BACKGROUND Inflammatory myofibroblastic tumor(IMT)is a relatively rare tumor.The global incidence of IMT is less than 1%.There is no specific clinical manifestation.It usually occurs in the lungs,but the pancreas is n...BACKGROUND Inflammatory myofibroblastic tumor(IMT)is a relatively rare tumor.The global incidence of IMT is less than 1%.There is no specific clinical manifestation.It usually occurs in the lungs,but the pancreas is not the predilection site.CASE SUMMARY We present a case of a male patient,51 years old,who was diagnosed with a pancreatic neck small mass on ultrasound one year ago during a physical examination.As he had no clinical symptoms and the mass was relatively small,he did not undergo treatment.However,the mass was found to be larger on review,and he was referred to our hospital.Since the primal clinical diagnosis was pancreatic neuroendocrine tumor,the patient underwent surgical treatment.However,the case was confirmed as pancreatic IMT by postoperative pathology.CONCLUSION Pancreatic IMT is relatively rare and easily misdiagnosed.We can better understand and correctly diagnose this disease by this case report.展开更多
BACKGROUND Low-grade myofibroblastic sarcoma(LGMS)is a rare spindle cell sarcoma espe-cially in the pancreas,with myofibroblastic differentiation.Hitherto,only a few cases have been reported.CASE SUMMARY Herein,we rep...BACKGROUND Low-grade myofibroblastic sarcoma(LGMS)is a rare spindle cell sarcoma espe-cially in the pancreas,with myofibroblastic differentiation.Hitherto,only a few cases have been reported.CASE SUMMARY Herein,we report a case involving the discovery of a pancreatic mass detected during a routine physical examination.Subsequent imaging and pathological tests of the patient led to the diagnosis of LGMS of the pancreas.Following surgical intervention,the patient experienced recurrence and metastasis.Conventional treatment is not effective for postoperative recurrent pancreatic LGMS with multiple metastases.After communicating with the patients and their families,informed consent was obtained for the treatment of anlotinib combined with pembrolizumab.Evaluation of imaging and clinical symptoms post-treatment revealed a relatively favorable response to the combination of anlotinib and pembrolizumab.CONCLUSION Based on the comprehensive literature review,our report aimed to provide evidence for a better understanding of the disease characteristics,diagnostic criteria,imaging findings,and identification of LGMS.And explore novel treatment strategies for this disease.展开更多
BACKGROUND The inflammatory myofibroblastic tumor(IMT)is a rare mesenquimal tumor of doubtful biological behaviour.It’s characterised for affecting mainly children and young adults,although it can appear at any age,b...BACKGROUND The inflammatory myofibroblastic tumor(IMT)is a rare mesenquimal tumor of doubtful biological behaviour.It’s characterised for affecting mainly children and young adults,although it can appear at any age,being the lungs the primary affected organ(in children it represents 20%of all primary pulmonary tumors).CASE SUMMARY We present the case of a 45 year old woman,with a computed tomography(CT)finding of injury on the anterior surface of the fundus/gastric body and a solid perigastric injury of 12 mm in the ecoendoscopy.The case is presented in the tumor committee deciding to perform a laparoscopic wedge resection.The histological diagnosis was a IMT.The diagnosis is based on imaging tests like the abdominal CT,abdominal ecography and the ecoendoscopy but to confirm the diagnosis a pathological study is necessary.CONCLUSION Due to the unpredictable nature of this tumor,surgical resection is the best therapeutic option.展开更多
BACKGROUND Hepatic inflammatory myofibroblastic tumor(HIMT)is a rare type of hepatic tumor.It is always misdiagnosed and mistreated because it is primarily found with no obvious specific manifestation,and its imaging ...BACKGROUND Hepatic inflammatory myofibroblastic tumor(HIMT)is a rare type of hepatic tumor.It is always misdiagnosed and mistreated because it is primarily found with no obvious specific manifestation,and its imaging findings are diverse.CASE SUMMARY Here,we report a case of HIMT that was initially diagnosed as liver malignancy but was confirmed as HIMT by histopathology after hepatectomy.Mostly,HIMTs are infiltrated with plasma cells and stain positively for anaplastic lymphoma kinase on immunohistochemistry as well as for some other kinases.CONCLUSION HIMT can be treated with single nonsteroidal anti-inflammatory drugs and without surgery when it is diagnosed accurately.Because the etiology of HIMT is unknown and the diagnosis is difficult,the pathogenesis and clinical process need to be further studied.展开更多
Inflammatory myofibroblastic tumor(IMT)of the biliary tract is rare,and often difficult to diagnose or to distinguish from other tumors due to its atypical clinical presentation and nonspecific radiological features.H...Inflammatory myofibroblastic tumor(IMT)of the biliary tract is rare,and often difficult to diagnose or to distinguish from other tumors due to its atypical clinical presentation and nonspecific radiological features.Histologically,IMTs are(myo)fibroblastic neoplasms with a prominent inflammatory infiltrate.They are characterized by receptor tyrosine kinase gene rearrangements,most often involving an anaplastic lymphoma kinase(ALK)translocation.The final diagnosis of IMT depends on histopathology and immunohistochemical examination.In this manuscript,we provide a clinical and morphomolecular overview of IMT and the difficulties that may arise in using immunohistochemical and molecular techniques in diagnosing IMT.展开更多
Plexiform angiomyxoid myofibroblastic tumor (PAMT) is a recently described gastric tumor with a peculiar plexiform pattern, bland spindle cells and a myxoid stroma rich in arborizing blood vessels. PAMT of the stomach...Plexiform angiomyxoid myofibroblastic tumor (PAMT) is a recently described gastric tumor with a peculiar plexiform pattern, bland spindle cells and a myxoid stroma rich in arborizing blood vessels. PAMT of the stomach is a very rare tumor without distinctive clinical manifestations. In this study, we report a new case of PAMT which is the first Chinese case in English literature. A 47-year-old Chinese woman was admitted with a 6-month history of intermittent epigastric discomfort, and abdominal pain for 2 months. Gastroscopy showed an elevated mass in the anterior wall of the gastric antrum. Endoscopic ultrasound revealed a focal hypoechoic lesion protruding into the lumen. A laparoscopic distal gastrectomy was performed, and the patient made an uneventful recovery and remains well 1.5 years later. A diagnosis of PAMT was made by histopathology and immunochemistry.展开更多
Inflammatory myofibroblastic tumor(IMT) is an uncommon benign neoplasm with locally aggressive behavior but malignant change is rare.We report an unusual case of pelvic-abdominal inflammatory myofibroblastic tumor wit...Inflammatory myofibroblastic tumor(IMT) is an uncommon benign neoplasm with locally aggressive behavior but malignant change is rare.We report an unusual case of pelvic-abdominal inflammatory myofibroblastic tumor with malignant transformation in a 14-year-old boy presenting with abdominal pain and 9 kg body weight loss in one month.Computed tomography revealed a huge pelvi-abdominal mass(30 cm),possibly originating from the pelvic extraperitoneal space,protruding into the abdomen leading to upward displacement of the bowel loops,downward displacement of the urinary bladder,massive central necrosis,a well-enhanced peripheral solid component with prominent peritumoral vascularity.Subsequent examination confirmed the computed tomographic findings.Histopathologic examination revealed proliferative epitheloid and spindle cells,inflammatory cell infiltration and high mitotic counts.Immunohistochemistry was strongly positive for anaplastic lymphoma kinase and revealed a high proliferative index(ki-67 = 40%).DNA sequencing and electronic microscopy further confirmed the primitive fibroblastic cell phenotype of the tumor and a final diagnosis of inflammatory myofibroblastic tumor with malignant transformation was established.Rapid tumor recurrence was noted 20 d after radical tumor resection.To our knowledge,this is the largest documented case of IMT in a pediatric patient and the first report of IMT with malignant transformation originating from the pelvic extraperitoneal space.展开更多
Low-grade myofibroblastic sarcoma (LGMS) is a rare, malignant tumor with myofibroblastic differentiation. Despite it being classified as a distinct entity by the World Health Organization, a few cases were reported ...Low-grade myofibroblastic sarcoma (LGMS) is a rare, malignant tumor with myofibroblastic differentiation. Despite it being classified as a distinct entity by the World Health Organization, a few cases were reported in the oral and maxillofacial region. Here, a LGMS developed on the palate of a 73-year-old man who presented with a 1-cm tumor on the posterior border of the palate. Based on the histological and immunohistochemical features, a diagnosis of LGMS was established. The tumor was resected, and no recurrence was observed over 2 years. Although the tongue is the most preferred site for LGMS, it may occur in any region of the oral cavity.展开更多
Inflammatory myofibroblastic tumors are usually treated by surgical resection. We herein report two cases of intra-abdominal inflammatory myofibroblastic tumors that were unresectable and underwent spontaneous regress...Inflammatory myofibroblastic tumors are usually treated by surgical resection. We herein report two cases of intra-abdominal inflammatory myofibroblastic tumors that were unresectable and underwent spontaneous regression without any treatment. Our case report and literature review show that regression is more common in the middle-aged and older male populations. Abdominal discomfort and fever were the most common symptoms, but the majority of patients had no obvious physical signs. There was no specific indicator for diagnosis. The majority of the lesions regressed within3 mo and nearly all of the masses completely resolved within 1 year. We conclude that the clinical characteristics of inflammatory myofibroblastic tumors are variable and, accordingly, the disease needs to be subdivided and treated on an individual basis. Surgery is always the first-line treatment; however, for those masses assessed as unresectable, conservative therapy with in-tense follow-up should be considered.展开更多
Only two cases of myofibroblastic sarcoma in the liver have been reported in the literature. Here, we report the case of a male patient with high-grade myofibroblastic sarcoma mimicking echinococcosis in the liver. Th...Only two cases of myofibroblastic sarcoma in the liver have been reported in the literature. Here, we report the case of a male patient with high-grade myofibroblastic sarcoma mimicking echinococcosis in the liver. The 25-year-old male patient complained of right upper quadrant swelling pain for one week and was initially diagnosed with echinococcosis. He was then scheduled for an exploratory laparotomy. During the operation, a huge mass exceeding 16 cm in diameter was found to occupy nearly the entire right trisegment of the liver, with a clear boundary and a round shape, and the mass was resected by right hepatic trisegmentectomy. Immunohistochemical staining revealed that the tumor tissue was positive for desmin, α-smooth muscle actin, CD56, and vimentin and negative for ALK-1, myogenin, calponin, β-catenin, S100, and glypican-3, with a Ki-67(MIB-1) index of approximately 20%. Based on the histological manifestations and immunohistochemical staining, a diagnosis of myofibroblastic sarcoma was established. The postoperative recovery was uneventful. There was no evidence of recurrence or metastasis through the last follow-up, 6 mo after surgery, despite a lack of postoperative chemotherapy or radiotherapy. To the best of our knowledge, the present case is the first reported case of high-grade myofibroblastic sarcoma in the liver, and it is also the first reported case in a male patient.展开更多
AIM To investigate the anti-fibrotic effects of the traditional oriental herbal medicine Daikenchuto(DKT) associated with transient receptor potential ankyrin 1(TRPA1) channels in intestinal myofibroblasts. METHODS In...AIM To investigate the anti-fibrotic effects of the traditional oriental herbal medicine Daikenchuto(DKT) associated with transient receptor potential ankyrin 1(TRPA1) channels in intestinal myofibroblasts. METHODS Inflammatory and fibrotic changes were detected in a2,4,6-trinitrobenzenesulfonic acid(TNBS) chronic colitis model of wild-type and TRPA1-knockout(TRPA1-KO) mice via pathological staining and immunoblotting analysis.Ca^(2+) imaging experiments examined the effects of DKT and its components/ingredients on intestinal myofibroblast(In Myo Fib) cell TRPA1 channel function.Profibrotic factors and transforming growth factor (TGF) -β1-associated signaling were tested in an In Myo Fib cell line by q PCR and immunoblotting experiments.Samples from non-stenotic and stenotic regions of the intestines of patients with Crohn’s disease (CD) were used for pathological analysis. RESULTS Chronic treatment with TNBS caused more severe inflammation and fibrotic changes in TRPA1-KO than in wild-type mice.A one-week enema administration of DKT reduced fibrotic lesions in wild-type but not in TRPA1-KO mice.The active ingredients of DKT,i.e.,hydroxyα-sanshool and 6-shogaol,induced Ca^(2+) influxes in In Myo Fib,and this was antagonized by co-treatment with a selective TRPA1 channel blocker,HC-030031.DKT counteracted TGF-β1-induced expression of TypeⅠcollagen andα-smooth muscle actin (α-SMA) ,which were accompanied by a reduction in the phosphorylation of Smad-2 and p38-mitogen-activated protein kinase (p38-MAPK) and the expression of myocardin.Importantly,24-h incubation with a DKT active component Japanese Pepper increased the m RNA and protein expression levels of TRPA1 in In Myo Fibs,which in turn negatively regulated collagen synthesis.In the stenotic regions of the intestines of CD patients,TRPA1 expression was significantly enhanced.CONCLUSION The effects of DKT on the expression and activation of the TRPA1 channel could be advantageous for suppressing intestinal fibrosis,and benefit inflammatory bowel disease treatment.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
BACKGROUND Inflammatory myofibroblastic tumors of the liver(IMTL)are extremely rare neoplasms and very little is known about their clinical presentation,pathogenesis,and biological behavior.Due to their absolute rarit...BACKGROUND Inflammatory myofibroblastic tumors of the liver(IMTL)are extremely rare neoplasms and very little is known about their clinical presentation,pathogenesis,and biological behavior.Due to their absolute rarity,it is almost impossible to obtain a definite diagnosis without histological examination.Because of their intermediate biological behavior with the risk for local recurrence and metastases,surgical resection is recommend whenever IMTL is suspect.CASE SUMMARY We herein present a case of an otherwise healthy 32-year-old woman who presented with intermittent fever,unclear anemia,malaise and right flank pain 4 mo postpartum.The liver mass in segment IVa/b was highly FDG avid in the positron emission tomography-computed tomography.Hepatic resection was performed achieving a negative resection margin and an immediate resolution of all clinical symptoms.Histological analysis diagnosed the rare finding of an inflammatory myofibroblastic tumor of the liver and revealed cytoplasmic anaplastic lymphoma kinase expression by immunohistochemistry.Twelve months follow-up magnetic resonance imaging showed no recurrence and no metastases in the fully recovered patient.CONCLUSION IMTLs are extremely rare and difficult to diagnose.Due to their intermediate biological behavior,surgical resection should be perform whenever feasible and patients should be followed-up in order to detect recurrence and metastasis as early as possible.展开更多
Fibroblasts are the most abundant cellular components of connective tissue. They possess phenotypical heterogenicity and may be present in the form of smooth muscle cells or myofibroblasts(MFs). MFs are spindle-shaped...Fibroblasts are the most abundant cellular components of connective tissue. They possess phenotypical heterogenicity and may be present in the form of smooth muscle cells or myofibroblasts(MFs). MFs are spindle-shaped cells with stress fibres and welldeveloped fibronexus,and they display α-smooth muscle actin immunohistochemically and smoothmuscle myofilaments ultrastructurally. MFs play a crucial role in physiological and pathological processes. Derived from various sources,they play pivotal roles not only by synthesizing and producing extracellular matrix components,such as other connective tissue cells,but also are involved in force production. In the tissue remodelling phase of wound closure,integrinmediated interactions between MFs and type I collagen result in scar tissue formation. The tumour stroma in oral cancer actively recruits various cell types into the tumour mass,where they act as different sources of MFs. This article reviews the importance of MFs and its role in pathological processes such as wound healing,odontogenic cysts and tumours,salivary gland tumours,oral preneoplasia,and oral squamous cell carcinoma. Research oriented on blocking the transdifferentiation of fibroblasts into MFs can facilitate the development of noninvasive therapeutic strategies for the treatment of fibrosis and/or cancer.展开更多
In order to investigate the roles of Wnt signal pathway in transformation of cardiac valvular myofibroblasts to the osteoblast-like phenotype, the primary cultured porcine aortic valve myofibroblasts were incubated wi...In order to investigate the roles of Wnt signal pathway in transformation of cardiac valvular myofibroblasts to the osteoblast-like phenotype, the primary cultured porcine aortic valve myofibroblasts were incubated with oxidized low density lipoprotein(ox-LDL, 50 mg/L), and divided into four groups according to the ox-LDL treatment time: control group, ox-LDL 24-h group, ox-LDL 48-h group, and ox-LDL 72-h group. Wnt signal pathway blocker Dickkopf-1(DDK-1, 100 μg/L) was added in ox-LDL 72-h group. The expression of α-smooth muscle actin(α-SMA), bone morphogenetic protein 2(BMP2), alkaline phosphatase(ALP), and osteogenic transcription factor Cbfa-1 was detected by Western blotting, and that of β-catenin, a key mediator of Wnt signal pathway by immunocytochemical staining method. The Wnt/β-catenin was observed and the transformation of myofibroblasts to the osteoblast-like phenotype was examined. The expression of α-SMA, BMP2, ALP and Cbfa-1 proteins in the control group was weaker than in the ox-LDL-treated groups. In ox-LDL-treated groups, the protein expression of α-SMA, BMP2, ALP, and Cbfa-1 was significantly increased in a time-dependent manner as compared with the control group, and there was significant difference among the three ox-LDL-treated groups(P〈0.05 for all); β-catenin protein was also up-regulated in the ox-LDL-treated groups in a time-dependent manner as compared with the control group(P〈0.05), and its transfer from cytoplasm to nucleus and accumulation in the nucleus were increased in the same fashion(P〈0.05). After addition of DKK-1, the expression of α-SMA, bone-related proteins and β-catenin protein was significantly reduced as compared with ox-LDL 72-h group(P〈0.05). The Wnt/ β-catenin signaling pathway may play an important role in transformation of valvular myofibroblasts to the osteoblast-like phenotype.展开更多
BACKGROUND Inflammatory myofibroblastic tumor(IMT) is a rare mesenchymal tumor that is characterized by spindle cells differentiated from muscle fibroblasts and infiltration of various types of inflammatory cells. IMT...BACKGROUND Inflammatory myofibroblastic tumor(IMT) is a rare mesenchymal tumor that is characterized by spindle cells differentiated from muscle fibroblasts and infiltration of various types of inflammatory cells. IMT can occur at any age and at any anatomic site. The most common location of IMT is the bladder in the genitourinary tract. Only scarce cases of kidney IMT have been reported in the literature.CASE SUMMARY A 77-year-old woman, with a history of bilateral renal calculus for 15 years, was admitted to the Department of Urology of our hospital complaining of recurrent painless gross hematuria for one month. The treatment with cephalosporin was ineffective. Computed tomography imaging showed a mixed density and slightly heterogeneously enhanced lesion in the middle pole of the left kidney and ipsilateral adrenal enlargement. The patient underwent surgical treatment by retroperitoneoscopic left radical nephrectomy plus adrenalectomy. A large number of typical spindle cells surrounded by plasma cells and lymphocytes were observed microscopically. Immunohistochemical analyses indicated that these spindle cells were positive for vimentin, cytokeratin(CK), Ki-67, CK7,CD34, and CD31 and were focally positive for CD10 and anaplastic lymphoma kinase(ALK-1). Thus, a diagnosis of IMT was made definitively. The patient recovered well after operation, and no recurrence or metastasis was noted during the 22-mo follow-up.CONCLUSION Since kidney IMT is very rare and lacks characteristic clinical manifestation, it is easily misdiagnosed as a malignant tumor before operation. Surgery remains the best choice for diagnosis and treatment, and such cases must be followed carefully because of the uncertain biological behavior of this tumor. This report suggests that renal calculus may be one of the causes of IMT, but further investigation is necessary to prove it.展开更多
The etiology and pathogenesis of pulmonary fibrosis is poorly understood. We and others reported that M-CSF/CSF-1, M-CSF-R and downstream AKT activation plays an important role in lung fibrosis in mice models and in I...The etiology and pathogenesis of pulmonary fibrosis is poorly understood. We and others reported that M-CSF/CSF-1, M-CSF-R and downstream AKT activation plays an important role in lung fibrosis in mice models and in IPF patients. To understand potential molecular pathways used by M-CSF-R activation to direct lung fibrosis, we used a novel transgenic mouse model that expresses a constitutively-active form of AKT, myristoylated AKT (Myr-Akt), driven by the c-fms (M-CSF-R) promoter. We were particularly interested in the basal immune state of the lungs of these Myr-Akt mice to assess M-CSF-R-related priming for lung fibrosis. In support of a priming effect, macrophages isolated from the lungs of unchallenged Myr-Akt mice displayed an M2-tropism, enhanced co-expression of M-CSF-R and α-SMA, reduced autophagy reflected by reduced expression of the key autophagy genes Beclin-1, MAP1-Lc3a(Lc3a), and MAP1-Lc3b(Lc3b), and increased p62/STSQM1 expression compared with littermate WT mice. Furthermore, Myr-Akt mice had more basal circulating fibrocytes than WT mice. Lastly, upon bleomycin challenge, Myr-Akt mice showed enhanced collagen deposition, increased F4/80+ and CD45+ cells, reduced autophagy genes Beclin-1, Lc3a, and Lc3b expression, and a shorter life-span than WT littermates. These data provide support that M-CSF-R/AKT activation may have a priming effect which can predispose lung tissue to pulmonary fibrosis.展开更多
Inflammatory myofibroblastic tumor(IMT)occurs infrequently in the liver.It is controversial whether it represents a low grade mesenchymal neoplasm or a reactive inflammatory lesion.Local recurrence and metastasis are ...Inflammatory myofibroblastic tumor(IMT)occurs infrequently in the liver.It is controversial whether it represents a low grade mesenchymal neoplasm or a reactive inflammatory lesion.Local recurrence and metastasis are rare and some tumors are associated with infectious agents.We report on a case of a large and partially resected IMT with local recurrence and diaphragm and kidney infiltration detected on routine surveillance two years later.Histologically,the tumor showed spindle cells without atypia,mitosis or necrotic areas in a myxoid and collagenized background with inflammatory cells.In the liver portal tracts,granulomatous lesions with viable eggs of Schistosoma mansoni were identified.Immunohistochemistry demonstrated spindle cells which were smooth-muscle actin and vimentin positive.In conclusion,this case points out that these histological patterns do not predict the aggressive biological behavior of the lesion.A reason for the recurrence and the infiltration may be incomplete tumor resection.Further investigation is necessary in order to better clarify an infectious cause in some IMTs.展开更多
BACKGROUND Pancreatic inflammatory myofibroblastic tumor(IMT)is a relatively rare disease that is often confused with pancreatic cancer or pancreatic neuroendocrine tumors.The histological features of IMTs show that t...BACKGROUND Pancreatic inflammatory myofibroblastic tumor(IMT)is a relatively rare disease that is often confused with pancreatic cancer or pancreatic neuroendocrine tumors.The histological features of IMTs show that tissue from this type of tumor contains an intermingling of fibroblast and myofibroblast proliferation,accompanied by a varying degree of inflammatory cell infiltration.CASE SUMMARY The management of an IMT occurring at the neck of the pancreas is presented in this paper.A 66-year-old female patient was diagnosed with a pancreatic neck mass after a series of tests.The patient underwent enucleation of the pancreatic neck tumor after a pathological diagnosis of IMT.Previous research on the clinical features,pathological diagnosis and treatment of pancreatic IMTs was reviewed.Compared with previous reports,this is a unique case of enucleation of a pancreatic IMT.CONCLUSION The enucleation of pancreatic IMTs may be a safe and efficient surgical method for managing such tumors with a better prognosis.Further cases are required to explore surgical measures for pancreatic IMTs.展开更多
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 character...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.展开更多
基金Supported by the Clinical Medical Technology Innovation Guiding Project of Hunan Province,No.2021SK50911Scientific Research Project of Hunan Health Commission,No.202209010030.
文摘BACKGROUND Inflammatory myofibroblastic tumor(IMT)is a relatively rare tumor.The global incidence of IMT is less than 1%.There is no specific clinical manifestation.It usually occurs in the lungs,but the pancreas is not the predilection site.CASE SUMMARY We present a case of a male patient,51 years old,who was diagnosed with a pancreatic neck small mass on ultrasound one year ago during a physical examination.As he had no clinical symptoms and the mass was relatively small,he did not undergo treatment.However,the mass was found to be larger on review,and he was referred to our hospital.Since the primal clinical diagnosis was pancreatic neuroendocrine tumor,the patient underwent surgical treatment.However,the case was confirmed as pancreatic IMT by postoperative pathology.CONCLUSION Pancreatic IMT is relatively rare and easily misdiagnosed.We can better understand and correctly diagnose this disease by this case report.
文摘BACKGROUND Low-grade myofibroblastic sarcoma(LGMS)is a rare spindle cell sarcoma espe-cially in the pancreas,with myofibroblastic differentiation.Hitherto,only a few cases have been reported.CASE SUMMARY Herein,we report a case involving the discovery of a pancreatic mass detected during a routine physical examination.Subsequent imaging and pathological tests of the patient led to the diagnosis of LGMS of the pancreas.Following surgical intervention,the patient experienced recurrence and metastasis.Conventional treatment is not effective for postoperative recurrent pancreatic LGMS with multiple metastases.After communicating with the patients and their families,informed consent was obtained for the treatment of anlotinib combined with pembrolizumab.Evaluation of imaging and clinical symptoms post-treatment revealed a relatively favorable response to the combination of anlotinib and pembrolizumab.CONCLUSION Based on the comprehensive literature review,our report aimed to provide evidence for a better understanding of the disease characteristics,diagnostic criteria,imaging findings,and identification of LGMS.And explore novel treatment strategies for this disease.
文摘BACKGROUND The inflammatory myofibroblastic tumor(IMT)is a rare mesenquimal tumor of doubtful biological behaviour.It’s characterised for affecting mainly children and young adults,although it can appear at any age,being the lungs the primary affected organ(in children it represents 20%of all primary pulmonary tumors).CASE SUMMARY We present the case of a 45 year old woman,with a computed tomography(CT)finding of injury on the anterior surface of the fundus/gastric body and a solid perigastric injury of 12 mm in the ecoendoscopy.The case is presented in the tumor committee deciding to perform a laparoscopic wedge resection.The histological diagnosis was a IMT.The diagnosis is based on imaging tests like the abdominal CT,abdominal ecography and the ecoendoscopy but to confirm the diagnosis a pathological study is necessary.CONCLUSION Due to the unpredictable nature of this tumor,surgical resection is the best therapeutic option.
基金Supported by Shandong Provincial Natural Science Foundation of China,No.ZR2021MH033Postgraduates Foundation of Linyi People’s Hospital(Tong Meng).
文摘BACKGROUND Hepatic inflammatory myofibroblastic tumor(HIMT)is a rare type of hepatic tumor.It is always misdiagnosed and mistreated because it is primarily found with no obvious specific manifestation,and its imaging findings are diverse.CASE SUMMARY Here,we report a case of HIMT that was initially diagnosed as liver malignancy but was confirmed as HIMT by histopathology after hepatectomy.Mostly,HIMTs are infiltrated with plasma cells and stain positively for anaplastic lymphoma kinase on immunohistochemistry as well as for some other kinases.CONCLUSION HIMT can be treated with single nonsteroidal anti-inflammatory drugs and without surgery when it is diagnosed accurately.Because the etiology of HIMT is unknown and the diagnosis is difficult,the pathogenesis and clinical process need to be further studied.
文摘Inflammatory myofibroblastic tumor(IMT)of the biliary tract is rare,and often difficult to diagnose or to distinguish from other tumors due to its atypical clinical presentation and nonspecific radiological features.Histologically,IMTs are(myo)fibroblastic neoplasms with a prominent inflammatory infiltrate.They are characterized by receptor tyrosine kinase gene rearrangements,most often involving an anaplastic lymphoma kinase(ALK)translocation.The final diagnosis of IMT depends on histopathology and immunohistochemical examination.In this manuscript,we provide a clinical and morphomolecular overview of IMT and the difficulties that may arise in using immunohistochemical and molecular techniques in diagnosing IMT.
文摘Plexiform angiomyxoid myofibroblastic tumor (PAMT) is a recently described gastric tumor with a peculiar plexiform pattern, bland spindle cells and a myxoid stroma rich in arborizing blood vessels. PAMT of the stomach is a very rare tumor without distinctive clinical manifestations. In this study, we report a new case of PAMT which is the first Chinese case in English literature. A 47-year-old Chinese woman was admitted with a 6-month history of intermittent epigastric discomfort, and abdominal pain for 2 months. Gastroscopy showed an elevated mass in the anterior wall of the gastric antrum. Endoscopic ultrasound revealed a focal hypoechoic lesion protruding into the lumen. A laparoscopic distal gastrectomy was performed, and the patient made an uneventful recovery and remains well 1.5 years later. A diagnosis of PAMT was made by histopathology and immunochemistry.
文摘Inflammatory myofibroblastic tumor(IMT) is an uncommon benign neoplasm with locally aggressive behavior but malignant change is rare.We report an unusual case of pelvic-abdominal inflammatory myofibroblastic tumor with malignant transformation in a 14-year-old boy presenting with abdominal pain and 9 kg body weight loss in one month.Computed tomography revealed a huge pelvi-abdominal mass(30 cm),possibly originating from the pelvic extraperitoneal space,protruding into the abdomen leading to upward displacement of the bowel loops,downward displacement of the urinary bladder,massive central necrosis,a well-enhanced peripheral solid component with prominent peritumoral vascularity.Subsequent examination confirmed the computed tomographic findings.Histopathologic examination revealed proliferative epitheloid and spindle cells,inflammatory cell infiltration and high mitotic counts.Immunohistochemistry was strongly positive for anaplastic lymphoma kinase and revealed a high proliferative index(ki-67 = 40%).DNA sequencing and electronic microscopy further confirmed the primitive fibroblastic cell phenotype of the tumor and a final diagnosis of inflammatory myofibroblastic tumor with malignant transformation was established.Rapid tumor recurrence was noted 20 d after radical tumor resection.To our knowledge,this is the largest documented case of IMT in a pediatric patient and the first report of IMT with malignant transformation originating from the pelvic extraperitoneal space.
文摘Low-grade myofibroblastic sarcoma (LGMS) is a rare, malignant tumor with myofibroblastic differentiation. Despite it being classified as a distinct entity by the World Health Organization, a few cases were reported in the oral and maxillofacial region. Here, a LGMS developed on the palate of a 73-year-old man who presented with a 1-cm tumor on the posterior border of the palate. Based on the histological and immunohistochemical features, a diagnosis of LGMS was established. The tumor was resected, and no recurrence was observed over 2 years. Although the tongue is the most preferred site for LGMS, it may occur in any region of the oral cavity.
文摘Inflammatory myofibroblastic tumors are usually treated by surgical resection. We herein report two cases of intra-abdominal inflammatory myofibroblastic tumors that were unresectable and underwent spontaneous regression without any treatment. Our case report and literature review show that regression is more common in the middle-aged and older male populations. Abdominal discomfort and fever were the most common symptoms, but the majority of patients had no obvious physical signs. There was no specific indicator for diagnosis. The majority of the lesions regressed within3 mo and nearly all of the masses completely resolved within 1 year. We conclude that the clinical characteristics of inflammatory myofibroblastic tumors are variable and, accordingly, the disease needs to be subdivided and treated on an individual basis. Surgery is always the first-line treatment; however, for those masses assessed as unresectable, conservative therapy with in-tense follow-up should be considered.
文摘Only two cases of myofibroblastic sarcoma in the liver have been reported in the literature. Here, we report the case of a male patient with high-grade myofibroblastic sarcoma mimicking echinococcosis in the liver. The 25-year-old male patient complained of right upper quadrant swelling pain for one week and was initially diagnosed with echinococcosis. He was then scheduled for an exploratory laparotomy. During the operation, a huge mass exceeding 16 cm in diameter was found to occupy nearly the entire right trisegment of the liver, with a clear boundary and a round shape, and the mass was resected by right hepatic trisegmentectomy. Immunohistochemical staining revealed that the tumor tissue was positive for desmin, α-smooth muscle actin, CD56, and vimentin and negative for ALK-1, myogenin, calponin, β-catenin, S100, and glypican-3, with a Ki-67(MIB-1) index of approximately 20%. Based on the histological manifestations and immunohistochemical staining, a diagnosis of myofibroblastic sarcoma was established. The postoperative recovery was uneventful. There was no evidence of recurrence or metastasis through the last follow-up, 6 mo after surgery, despite a lack of postoperative chemotherapy or radiotherapy. To the best of our knowledge, the present case is the first reported case of high-grade myofibroblastic sarcoma in the liver, and it is also the first reported case in a male patient.
基金MEXT,KAKENHI,No.15K08978,No.22790677 and No.25860571a MEXT-Supported Program supporting research activities of female researchers+1 种基金the Clinical Research Foundationthe Central Research Institute of Fukuoka University,No.151045 and No.147104
文摘AIM To investigate the anti-fibrotic effects of the traditional oriental herbal medicine Daikenchuto(DKT) associated with transient receptor potential ankyrin 1(TRPA1) channels in intestinal myofibroblasts. METHODS Inflammatory and fibrotic changes were detected in a2,4,6-trinitrobenzenesulfonic acid(TNBS) chronic colitis model of wild-type and TRPA1-knockout(TRPA1-KO) mice via pathological staining and immunoblotting analysis.Ca^(2+) imaging experiments examined the effects of DKT and its components/ingredients on intestinal myofibroblast(In Myo Fib) cell TRPA1 channel function.Profibrotic factors and transforming growth factor (TGF) -β1-associated signaling were tested in an In Myo Fib cell line by q PCR and immunoblotting experiments.Samples from non-stenotic and stenotic regions of the intestines of patients with Crohn’s disease (CD) were used for pathological analysis. RESULTS Chronic treatment with TNBS caused more severe inflammation and fibrotic changes in TRPA1-KO than in wild-type mice.A one-week enema administration of DKT reduced fibrotic lesions in wild-type but not in TRPA1-KO mice.The active ingredients of DKT,i.e.,hydroxyα-sanshool and 6-shogaol,induced Ca^(2+) influxes in In Myo Fib,and this was antagonized by co-treatment with a selective TRPA1 channel blocker,HC-030031.DKT counteracted TGF-β1-induced expression of TypeⅠcollagen andα-smooth muscle actin (α-SMA) ,which were accompanied by a reduction in the phosphorylation of Smad-2 and p38-mitogen-activated protein kinase (p38-MAPK) and the expression of myocardin.Importantly,24-h incubation with a DKT active component Japanese Pepper increased the m RNA and protein expression levels of TRPA1 in In Myo Fibs,which in turn negatively regulated collagen synthesis.In the stenotic regions of the intestines of CD patients,TRPA1 expression was significantly enhanced.CONCLUSION The effects of DKT on the expression and activation of the TRPA1 channel could be advantageous for suppressing intestinal fibrosis,and benefit inflammatory bowel disease treatment.
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.
文摘BACKGROUND Inflammatory myofibroblastic tumors of the liver(IMTL)are extremely rare neoplasms and very little is known about their clinical presentation,pathogenesis,and biological behavior.Due to their absolute rarity,it is almost impossible to obtain a definite diagnosis without histological examination.Because of their intermediate biological behavior with the risk for local recurrence and metastases,surgical resection is recommend whenever IMTL is suspect.CASE SUMMARY We herein present a case of an otherwise healthy 32-year-old woman who presented with intermittent fever,unclear anemia,malaise and right flank pain 4 mo postpartum.The liver mass in segment IVa/b was highly FDG avid in the positron emission tomography-computed tomography.Hepatic resection was performed achieving a negative resection margin and an immediate resolution of all clinical symptoms.Histological analysis diagnosed the rare finding of an inflammatory myofibroblastic tumor of the liver and revealed cytoplasmic anaplastic lymphoma kinase expression by immunohistochemistry.Twelve months follow-up magnetic resonance imaging showed no recurrence and no metastases in the fully recovered patient.CONCLUSION IMTLs are extremely rare and difficult to diagnose.Due to their intermediate biological behavior,surgical resection should be perform whenever feasible and patients should be followed-up in order to detect recurrence and metastasis as early as possible.
文摘Fibroblasts are the most abundant cellular components of connective tissue. They possess phenotypical heterogenicity and may be present in the form of smooth muscle cells or myofibroblasts(MFs). MFs are spindle-shaped cells with stress fibres and welldeveloped fibronexus,and they display α-smooth muscle actin immunohistochemically and smoothmuscle myofilaments ultrastructurally. MFs play a crucial role in physiological and pathological processes. Derived from various sources,they play pivotal roles not only by synthesizing and producing extracellular matrix components,such as other connective tissue cells,but also are involved in force production. In the tissue remodelling phase of wound closure,integrinmediated interactions between MFs and type I collagen result in scar tissue formation. The tumour stroma in oral cancer actively recruits various cell types into the tumour mass,where they act as different sources of MFs. This article reviews the importance of MFs and its role in pathological processes such as wound healing,odontogenic cysts and tumours,salivary gland tumours,oral preneoplasia,and oral squamous cell carcinoma. Research oriented on blocking the transdifferentiation of fibroblasts into MFs can facilitate the development of noninvasive therapeutic strategies for the treatment of fibrosis and/or cancer.
基金supported by grants from the National Nature Science Foundation of China(No.81070190)the Foundation of Natural Sciences of Hubei Province of China(No.2014CFB962)
文摘In order to investigate the roles of Wnt signal pathway in transformation of cardiac valvular myofibroblasts to the osteoblast-like phenotype, the primary cultured porcine aortic valve myofibroblasts were incubated with oxidized low density lipoprotein(ox-LDL, 50 mg/L), and divided into four groups according to the ox-LDL treatment time: control group, ox-LDL 24-h group, ox-LDL 48-h group, and ox-LDL 72-h group. Wnt signal pathway blocker Dickkopf-1(DDK-1, 100 μg/L) was added in ox-LDL 72-h group. The expression of α-smooth muscle actin(α-SMA), bone morphogenetic protein 2(BMP2), alkaline phosphatase(ALP), and osteogenic transcription factor Cbfa-1 was detected by Western blotting, and that of β-catenin, a key mediator of Wnt signal pathway by immunocytochemical staining method. The Wnt/β-catenin was observed and the transformation of myofibroblasts to the osteoblast-like phenotype was examined. The expression of α-SMA, BMP2, ALP and Cbfa-1 proteins in the control group was weaker than in the ox-LDL-treated groups. In ox-LDL-treated groups, the protein expression of α-SMA, BMP2, ALP, and Cbfa-1 was significantly increased in a time-dependent manner as compared with the control group, and there was significant difference among the three ox-LDL-treated groups(P〈0.05 for all); β-catenin protein was also up-regulated in the ox-LDL-treated groups in a time-dependent manner as compared with the control group(P〈0.05), and its transfer from cytoplasm to nucleus and accumulation in the nucleus were increased in the same fashion(P〈0.05). After addition of DKK-1, the expression of α-SMA, bone-related proteins and β-catenin protein was significantly reduced as compared with ox-LDL 72-h group(P〈0.05). The Wnt/ β-catenin signaling pathway may play an important role in transformation of valvular myofibroblasts to the osteoblast-like phenotype.
基金Supported by the Science and Technology Research and Development Foundation of Shenzhen,No.JCYJ20170307094039571
文摘BACKGROUND Inflammatory myofibroblastic tumor(IMT) is a rare mesenchymal tumor that is characterized by spindle cells differentiated from muscle fibroblasts and infiltration of various types of inflammatory cells. IMT can occur at any age and at any anatomic site. The most common location of IMT is the bladder in the genitourinary tract. Only scarce cases of kidney IMT have been reported in the literature.CASE SUMMARY A 77-year-old woman, with a history of bilateral renal calculus for 15 years, was admitted to the Department of Urology of our hospital complaining of recurrent painless gross hematuria for one month. The treatment with cephalosporin was ineffective. Computed tomography imaging showed a mixed density and slightly heterogeneously enhanced lesion in the middle pole of the left kidney and ipsilateral adrenal enlargement. The patient underwent surgical treatment by retroperitoneoscopic left radical nephrectomy plus adrenalectomy. A large number of typical spindle cells surrounded by plasma cells and lymphocytes were observed microscopically. Immunohistochemical analyses indicated that these spindle cells were positive for vimentin, cytokeratin(CK), Ki-67, CK7,CD34, and CD31 and were focally positive for CD10 and anaplastic lymphoma kinase(ALK-1). Thus, a diagnosis of IMT was made definitively. The patient recovered well after operation, and no recurrence or metastasis was noted during the 22-mo follow-up.CONCLUSION Since kidney IMT is very rare and lacks characteristic clinical manifestation, it is easily misdiagnosed as a malignant tumor before operation. Surgery remains the best choice for diagnosis and treatment, and such cases must be followed carefully because of the uncertain biological behavior of this tumor. This report suggests that renal calculus may be one of the causes of IMT, but further investigation is necessary to prove it.
文摘The etiology and pathogenesis of pulmonary fibrosis is poorly understood. We and others reported that M-CSF/CSF-1, M-CSF-R and downstream AKT activation plays an important role in lung fibrosis in mice models and in IPF patients. To understand potential molecular pathways used by M-CSF-R activation to direct lung fibrosis, we used a novel transgenic mouse model that expresses a constitutively-active form of AKT, myristoylated AKT (Myr-Akt), driven by the c-fms (M-CSF-R) promoter. We were particularly interested in the basal immune state of the lungs of these Myr-Akt mice to assess M-CSF-R-related priming for lung fibrosis. In support of a priming effect, macrophages isolated from the lungs of unchallenged Myr-Akt mice displayed an M2-tropism, enhanced co-expression of M-CSF-R and α-SMA, reduced autophagy reflected by reduced expression of the key autophagy genes Beclin-1, MAP1-Lc3a(Lc3a), and MAP1-Lc3b(Lc3b), and increased p62/STSQM1 expression compared with littermate WT mice. Furthermore, Myr-Akt mice had more basal circulating fibrocytes than WT mice. Lastly, upon bleomycin challenge, Myr-Akt mice showed enhanced collagen deposition, increased F4/80+ and CD45+ cells, reduced autophagy genes Beclin-1, Lc3a, and Lc3b expression, and a shorter life-span than WT littermates. These data provide support that M-CSF-R/AKT activation may have a priming effect which can predispose lung tissue to pulmonary fibrosis.
文摘Inflammatory myofibroblastic tumor(IMT)occurs infrequently in the liver.It is controversial whether it represents a low grade mesenchymal neoplasm or a reactive inflammatory lesion.Local recurrence and metastasis are rare and some tumors are associated with infectious agents.We report on a case of a large and partially resected IMT with local recurrence and diaphragm and kidney infiltration detected on routine surveillance two years later.Histologically,the tumor showed spindle cells without atypia,mitosis or necrotic areas in a myxoid and collagenized background with inflammatory cells.In the liver portal tracts,granulomatous lesions with viable eggs of Schistosoma mansoni were identified.Immunohistochemistry demonstrated spindle cells which were smooth-muscle actin and vimentin positive.In conclusion,this case points out that these histological patterns do not predict the aggressive biological behavior of the lesion.A reason for the recurrence and the infiltration may be incomplete tumor resection.Further investigation is necessary in order to better clarify an infectious cause in some IMTs.
文摘BACKGROUND Pancreatic inflammatory myofibroblastic tumor(IMT)is a relatively rare disease that is often confused with pancreatic cancer or pancreatic neuroendocrine tumors.The histological features of IMTs show that tissue from this type of tumor contains an intermingling of fibroblast and myofibroblast proliferation,accompanied by a varying degree of inflammatory cell infiltration.CASE SUMMARY The management of an IMT occurring at the neck of the pancreas is presented in this paper.A 66-year-old female patient was diagnosed with a pancreatic neck mass after a series of tests.The patient underwent enucleation of the pancreatic neck tumor after a pathological diagnosis of IMT.Previous research on the clinical features,pathological diagnosis and treatment of pancreatic IMTs was reviewed.Compared with previous reports,this is a unique case of enucleation of a pancreatic IMT.CONCLUSION The enucleation of pancreatic IMTs may be a safe and efficient surgical method for managing such tumors with a better prognosis.Further cases are required to explore surgical measures for pancreatic IMTs.
文摘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.