Plexiform angiomyxoid myofi broblastic tumor of the stomach

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.

Gastric plexiform fibromyxoma resected by endoscopic submucosal dissection after observation of chronological changes:A case report

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.

Rarity among benign gastric tumors: Plexiform fibromyxoma-Report of two cases

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.

Gastric plexiform fibromyxoma:A case report

BACKGROUND Plexiform fibromyxoma(PF)is a rare mesenchymal tumor of the stomach.The clinical features of PF frequently include upper abdominal pain,abdominal discomfort,hematemesis,melena,pyloric obstruction and an upper abdominal mass.We herein report a case of PF resected by laparoscopic radical distal gastrectomy plus Roux-en-Y gastrojejunostomy.CASE SUMMARY The patient was admitted to hospital,due to a 1-wk history of an abdominal space-occupying lesion identified during a health examination.He underwent complete resection by laparoscopic radical distal gastrectomy plus Roux-en-Y gastrojejunostomy.During the operation,the tumor was located in the anterior wall of the gastric antrum(approximately 7 cm×6 cm×5.5 cm)and did not show evidence of invasion of the serosa.Histology showed that the tumor cells were oval fibroblast-like and spindle-shaped cells,with numerous thin-walled blood vessels and abundant myxoid stroma.Cellular atypia and mitosis were both rare.Immunohistochemistry showed that the tumor cells were immunoreactive for smooth muscle actin,S-100 and CD-10,but were negative for CD-117,CD-34,DOG-1,and ALK.In this case,S-100 was positive and no significant disease was observed during the follow-up period.CONCLUSION The fact that PF is a rare tumor with only a few cases in this region can lead to misdiagnosis of this entity and pose a real diagnostic challenge for general surgeons and pathologists when encountering such patients and differentiating PF from other primary tumors of gastric mesenchymal origin.Our report may help increase awareness of this rare,but important new disease entity.

Plexiform fibromyxoma of the small bowel: A case report

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.

Plexiform angiomyxoid myofibroblastic tumor of stomach:A rare case

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.

Discriminating performance of macular ganglion cellinner plexiform layer thicknesses at different stages of glaucoma

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 performance of GC-IPL thicknesses is comparable to that.

Plexiform fibromyxoma:Review of rare mesenchymal gastric neoplasm and its differential diagnosis

Plexiform fibromyxoma(PF)is a very rare mesenchymal neoplasm of the stomach that was first described in 2007 and was officially recognized as a subtype of gastric mesenchymal neoplasm by World Health Organization(WHO)in 2010.Histologically,PF is characterized by a plexiform growth of bland spindle to ovoid cells embedded in a myxoid stroma that is rich in small vessels.The lesion is usually paucicellular.While mucosal and vascular invasion have been documented,no metastasis or malignant transformation has been reported.Its pathogenesis is largely unknown and defining molecular alterations are not currently available.There are other mesenchymal tumors arising in the gastrointestinal tract that need to be differentiated from PF given their differing biologic behaviors and malignant potential.Histologic mimics with spindle cells include gastrointestinal stromal tumor,smooth muscle tumor,and nerve sheath tumor.Histologic mimics with myxoid stroma include myxoma and aggressive angiomyxoma.Molecular alterations that have been described in a subset of PF may be seen in gastroblastoma and malignant epithelioid tumor with gliomaassociated oncogene homologue 1(GLI1)rearrangement.The recent increase in publications on PF reflects growing recognition of this entity with expansion of clinical and pathologic findings in these cases.Herein we provide a review of PF in comparison to other mesenchymal tumors with histologic and molecular resemblance to raise the awareness of this enigmatic neoplasm.Also,we highlight the challenges pathologists face when the sample is small,or such rare entity is encountered intraoperatively.

Solitary plexiform neurofibroma of the stomach:A case report

Plexiform neurofibroma(PN)of the digestive tract is very rare and usually part of the generalized syndrome of neurofibromatosis type 1(von Recklinghausen disease).Solitary PN of the stomach is extremely rare and has not been reported in the literatures.Here we present a case of solitary PN of the stomach,which was not associated with von Recklinghausen disease.A38-year-old male presented abdominal pain and distention for 7 d.The patient underwent endoscopy of the upper gastrointestinal tract,which revealed a 3.5 cm protruding and cauliflower-shaped mass with a shallow1 cm central ulcer in the greater curvature of the stomach.The lesion was removed by laparoscopic surgery.Histological examination demonstrated characteristic histological findings of spindle-shaped cells.Immunohistochemical analysis showed that the tumor cells were positive for S-100 protein,but negative for CD34,KI-67,CD117,and actin.Based on histological findings,gastrointestinal stromal tumor could be excluded,and thus the case was confirmed as PN.We described the clinical features,physical examination,endoscopic findings,and histopathological examination of this case.

Magnetic resonance imaging findings for differential diagnosis of perianal plexiform schwannoma: Case report and review of the literature

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.

Retinal nerve fiber layer and ganglion cell-inner plexiform layer thickness in children with obesity

AIM:To evaluate retinal nerve fiber layer(RNFL)thickness analysis of peripapillary optic nerve head(PONH) and macula as well as ganglion cell-inner plexiform layer(GCIPL) thickness in obese children.· METHODS:Eighty-five children with obesity and 30 controls were included in the study.The thicknesses of the PONH and macula of each subject's right eye were measured by high-resolution spectral-domain optic coherence tomography(OCT).· RESULTS:The RNFL thicknesses of central macular and PONH were similar between the groups(all P >0.05).The GCIPL thickness was also similar between the groups.However,the RNFL thickness of temporal outer macula were 261.7±13.7 and 268.9±14.3 μm for the obesity and the control group,respectively(P =0.034).· CONCLUSION:Obesity may cause a reduction in temporal outer macular RNFL thickness.

Retinal ganglion cell-inner plexiform and nerve fiber layers in neuromyelitis optica

AIM: To determine the thickness of the retinal ganglion cell-inner plexiform layer(GCIPL) and the retinal nerve fiber layer(RNFL) in patients with neuromyelitis optica(NMO).METHODS: We conducted a cross-sectional study that included 30 NMO patients with a total of 60 eyes. Based on the presence or absence of optic neuritis(ON), subjects were divided into either the NMO-ON group(30 eyes) or the NMO-ON contra group(10 eyes). A detailed ophthalmologic examination was performed for each group; subsequently, the GCIPL and the RNFL were measured using highdefinition optical coherence tomography(OCT). RESULTS: In the NMO-ON group, the mean GCIPL thickness was 69.28±21.12 μm, the minimum GCIPL thickness was 66.02±10.02 μm, and the RNFL thickness were 109.33±11.23, 110.47±3.10, 64.92±12.71 and 71.21±50.22 μm in the superior, inferior, temporal and nasal quadrants, respectively. In the NMO-ON contra group, the mean GCIPL thickness was 85.12±17.09 μm, the minimum GCIPL thickness was 25.39±25.1 μm, and the RNFL thicknesses were 148.33±23.22, 126.36±23.45, 82.21±22.30 and 83.36±31.28 μm in the superior, inferior, temporal and nasal quadrants, respectively. In the control group, the mean GCIPL thickness was 86.98±22.37 μm, the minimum GCIPL thickness was 85.28±10.75 μm, and the RNFL thicknesses were 150.22±22.73, 154.79±60.23, 82.33±7.01 and 85.62±13.81 μm in the superior, inferior, temporal and nasal quadrants, respectively. The GCIPL and RNFL were thinner in the NMO-ON contra group than in the control group(P<0.05); additionally, the RNFL was thinner in the inferior quadrant in the NMO-ON group than in the control group(P<0.05). Significant correlations were observed between the GCIPL and RNFL thickness measurements as well as between thickness measurements and the two visual field parameters of mean deviation(MD) and corrected pattern standard deviation(PSD) in the NMO-ON group(P<0.05). CONCLUSION: The thickness of the GCIPL and RNFL, as measured using OCT, may indicate optic nerve damage in patients with NMO.

Comparisons of ganglion cell-inner plexiform layer loss patterns and its diagnostic performance between normal tension glaucoma and primary open angle glaucoma: a detailed, severity-based study

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.

Plexiform angiomyxoid myofibroblastic tumor treated by endoscopic submucosal dissection:A case report and review of the literature

BACKGROUND Plexiform angiomyxoid myofibroblastic tumor(PAMT)is a rare mesenchymal tumor characterized by multiple nodular plexiform growth patterns and an immunophenotype with myofibroblasts.The pathological characteristics,immunohistochemistry,diagnostic criteria,differential diagnosis,and gene-level changes of PAMT have been reported in many studies.At present,the main treatment for PAMT in the reported cases is surgery;only eight cases were treated via endoscopy(excluding 1 thoracoscopic resection),and the lesions were all smaller than 5 cm.There are no reports on the prognosis and follow-up of young patients with lesion sizes reaching 5 cm who undergo endoscopic submucosal dissection(ESD).Herein,we present the first case of a young patient with a lesion size reaching 5 cm who was diagnosed with PAMT via endoscopic submucosal dissection.CASE SUMMARY A 15-year-old young man with upper abdominal pain for 2 years presented to the Gastroenterology Department of our hospital.Painless gastroscopy showed a semicircular bulge approximately 5 cm in size in the lesser curvature near the cardia of the fundus;the surface was eroded,and shallow ulcers had formed.The pathological manifestations of the biopsy were spindle cell proliferative lesions with interstitial mucinous changes,and the surface mucosa showed chronic inflammatory changes with active lesions;immunohistochemistry showed smooth muscle actin(SMA)(+),CD117(-),CD34(-),DOG-1(-),S-100(-),and Ki67(LI:<1%).We performed ESD on the patient.The lesion that we removed was 5 cm×4 cm×2 cm in size.Pathologically,the resected tissue displayed typical manifestations,such as fat spindle-shaped fibroblasts and myofibroblast-like cells showing irregular nodular hyperplasia.Immunohistochemistry staining of the tumor cells revealed the following:CD34(partially+),SMA(weakly+),CD117(-),DOG-1(-),S-100(-),SDHB(+),PCK(-),and Ki67(labelling index:2%).There was no recurrence or metastasis during the 3-mo follow-up after the operation,and the treatment effect was good.We also performed a review of the literature on the clinical manifestations,pathological features,immunohistochemistry,and differential diagnosis of PAMT.CONCLUSION At present,the diagnostic criteria for PAMT are relatively clear,but the pathogenesis and genetic changes require further study.PAMT is benign in nature,and these patients are less likely to experience local or metastatic recurrence.The main treatment is still surgery if the lesion is in the stomach.Partial gastrectomy and distal gastrectomy are the most frequently performed surgical treatments for PAMT,followed by local resection,subtotal gastrectomy,and wedge resection.But for comprehensive evaluation of the disease,ESD can be considered a suitable method to avoid excessive treatment.

Correlation between macular ganglion cell-inner plexiform layer thickness and visual acuity after resolution of the macular edema secondary to central retinal vein occlusion

AIM: To examine the thickness of the ganglion cell-inner plexiform layer(GCIPL) in eyes with resolved macular edema(ME) in non-ischemic central retinal vein occlusion(CRVO), applying spectral-domain optical coherence tomography(SD-OCT), and its relationship with visual acuity.METHODS: The retrospective observational case-control study included 30 eyes of non-ischemic CRVO patients with resolved ME(ME eyes) after treatment, and 30 eyes of non-ischemic CRVO patients without ME(non-ME eyes). The macular GCIPL thickness, peripapillary retinal nerve fiber layer(p RNFL) thickness and central macular thickness(CMT) were measured on a SD-OCT scan. Linear regression analyses were performed to determine the correlation between the thickness of each and the visual acuity(VA).RESULTS: No significant difference in average GCIPL thickness, mean pR NFL thickness and CMT were observed between ME group and non-ME group(P=0.296, 0.183, 0.846). But, minimum GCIPL thickness was reduced in ME eyes compared with non-ME eyes(P=0.022). Final VA significantly correlated with the minimum GCIPL thickness in ME eyes(r=-0.482, P=0.007), whereas no correlation was found with average GCIPL thickness, average pR NFL thickness and mean CMT.CONCLUSION: Minimum GCIPL thickness is reduced in ME eyes compared with non-ME eyes, and correlated with the VA in non-ischemic CRVO. These results propose that inner retinal damage occurring in patients with ME secondary to non-ischemic CRVO may lead to permanent visual defect after treatment.

Different damage patterns of retinal nerve fiber layer and ganglion cell-inner plexiform layer between early glaucoma and non-glaucomatous optic neuropathy

AIM:To compare the damage pattern of the peripapillary retinal nerve fiber layer(pRNFL)and the macular ganglion cell-inner plexiform layer(mGCIPL)between early glaucomatous and non-glaucomatous optic neuropathy(EGON and NGON).METHODS:It is a cross-sectional study.Thirty-eight healthy controls,74 EGONs and 70 NGONs with comparable average pRNFL loss were included.The NGON group included 23 eyes of optic neuritis(ON),13 eyes of hereditary optic neuropathy(HON),19 eyes of toxic optic neuropathy(TON)and 15 eyes of compressive neuropathy(CON).The sectoral pRNFL and mGCIPL thickness obtained by high definition optical coherence tomography were analyzed.RESULTS:Compared to normal controls,the pRNFL thickness in all quadrants showed a decrease in both EGON and NGON group(P<0.001),but the average pRNFL thickness of EGON group was not different to that of NGON group(P=0.94).The inferior and superior pRNFL was thinner in EGON group compared to NGON group(P<0.001).The temporal pRNFL was thinner in NGON group compared to EGON group(P<0.001).No statistically significant difference was found in nasal pRNFL between EGON and NGON.While the nasal pRNFL was thinner in CON than other three types of NGON(P=0.01),no statistically significant difference was found in other three quadrantal pRNFL among the four types of NGON(P>0.05).The mGCIPL of EGON and NGON group were thinner than control group(P<0.001).In EGON group the severest sites of mGCIPL reduction was located at inferotemporal and inferior sectors.While,compared to EGON group,the average mGCIPL of NGON groupwere significantly thinner,especially in superonasal and inferonasal sectors(P<0.001).CONCLUSION:The damage pattern of pRNFL and mGCIPL caused by glaucoma is distinct from other NGON such as ON,TON,HON and CON,and this characteristic damage pattern is helpful in differentiating early glaucoma from other NGON.

Huge gastric plexiform fibromyxoma presenting as pyemia by rupture of tumor:A case report

BACKGROUND Plexiform fibromyxoma(PF)is a rare mesenchymal tumor,with limited case reports worldwide.Common clinical symptoms are abdominal discomfort and bleeding signs,which frequently present slow-onset in reported cases.Herein,we report a case of gastric PF presenting as acute onset and with pyemia accompanying tumor rupture.We resected the tumor as well as the distal gastric,bulbus duodeni and gallbladder for treatment in emergency surgery.Notably,before the onset of the disease,the patient received coronavirus disease 2019(COVID-19)vaccines.CASE SUMMARY A 26-year-old man was admitted to our hospital,due to abdominal pain and fever after having received COVID-19 vaccines.Laboratory examination indicated severe sepsis.Computed tomography scan revealed a large mass in the abdomen.Deformation of the gastrointestinal tract was seen during gastroscopy.After failure of anti-infective treatment and symptoms of shock developed,he received an emergency surgery.We found a huge and partly ruptured mass,with thick purulence.Microscopically,the mass was composed of spindle cells with clarified cytoplasm,accompanied by myxoid stroma and arborizing blood vessels.Immunohistochemistry showed the tumor cells as positive for smooth muscle actin and succinate dehydrogenase subunit B but negative for DOG-1 and CD117.Finally,the patient was diagnosed with gastric PF and discharged from the hospital.CONCLUSION Gastric PF manifesting as tumor rupture combined with pyemia is rare.Timely surgery is critical for optimal prognosis.

Plexiform neurofibroma of the cauda equina with follow-up of 10 years:A case report

BACKGROUND Plexiform neurofibromas are extremely rarely found in the region of cauda equina and can pose a significant challenge in the diagnostic and management sense.To our knowledge,only 7 cases of cauda equina neurofibromatosis(CENF)have been reported up-to-date.CASE SUMMARY We describe a case of a 55-year-old man with a 10 years history of progressive lower extremities weakness and bladder dysfunction.Before presenting,patient was misdiagnosed with idiopathic polyneuropathy.Lumbar spine MRI revealed a tortuous tumorous masses in the cauda equina region,extending through the Th12-L4 vertebrae.The patient underwent Th12-L3 Laminectomy with duraplasty.During the operation,the most enlarged electroneurographically silent nerve root was resected,anticipating inadequate decompression if nerve root was spared.The patient’s neurological condition improved post-operatively,but urinary retention became the major complaint.We provide a follow-up period of 10 years.During this time,the patient’s condition progressively worsened despite extensive decompression.The consequent MRI scans showed progressive enlargement of cauda equina roots and increasing lumbar stenosis,predominantly affecting L3-L4 segment.During the follow-up 8 years after the operation,the patient complained of worsening lower extremities sensorimotor function and neurogenic claudication.Subsequent MRI revealed lumbar spine stenosis at the level of L3-L4,requiring further decompression.The patient underwent a second surgery involving L4-L5 Laminectomy with duraplasty and L2-L5 transpedicular fixation.The post-operative period was uneventful.Latest follow-up 18 mo after the second surgery revealed substantial improvement in patient’s well-being.CONCLUSION CENF should be kept in mind during the differential diagnostic work-up for polyneuropathies.Management with an extensive decompression,duraplasty and primary spinal fixation represents a rational approach to achieve a sustained symptomatic improvement and superior overall outcome.

Solitary Intraparotid Facial Nerve Plexiform Neurofibroma

Solitary intraparotid facial nerve plexiform neurofibromas are extremely rare. These tumors arise from Schwann cells. The plexiform variant is recognized by tortuous and multinodular gross and microscopic lesions. It has a high risk of malignant transformation. We report a case of a solitary plexiform neurofibroma in a 5-year-old Italian male, who initially presented a right parotid mass of four-month duration. He had not pain, trismus, facial weakness or previous trauma. There was not familiar history of Von Recklinghausen’s disease. TC and MRI scans revealed a mass in the superficial lobe of the parotid gland. A partial parotidectomy was performed. Histopatological examination indicated plexiform neurofibroma. The incidence, presentation, diagnosis and surgical treatment of this lesion are discussed. In our case, the tumor could be readily separated from the main trunk, and facial movement was completely preserved owing to the rich neural network around the mass.

Plexiform Schwannoma: A Report of Two Unusual Cases, and a Review of the Literature

Plexiform schwannoma is a benign tumour arising from the sheath of myelinated nerve fibres that may occur in any part of the body. It is very rare in the head and neck region and poses diagnostic challenges. Here we present two unusual cases. The first is about a fifteen-year-old boy with a large plexiform schwannoma in the right retro maxillary region, extending up to but not invading the orbit. Initial cytology reported it as a pleomorphic adenoma, but histological findings after excision confirmed it to be a benign plexiform schwannoma. A zygomatic osteotomy was required to excise the tumour in its entirety. The second reported case is about solitary lesion on the tongue of a 46-year-old Chinese man, clinically mimicking a mucocoele. This case report highlights the diagnostic challenges posed by plexiform schwannoma, and the role of imaging and cytopathology, as well as the importance of definitive histopathological analysis. It also describes the surgical management of these lesions, and provides a review of the literature, in particular of cases occurring in the head and neck region.