
文摘AIM:To assess the performance of macular ganglion cell-inner plexiform layer thickness(mGCIPLT)and 10-2 visual field(VF)parameters in detecting early glaucoma and evaluating the severity of advanced glaucoma.METHODS:Totally 127 eyes from 89 participants(36 eyes of 19 healthy participants,45 eyes of 31 early glaucoma patients and 46 eyes of 39 advanced glaucoma patients)were included.The relationships between the optical coherence tomography(OCT)-derived parameters and VF sensitivity were determined.Patients with early glaucoma were divided into eyes with or without central 10°of the VF damages(CVFDs),and the diagnostic performances of OCT-derived parameters were assessed.RESULTS:In early glaucoma,the mGCIPLT was significantly correlated with 10-2 VF pattern standard deviation(PSD;with average mGCIPLT:β=-0.046,95%CI,-0.067 to-0.024,P<0.001).In advanced glaucoma,the mGCIPLT was related to the 24-2 VF mean deviation(MD;with average mGCIPLT:β=0.397,95%CI,0.199 to 0.595,P<0.001),10-2 VF MD(with average mGCIPLT:β=0.762,95%CI,0.485 to 1.038,P<0.001)and 24-2 VF PSD(with average mGCIPLT:β=0.244,95%CI,0.124 to 0.364,P<0.001).Except for the minimum and superotemporal mGCIPLT,the decrease of mGCIPLT in early glaucomatous eyes with CVFDs was more severe than that of early glaucomatous eyes without CVFDs.The area under the curve(AUC)of the average mGCIPLT(AUC=0.949,95%CI,0.868 to 0.982)was greater than that of the average circumpapillary retinal nerve fiber layer thickness(cpRNFLT;AUC=0.827,95%CI,0.674 to 0.918)and rim area(AUC=0.799,95%CI,0.610 to 0.907)in early glaucomatous eyes with CVFDs versus normal eyes.CONCLUSION:The 10-2 VF and mGCIPLT parameters are complementary to 24-2 VF,cpRNFLT and ONH parameters,especially in detecting early glaucoma with CVFDs and evaluating the severity of advanced glaucoma in group level.
基金supported by Science and Technology Research Project of Jilin Provincial Department of Education,No.JJKH20220072KJ(to XL)Science and Technology Development Program of Jilin Province,No.20200201495JC(to YL)。
文摘The integrity of retinal ganglion cells is tightly associated with diabetic macular degeneration that leads to damage and death of retinal ganglion cells,affecting vision.The major clinical treatments for diabetic macular edema are anti-vascular endothelial growth factor drugs and laser photocoagulation.However,although the macular thickness can be normalized with each of these two therapies used alone,the vision does not improve in many patients.This might result from the incomplete recovery of retinal ganglion cell injury.Therefore,a prospective,non-randomized,controlled clinical trial was designed to investigate the effect of anti-vascular endothelial growth factor drugs combined with laser photocoagulation on the integrity of retinal ganglion cells in patients with diabetic macular edema and its relationship with vision recovery.In this trial,150 patients with diabetic macular edema will be equally divided into three groups according to therapeutic methods,followed by treatment with anti-vascular endothelial growth factor drugs,laser photocoagulation therapy,and their combination.All patients will be followed up for 12 months.The primary outcome measure is retinal ganglion cell-inner plexiform layer thickness at 12 months after treatment.The secondary outcome measures include retinal ganglion cell-inner plexiform layer thickness before and 1,3,6,and 9 months after treatment,retinal nerve fiber layer thickness,best-corrected visual acuity,macular area thickness,and choroidal thickness before and 1,3,6,9,and 12 months after treatment.Safety measure is the incidence of adverse events at 1,3,6,9,and 12 months after treatment.The study protocol hopes to validate the better efficacy and safety of the combined treatment in patients with diabetic macula compared with the other two monotherapies alone during the 12-month follow-up period.The trial is designed to focus on clarifying the time-effect relationship between imaging measures related to the integrity of retinal ganglion cells and best-corrected visual acuity.
基金Supported by the Youth National Natural Science Foundation of China(No.81700800,No.81800800)the Natural Science Foundation of Shandong Province(No.ZR2017MH008)Taishan Scholar Project of Shandong Province(No.tsqn201812151)。
文摘AIM:To compare the macular ganglion cell-inner plexiform layer(GCIPL)thickness,retinal nerve fiber layer(RNFL)thickness,optic nerve head(ONH)parameters,and retinal vessel density(VD)measured by spectral-domain optical coherence tomography(SD-OCT)and analyze the correlations between them in the early,moderate,severe primary angle-closure glaucoma(PACG)and normal eyes.METHODS:Totally 70 PACG eyes and 20 normal eyes were recruited for this retrospective analysis.PACG eyes were further separated into early,moderate,or severe PACG eyes using the Enhanced Glaucoma Staging System(GSS2).The GCIPL thickness,RNFL thickness,ONH parameters,and retinal VD were measured by SD-OCT,differences among the groups and correlations within the same group were calculated.RESULTS:The inferior and superotemporal sectors of the GCIPL thickness,rim area of ONH,average and inferior sector of the retinal VD were significantly reduced(all P<0.05)in the early PACG eyes compared to the normal and the optic disc area,cup to disc ratio(C/D),and cup volume were significantly higher(all P<0.05);but the RNFL was not significant changes in early and moderate PACG.In severe group,the GCIPL and RNFL thickness were obvious thinning with retinal VD were decreasing as well as C/D and cup volume increasing than other three groups(all P<0.01).In the early PACG subgroup,there were significant positive correlations between retinal VD and GCIPL thickness(except superonasal and inferonasal sectors,r=0.573 to 0.641,all P<0.05),superior sectors of RNFL thickness(r=0.055,P=0.049).More obvious significant positive correlations were existed in moderate PACG eyes between retinal VD and superior sectors of RNFL thickness(r=0.650,P=0.022),and temporal sectors of RNFL thickness(r=0.740,P=0.006).In the severe PACG eyes,neither GCIPL nor RNFL thickness was associated with retinal VD.CONCLUSION:The ONH damage and retinal VD loss appears earlier than RNFL thickness loss in PACG eyes.As the PACG disease progressed from the early to the moderate stage,the correlations between
文摘A 66-year-old man was diagnosed with a gastric submucosal tumor. Endoscopic ultrasound(EUS) revealed an iso/hypoechoic mass in the third layer. No malignant cells were detected in a histological examination. Yearly follow-up endoscopy and EUS showed the slow growth of the tumor. Endoscopic submucosal dissection(ESD) was performed and a glistening tumor was resected. The lesion showed a multinodular plexiform growth pattern consisting of spindle cells with an abundant fibromyxoid stroma that was rich in small vessels. The tumor was diagnosed as plexiform fibromyxoma(PF) by immunohistochemistry. Although difficulties are associated with reaching a diagnosis preoperatively, chronological changes on EUS may contribute to the diagnosis of PF. ESD may also be useful in the diagnosis and treatment of PF.
文摘AIM: To determine the discriminating performance of the macular ganglion cell-inner plexiform layer(GCIPL) parameters between all the consecutive stages of glaucoma(from healthy to moderate-to-severe glaucoma), and to compare it with the discriminating performances of the peripapillary retinal nerve fiber layer(RNFL) parameters and optic nerve head(ONH) parameters.METHODS: Totally 147 eyes(40 healthy, 40 glaucoma suspects, 40 early glaucoma, and 27 moderate-to-severe glaucoma) of 133 subjects were included. Optical coherence tomography(OCT) was obtained using Cirrus HD-OCT 5000. The diagnostic performances of GC-IPL, RNFL, and ONH parameters were evaluated by determining the area under the curve(AUC) of the receiver operating characteristics. RESULTS: All GC-IPL parameters discriminated glaucoma suspect patients from subjects with healthy eyes and moderate-to-severe glaucoma from early glaucoma patients(P<0.017, for all). Also, minimum, inferotemporal and inferonasal GC-IPL parameters discriminated early glaucoma patients from glaucoma suspects, whereas no RNFL or ONH parameter could discriminate between the two. The best parameters to discriminate glaucoma suspects from subjects with healthy eyes were superonasal GC-IPL, superior RNFL and average c/d ratio(AUC=0.746, 0.810 and 0.746, respectively). Discriminating performances of all the parameters for early glaucoma vs glaucoma suspect comparison were lower than that of the other consecutive group comparisons, with the bestGC-IPL parameters being minimum and inferotemporal(AUC=0.669 and 0.662, respectively). Moreover, minimum GC-IPL, average RNFL, and rim area(AUC=0.900, 0.858, 0.768, respectively) were the best parameters for discriminating moderate-to-severe glaucoma patients from early glaucoma patients.CONCLUSION: GC-IPL parameters can discriminate glaucoma suspect patients from subjects with healthy eyes, and also all the consecutive stages of glaucoma from each other(from glaucoma suspect to moderate-tosevere glaucoma). Further, the discriminating performa
基金Suzhou Municipal Science and Technology Bureau,No.SYSD2017126
文摘Plexiform schwannoma is an extremely rare variant of schwannoma, accounting for approximately 5% of cases. Due to the rarity and lack of typical symptoms, signs and radiological images, a definite diagnosis of plexiform schwannoma may not be made by clinicians prior to biopsy. In the present study, we report the first case(to our knowledge) of perianal plexiform schwannoma arising from the overlapped skin of the ischioanal fossa, and we propose an intratumorally nonenhanced circumferential capsule dividing the tumour into multiple homogeneously enhanced nodules as a magnetic resonance imaging feature to aid in the differential diagnosis of plexiform schwannoma from ancient schwannoma, cavernous haemangioma, liposarcoma and plexiform neurofibroma.
文摘Plexiform angiomyxoid myofibroblastic tumor(PAMT) is a rare benign mesenchymal tumor of stomach. Rarity of this kind of tumors and scarce review articles may cause underrecognition of this entity and pose a real diagnostic challenge to gastroenterologists, pathologists and surgeons when encountering such patients and differentiating PAMT from other gastric intramural tumors. We report a case of 28-year-old woman, who presented with epigastric pain after meals, iron-deficiency anaemia and weight loss. Upper gastrointestinal endoscopy revealed submucosal tumorlike elevated lesion in the anterior wall of the antrum with intact overlying mucosa. Endoscopic ultrasound showed a 3-cm hypoechoic homogenous mass, originating from the third layer of the gastric wall. Endoscopic ultrasound-guided fine needle aspiration was not informative. Endoscopic buttonhole biopsy was performed to obtain specimens. Following this, the unexpected prolapse of the tumor occurred into the lumen of the stomach, causing gastric outlet obstruction- the biopsy was obtained. Pathomorphological features suggested the diagnosis of PAMT. Gastric resection of the Billroth I type was performed. Diagnosis was confirmed by histological analysis of the surgical specimen.
基金Supported by National Natural Science Foundation of China(No.81800879)Natural Science Foundation of Guangdong Province(No.2017A030310372)+2 种基金Fundamental Research Funds of the State Key Laboratory of Ophthalmology,China(No.2018KF04 No.2017QN05)Sun Yat-Sen University Clinical Research 5010 Program(No.2014016)
文摘AIM: To evaluate the patterns of macular ganglion cell-inner plexiform layer(GCIPL) loss in normal tension glaucoma(NTG) and primary open angle glaucoma(POAG) in a detailed, disease severity-matched way;and to assess the diagnostic capabilities of GCIPL thickness parameters in discriminating NTG or POAG from normal subjects.METHODS: A total of 157 eyes of 157 subjects, including 57 normal eyes, 51 eyes with POAG and 49 eyes with NTG were enrolled and strictly matched in age, refraction, and disease severity between POAG and NTG groups. The average, minimum, superotemporal, superior, superonasal, inferonasal, inferior, and inferotemporal GCIPL thickness, and the average, superior, temporal, inferior, and nasal retinal nerve fiber layer(RNFL) thickness were obtained by Cirrus optical coherence tomography(OCT). The diagnostic capabilities of OCT parameters were assessed by area under receiver operating characteristic(AUROC) curves. RESULTS: Among all the OCT thickness parameters, no statistical significant difference between NTG group and POAG group was found(all P>0.05). In discriminating NTG or POAG from normal subjects, the average and inferior RNFL thickness, and the minimum GCIPL thickness had better diagnostic capabilities. There was no significant difference in AUROC curve between the best GCIPL thickness parameter(minimum GCIPL) and the best RNFL thickness parameter in discriminating NTG(inferior RNFL;P=0.076) and indiscriminating POAG(average RNFL;P=0.913) from normal eyes.CONCLUSION: Localized GCIPL loss, especially in the inferior and inferotemporal sectors, is more common in NTG than in POAG. Among all the GCIPL thickness parameters, the minimum GCIPL thickness has the best diagnostic performance in differentiating NTG or POAG from normal subjects, which is comparable to that of the average and inferior RNFL thickness.
文摘Plexiform fibromyxoma is a very rare mesenchymal tumor of the stomach, found almost exclusively in the antrum/pylorus region. The most common presenting symptoms are anemia, hematemesis, nausea and unintentional weight loss, without sex or age predilection. We describe here two cases of plexiform fibromyxoma, involving a 16-year-old female and a 34-year-old male. Both patients underwent complete resection(R0) by distal gastrectomy and retrocolic gastrojejunostomy(according to Billroth 2); for both, the postoperative course was uneventful. Histology showed multiple intramural and subserosal nodules with characteristic plexiform growth, featuring bland spindle cells situated in an abundant myxoid stroma with low mitotic activity. Immunohistochemistry showed α-smooth muscle actin-positive spindle cells, focal positivity for CD10, and negative staining for KIT, DOG1, CD34, S100, β-catenin, STAT-6 and anaplastic lymphoma kinase. One of the cases showed focal positivity for h-caldesmon and desmin. Upon followup, no sign of disease was found. In the differential diagnosis of plexiform fibromyxoma, it is important to exclude the more common gastrointestinal stromal tumors as they have greater potential for aggressivebehavior. Other lesions, like neuronal and vascular tumors, inflammatory fibroid polyps, abdominal desmoid-type fibromatosis, solitary fibrous tumors and smooth muscle tumors, must also be excluded.
