Mucinous cystic neoplasms of the liver(MCN-L)are rare cystic lesions characterized by mucin-producing epithelium and ovarian-like stroma.Although they constitute fewer than 5%of hepatic cystic lesions,MCN-L poses sign...Mucinous cystic neoplasms of the liver(MCN-L)are rare cystic lesions characterized by mucin-producing epithelium and ovarian-like stroma.Although they constitute fewer than 5%of hepatic cystic lesions,MCN-L poses significant diagnostic challenges due to overlapping features with other cystic lesions and their potential for malignant transformation.Early recognition and definitive surgical intervention are therefore critical to ensure optimal patient outcomes.A literature review was conducted to summarize epidemiology,clinical presentation,diagnostic modalities,and management strategies for MCN-L.Additionally,from 2019 to 2025,9 patients with MCN-L were identified at our center.Clinical data and outcomes were retrospectively analyzed.MCN-L predominantly affects middle-aged women and presents as large,multiloculated cystic lesions without biliary communication.The revised 2010 World Health Organization classification emphasizes the presence of ovarian-like stroma for definitive diagnosis.Contrast-enhanced computed tomography or magnetic resonance imaging are often suggestive but not pathognomonic,reinforcing the need for histopathological confirmation.MCN-L remains a diagnostic and therapeutic challenge due to its resemblance to other cystic liver lesions.Complete surgical resection is the treatment of choice to prevent recurrence and malignant transformation,reinforcing the importance of early intervention.Further research is needed to improve diagnostic accuracy and refine management strategies.展开更多
BACKGROUND Acinar cystic transformation(ACT)of the pancreas is a rare non-neoplastic transformation of the pancreas.Adult women are the majority of patients with ACT,but few cases have been reported in pediatric patie...BACKGROUND Acinar cystic transformation(ACT)of the pancreas is a rare non-neoplastic transformation of the pancreas.Adult women are the majority of patients with ACT,but few cases have been reported in pediatric patients.Given that there are currently no guidelines for the treatment of ACT,current treatment is based primarily on expert opinions and clinical experiences.Here,we report the case of the youngest child with ACT to date.Additionally,a literature review on pediatric ACT cases was performed to summarize previous clinical experience and treatment methods.CASE SUMMARY A 1-year-old Chinese girl presented with progressive abdominal distension for 6 months.A detailed consultation revealed an uneventful history.The patient showed no signs of fever or abdominal pain and had a good appetite and normal feces.A mass of about 20 cm×10 cm×10 cm in size was detected in the abdomen.Both abdominal ultrasound and computed tomography examination revealed a multilocular cystic mass about 21.7 cm×16.8 cm×8.9 cm in size.At first,due to the large size and the possible retroperitoneal origin of the cyst,a total resection of the lesion was not possible.A single-port laparoscopic lymphangioma puncture and Pingyangmycin injection were performed in March 2023.One month after surgery,the abdominal cyst rapidly enlarged to its pre-operative size.After consulting with the experts in the angiology department and interventional department,sclerotherapy combined with oral sirolimus was performed in May 2023.After confirming that the tumor was not sensitive to sclerotherapy combined with oral sirolimus,our surgical team performed tumor reduction in August 2023.This surgery confirmed that the polycystic mass originated from the head of the pancreas,and pathological and immunohistochemical findings diagnosed pancreatic ACT.The patient showed no signs of cyst lesions after 6 months of follow-up and remains in good health up to the time of this report.CONCLUSION ACT is a rare non-neoplastic transformation of the pancreas,more rarely seen in children.Manifestation and examinations show no specificity for diagnosis,and final diagnosis is mainly based on histological findings.To reach a specific diagnosis and rule out malignancy is a priority in clinical practice,and repeated biopsy or radical surgery should be considered before malignancy is ruled out.However,once a diagnosis of ACT is made,a conser-vative treatment with consecutive follow-up is recommended until symptoms present or obvious enlargement occurs because ACT is considered a slow-growing and benign tumor.展开更多
BACKGROUND As a heterogeneous group of lesions,pancreatic cystic lesions(PCLs)vary enormously in malignant potential,mandating different treatment strategies.Despite significant advances in diagnostic imaging and labo...BACKGROUND As a heterogeneous group of lesions,pancreatic cystic lesions(PCLs)vary enormously in malignant potential,mandating different treatment strategies.Despite significant advances in diagnostic imaging and laboratory tests,the accurate diagnosis of PCLs remains challenging,leading to overtreatment or delayed/missed surgical timing in patients with PCLs.CASE SUMMARY We present a case of a 64-year-old female patient in whom an asymptomatic,incidental cystic mass was found in the pancreatic tail on a routine abdominal ultrasound.After a comprehensive work-up with laboratory examinations,contrast-enhanced computed tomography,magnetic resonance imaging,and magnetic resonance cholangiopancreatography,a pancreatic pseudocyst was suspected.Subsequent endoscopic ultrasound with fine-needle aspiration and needle-based confocal laser endomicroscopy supported a benign diagnosis.Follow-up computed tomography and magnetic resonance imaging examinations five months later showed significant cyst shrinkage without any abnormalities.However,three years after being lost to follow-up,the patient was readmitted and diagnosed with pancreatic adenocarcinoma with multiple metastases,suggesting that the initial lesion was a mucinous cystic neoplasm misdiagnosed as a pan-creatic pseudocyst.CONCLUSION Comprehensive integration of all available information(e.g.,cyst features,abnormal imaging findings,cyst biochemistry,clinical history,and patient demographics)rather than over-reliance on imaging or endoscopic findings is pivotal to diagnosing PCLs,and patients with concerning features should undergo strict surveillance.展开更多
Individuals with congenital absence of the vas deferens(CAVD)may transmit cystic fibrosis(CF)-causing variants of the cystic fibrosis transmembrane conductance regulator(CFTR)gene to their offspring through assisted r...Individuals with congenital absence of the vas deferens(CAVD)may transmit cystic fibrosis(CF)-causing variants of the cystic fibrosis transmembrane conductance regulator(CFTR)gene to their offspring through assisted reproductive technology(ART).We aimed to delineate the spectrum and estimate the prevalence of CF-causing variants in Chinese individuals with CAVD through a cohort analysis and meta-analysis.CFTR was sequenced in 145 Chineseindividuals with CAVD.CFTR variants were classified as CF-causing or non-CF-causing variants regarding clinical significance.A comprehensive genotype analysis was performed in Chinese individuals with CAVD,incorporating previous studies and our study cohort.The prevalence of CF-causing variants was estimated through meta-analysis.In our cohort,56 differentCFTR variants were identified in 108(74.5%)patients.Twenty variants were categorized as CF-causing and were detected in 28(19.3%)patients.A comprehensive genotype analysis of 867 patients identified 174 differentCFTR variants.Sixty-four were classified as CF-causing variants,56.3%of which had not been previously reported in Chinese patients with CF.Meta-analysis showed that 14.8%(95%confidence interval[CI]:11.0%-18.9%)CAVD cases harbored one CF-causing variant,and 68.6%(95%CI:65.1%-72.0%)CAVD cases carried at least one CFTR variant.Our study underscores the urgent need for extensiveCFTR screening,including sequencing of whole exons and flanking regions and detection of large rearrangements and deep intronic CF-causing variants,in Chinese individuals with CAVDbefore undergoing ART.The established CF-causing variants spectrum may aid in the development of genetic counseling strategies and preimplantation diagnosis to prevent the birth of a child with CF.展开更多
BACKGROUND Malignant transformation(MT)of mature cystic teratoma(MCT)has a poor prognosis,especially in advanced cases.Concurrent chemoradiotherapy(CCRT)has an inhibitory effect on MT.CASE SUMMARY Herein,we present a ...BACKGROUND Malignant transformation(MT)of mature cystic teratoma(MCT)has a poor prognosis,especially in advanced cases.Concurrent chemoradiotherapy(CCRT)has an inhibitory effect on MT.CASE SUMMARY Herein,we present a case in which CCRT had a reduction effect preoperatively.A 73-year-old woman with pyelonephritis was referred to our hospital.Computed tomography revealed right hydronephrosis and a 6-cm pelvic mass.Endoscopic ultrasound-guided fine-needle biopsy(EUS-FNB)revealed squamous cell carci-noma.The patient was diagnosed with MT of MCT.Due to her poor general con-dition and renal malfunction,we selected CCRT,expecting fewer adverse effects.After CCRT,her performance status improved,and the tumor size was reduced;surgery was performed.Five months postoperatively,the patient developed dis-semination and lymph node metastases.Palliative chemotherapy was ineffective.She died 18 months after treatment initiation.CONCLUSION EUS-FNB was useful in the diagnosis of MT of MCT;CCRT suppressed the disea-se and improved quality of life.展开更多
Objective:In recent years,the incidence and detection rate of pancreatic cystic lesions(PCLs)have increased significantly.Endoscopic ultrasound(EUS)plays an indispensable role in the diagnosis and differential diagnos...Objective:In recent years,the incidence and detection rate of pancreatic cystic lesions(PCLs)have increased significantly.Endoscopic ultrasound(EUS)plays an indispensable role in the diagnosis and differential diagnosis of PCLs.However,evidence comparing the diagnostic performance of EUS-guided fine-needle aspiration(EUS-FNA)and fine-needle biopsy(FNB)remains limited.This study aims to compare the diagnostic yield,adequacy of tissue acquisition,and safety between EUS-FNA and EUS-FNB in evaluating PCLs to inform clinical practice.Methods:A retrospective review was conducted on patients with PCLs who underwent either EUS-FNA or EUS-FNB between January 2014 and August 2021.The diagnostic yield,tissue acquisition adequacy,and incidence of adverse events were compared between the 2 groups.Results:A total of 90 patients with PCLs were included(52 in the FNA group and 38 in the FNB group).The diagnostic yield was similar between the FNA and FNB groups(94.2%vs 94.7%,P>0.05).The adequacy of tissue acquisition was 71.2%in the FNA group and 81.6%in the FNB group(P>0.05).No statistically significant difference was observed in the incidence of adverse events between the 2 groups(P>0.05).Conclusion:Both EUS-FNA and EUS-FNB demonstrate equally high diagnostic yields and tissue adequacy in PCLs,with excellent safety profiles.Both methods are safe and effective diagnostic tools for evaluating PCLs.展开更多
BACKGROUND Neonatal screening(NS)is a public health policy to identify genetic pathologies such as cystic fibrosis(CF),sickle cell disease,and other diseases.Sickle cell disea-se is the comprehensive term for a group ...BACKGROUND Neonatal screening(NS)is a public health policy to identify genetic pathologies such as cystic fibrosis(CF),sickle cell disease,and other diseases.Sickle cell disea-se is the comprehensive term for a group of hemoglobinopathies characterized by the presence of hemoglobin S.CF is an autosomal recessive multisystemic disease with pathophysiology involving deleterious mutations in the transmembrane re-gulatory gene that encodes a protein that regulates the activity of chloride and sodium channels in the cell surface epithelium.NS is crucial for early diagnosis and management,which ensures a better quality of life.AIM To report a case of the coexistence of sickle cell anemia(SCA)and CF and perform an integrative literature review.METHODS This is an observational study and a review of the literature focusing on two rare genetic pathologies identified simultaneously in NS from the perspective of a clinical case.The authors identified only 5 cases of SCA associated with CF.No clinical trials or review articles were identified considering the rarity of the coexistence of these two pathologies.RESULTS Herein,the authors reported the case of a girl who after undergoing NS on day 8 of life was diagnosed with SCA with an alteration in the dosage of immunoreactive trypsin.The diagnosis of CF was confirmed by the Coulometry Sweat Test.The rarity of the co-occurrence of these two severe genetic pathologies(CF and SCA)is a challenge for medical science.CONCLUSION This study adds to the few case reports present in the literature that highlight the identification of two severe diseases via NS.展开更多
BACKGROUND Alveolar and cystic echinococcoses are lethal zoonotic diseases caused by Echinococcus multilocularis and Echinococcus granulosus infections,leading to alveolar echinococcosis(AE)or cystic echinococcosis(CE...BACKGROUND Alveolar and cystic echinococcoses are lethal zoonotic diseases caused by Echinococcus multilocularis and Echinococcus granulosus infections,leading to alveolar echinococcosis(AE)or cystic echinococcosis(CE),respectively.No study has hitherto reported effective treatment approaches for AE or CE with concurrent hepatorenal involvement.AIM To investigate the feasibility and efficacy of simultaneous combined surgery(SCS)as a comprehensive treatment approach for patients with hepatorenal echinococcosis.METHODS Clinical datasets of hepatorenal AE(n=10)and CE(n=11)patients were retrospectively collected and systematically analyzed.The SCS approach was introduced,and surgical outcomes,complications,and prognoses were documented in detail.RESULTS The SCS approach incorporated hybridized techniques,including partial hepatectomy,partial or total nephrectomy,ex vivo liver resection and autotransplantation,and total or subtotal cystectomy with endocystectomy.Radical SCS was achieved in 100%of AE patients and 63.6%of CE patients.All surgeries were completed without intraoperative complications.The short-term complication rate was 28.6%(Clavien-Dindo classification:AE-1 IIIb,3 IIIa;CE-2 II),while the long-term complication rate was 4.8%(Clavien-Dindo classification:AE-1 IIIb).Patients were followed up for a median of 37 months(AE:6-81 months;CE:34-123 months),with no reported deaths or disease relapses.CONCLUSION CS appears to be a feasible and effective treatment method for patients with hepatorenal involvement of AE or CE.It fulfills the management criteria for advanced AE or CE cases,aiming to maximize patient benefits.展开更多
BACKGROUND Acute cholecystitis due to unintended cystic artery embolism is an uncommon and mostly self-limiting complication after transarterial chemoembolization procedure for treatment of hepatocellular carcinoma.Us...BACKGROUND Acute cholecystitis due to unintended cystic artery embolism is an uncommon and mostly self-limiting complication after transarterial chemoembolization procedure for treatment of hepatocellular carcinoma.Usually,conservative management is sufficient for complete recovery of patients who develop this complication.If conservative treatment is ineffective,urgent surgical inter-vention may be required to prevent the progression of complications.CASE SUMMARY This article reports a rare and serious case of acute cholecystitis complicated by gallbladder necrosis and biliary peritonitis,which was initially treated conservatively but eventually necessitated emergency laparotomy.The patient initially presented with equivocal symptoms of fever and upper abdominal pain and distention,which worsened at the two weeks mark along with emergence of signs of peritonitis.This was managed by emergency laparotomy and cholecystostomy,allowing rapid symptom relief.The patient ultimately discharged and succumbed to advanced liver cancer 11 months after diagnosis.CONCLUSION After cholecystostomy,the patient showed symptom relief and was discharged,surviving 11 months post-stage IIIB liver cancer diagnosis.展开更多
BACKGROUND Leiomyomas or fibroids commonly originate from the uterus;extrauterine leiomyomas are rare and most often arise from the broad ligament.Diagnosing broad ligament leiomyomas becomes particularly challenging ...BACKGROUND Leiomyomas or fibroids commonly originate from the uterus;extrauterine leiomyomas are rare and most often arise from the broad ligament.Diagnosing broad ligament leiomyomas becomes particularly challenging when they undergo degenerative changes because their clinical and radiological features often mimic those of ovarian tumors.We report a rare case of a giant broad ligament fibroid with cystic degeneration,which was initially mistaken for an ovarian mass.CASE SUMMARY A 49-year-old woman presented with mild abdominal distension and pain as the only symptoms.Upon abdominal examination,a large mass measuring approximately 30 cm and extending from the pelvic cavity to just below the xiphoid process was identified.Both transvaginal ultrasound and contrast-enhanced computed tomography suggested an ovarian origin of the mass.However,laparotomy confirmed that the mass originated from the right broad ligament.The mass was separated from the uterus and bilateral ovaries,with no involvement of the uterus or ovaries.The mass was completely resected with respecting the patient’s desire to retain her uterus and adnexa.Postoperative histopathological examination confirmed leiomyoma with cystic degeneration.CONCLUSION Broad ligament myomas mimic ovarian tumors;accurate diagnosis and careful operation are critical to avoid complications and ensure safety.展开更多
BACKGROUND Cystic lymphangioma is a rare hamartoma that is especially found in the adult gastrointestinal tract.In the early stage,most patients are asymptomatic;after the onset of symptoms,there is often no specifici...BACKGROUND Cystic lymphangioma is a rare hamartoma that is especially found in the adult gastrointestinal tract.In the early stage,most patients are asymptomatic;after the onset of symptoms,there is often no specificity regarding symptoms.CASE SUMMARY Here we report the endoscopic diagnosis and treatment of an adult patient with cystic lymphangioma of the ascending colon.One patient who came to our hospital with“dull pain in the left lower abdomen for 2 days”was initially misdiagnosed with a colon cyst according to endoscopy and then underwent endoscopic submucosal dissection.The final pathological results suggested cystic lymphangioma.One year later,no recurrence was found on re-examination via colonoscopy.CONCLUSION Cystic lymphangioma in the gastrointestinal tract rarely occurs in adults and is easily misdiagnosed or missed.Endoscopy,imaging,histology,and immunohistochemical staining are useful for diagnosis.Surgical resection is the preferred treatment.展开更多
Adenoid cystic carcinoma of the lacrimal gland(LGACC)is the most common type of malignant epithelial tumor of the lacrimal gland,which is characterized by a high recurrence rate,perineural invasion,and a propensity to...Adenoid cystic carcinoma of the lacrimal gland(LGACC)is the most common type of malignant epithelial tumor of the lacrimal gland,which is characterized by a high recurrence rate,perineural invasion,and a propensity to metastasize to distant sites.Due to its unclear pathogenesis,LGACC has a poor prognosis and a high mortality rate.In recent years,a range of radiotherapy and chemotherapy have been clinically applied,leading to a shift in the treatment approach for LGACC.This article discussed the advances being made in the treatment of LGACC and provides readers with an overview of the impact of LGACC treatment modalities on patient survival and prognostic levels.展开更多
BACKGROUND Choledochal cysts(CC)and cystic biliary atresia(CBA)present similarly in early infancy but require different treatment approaches.While CC surgery can be delayed until 3-6 months of age in asymptomatic pati...BACKGROUND Choledochal cysts(CC)and cystic biliary atresia(CBA)present similarly in early infancy but require different treatment approaches.While CC surgery can be delayed until 3-6 months of age in asymptomatic patients,CBA requires intervention within 60 days to prevent cirrhosis.AIM To develop a diagnostic model for early differentiation between these conditions.METHODS A total of 319 patients with hepatic hilar cysts(<60 days old at surgery)were retrospectively analyzed;these patients were treated at three hospitals between 2011 and 2022.Clinical features including biochemical markers and ultrasonographic measurements were compared between CC(n=274)and CBA(n=45)groups.Least absolute shrinkage and selection operator regression identified key diagnostic features,and 11 machine learning models were developed and compared.RESULTS The CBA group showed higher levels of total bile acid,total bilirubin,γ-glutamyl transferase,aspartate aminotransferase,and alanine aminotransferase,and direct bilirubin,while longitudinal diameter of the cysts and transverse diameter of the cysts were larger in the CC group.The multilayer perceptron model demonstrated optimal performance with 95.8% accuracy,92.9% sensitivity,96.3% specificity,and an area under the curve of 0.990.Decision curve analysis confirmed its clinical utility.Based on the model,we developed user-friendly diagnostic software for clinical implementation.CONCLUSION Our machine learning approach differentiates CC from CBA in early infancy using routinely available clinical parameters.Early accurate diagnosis facilitates timely surgical intervention for CBA cases,potentially improving patient outcomes.展开更多
Objective: This study aimed to demonstrate the feasibility of laparoscopic-resection of the abdominal cystic lymphangiomas in Pediatric Surgery and describe the morbidity associated to this management in Queen fabiola...Objective: This study aimed to demonstrate the feasibility of laparoscopic-resection of the abdominal cystic lymphangiomas in Pediatric Surgery and describe the morbidity associated to this management in Queen fabiola children’s university hospital (HUDERF) in Brussels. Methods: We retrospectively conducted a study at the Pediatric Surgery Department of HUDERF, Brussels. The studied period was from January 1, 2014, to January 1, 2024;a span of 10 years. All patients with a confirmed diagnosis of cystic lymphangiomas and who underwent laparoscopic surgery were included in our study. Those who have been operated exclusively by open surgery have been excluded. The technique involved either total laparoscopic resection or laparoscopic-assisted with extra-abdominal resection of the tumor. Parameters that were studied included age, sex, weight, symptoms, preoperative diagnosis, imaging assessment, location, size of the tumor, type of mass, surgical procedure, duration of the surgery, conversion to open surgery, morbidity, and histopathology. Data were analyzed using Microsoft Office Excel 2010 and SPSS. Results: We retrieved 10 files of patients presenting with abdominal cystic lymphangiomas within two patients underwent exclusive open surgery and have excluded from our study. Then our sample was constituted with 8 patients. The mean age of the patients was 6.4 years (standard deviation: 3.6 years, range: 1 to 11 years). Male patients were predominant. The mean weight was 26.7 kg (standard deviation: 14.7 kg, range: 10 to 55 kg). The most common symptom was abdominal pain. Preoperative diagnosis of abdominal cystic lymphangioma was made in 8 cases. Abdominal ultrasound was performed in all patients. MRI was done in 5 patients, and CT scan in 2 patients. All patients presented a multicystic mass. Pure laparoscopic resection of the cyst was done in 2 cases. Laparoscopic-assisted resection in 4 cases (with extra-peritoneal with small bowel resection and mesenteric detorsion in 1 case), and conversion in 2 cases due to the complex location of the cyst. After a follow-up period of 5 years, morbidity was noted in one patient (Patient 3) who developed postoperative bowel obstruction 1 month post-surgery. This patient was re-operated on with a favorable clinical outcome following conventional small bowel resection and anastomosis. The other patients (1, 2, 4, 5, 6, 7 and 8) had a simple clinical course, and no recurrence was observed in our series. Conclusion: Laparoscopic-resection of the abdominal cystic lymphangiomas is feasible in Pediatric Surgery. As minimally invasive surgery it gives many advantages even for complex abdominal cystic lymphangiomas with less morbidity as shown in our series.展开更多
BACKGROUND Pancreatic cystic lesions are common in patients eligible for solid organ transplan-tation.It has been shown that the need for immunosuppression after organ transplantation increases the rate of malignancie...BACKGROUND Pancreatic cystic lesions are common in patients eligible for solid organ transplan-tation.It has been shown that the need for immunosuppression after organ transplantation increases the rate of malignancies in organ recipients.However,the impact of immunosuppression on pancreatic cystic lesions is yet unknown.AIM To evaluate the prevalence of pancreatic cystic lesions and the risk of cyst progression in immunosuppressed patients.METHODS A systematic literature search was performed in relevant databases.Studies reporting either on the prevalence and/or the incidence of pancreatic cyst progression compared to a control group were implemented in the first systematic review and meta-analysis on this topic.RESULTS The prevalence of pancreatic cystic lesions was comparable with 7%(95%CI:5%-11%)in the immunosuppressed cohort and 9%(95%CI:5%-16%)in the control cohort.The mean cyst size increase in the immunosuppression group was 3.2 mm(range 1.0-5.2mm)compared to 3.5 mm(1.0-6.9)in the control group(standar-dized mean difference 0.0 mm,95%CI:-0.3-0.2 mm,P=0.72).There was also no significant increase in the development of resection criteria or worrisome features under immunosuppression either[relative risk 1.1(fixed effect model),1.2(ran-dom effects model),P=0.61].CONCLUSION Immunosuppression does not increase the prevalence of pancreatic cystic lesions,nor does it increase the risk of cyst progression in terms of cyst size and development of resection criteria.Therefore,pancreatic cystic lesions in transplant candidates should not be a contraindication for solid organ transplantation.展开更多
BACKGROUND Adenoid cystic carcinoma(ACC)of the Bartholin’s gland represents an exceptionally rare malignancy with limited documented cases in the medical literature.It typically manifests as a solid mass that clinica...BACKGROUND Adenoid cystic carcinoma(ACC)of the Bartholin’s gland represents an exceptionally rare malignancy with limited documented cases in the medical literature.It typically manifests as a solid mass that clinically warrants suspicion for neoplastic processes.CASE SUMMARY This case series details two cases of primary ACC cases involving the Bartholin’s gland treated with radical surgical resection of the vaginal lesions.Notably,divergent therapeutic approaches resulted in contrasting prognoses:The patient receiving adjuvant radiotherapy following surgery maintained disease-free status with no locoregional recurrence or metastatic progression through 58 months of surveillance.Conversely,the non-radiated patient experienced disease recurrence within 18 months postoperatively.CONCLUSION Our findings suggest that postoperative radiation therapy may significantly decrease local recurrence rates in Bartholin’s gland ACC,potentially influencing long-term disease control.This comparative outcome analysis underscores the importance of integrating adjuvant radiotherapy integration into treatment protocols for this rare malignancy.展开更多
BACKGROUND The management of pediatric choledocholithiasis is complicated by anatomical constraints and the subsequent risks of conventional therapies requiring external drainage.This case report introduces a novel la...BACKGROUND The management of pediatric choledocholithiasis is complicated by anatomical constraints and the subsequent risks of conventional therapies requiring external drainage.This case report introduces a novel laparoscopic microincision tech-nique at the cystic duct confluence,designed to eliminate T-tube dependence,minimize ductal trauma,and expedite recovery,which are critical priorities for active children.We present this innovation to address unmet pediatric-specific surgical needs and demonstrate its feasibility as a tailored,minimally invasive solution for choledocholithiasis in children.A 12-year-old girl with a 5-year history of recurrent upper abdominal pain was diagnosed with choledocholithiasis,cholelithiasis,and biliary pancreatitis based on imaging and laboratory tests.After failed conservative management,laparo-scopic cholecystectomy with a microincision at the cystic duct confluence enabled choledochoscopic extraction of seven stones without T-tube placement.Primary closure using absorbable sutures with cystic duct confluence preserved biliary integrity.Postoperatively,liver function and amylase levels normalized by day 3,and abdominal ultrasonography confirmed no complications.The patient promptly resumed normal activity with no recurrence observed at the 16-month follow-up visit.This approach avoids external drainage,minimizes ductal manipulation,and optimizes recovery,which are key advantages for pediatric patients.CONCLUSION Microincision at the cystic duct confluence safely eliminates T-tubes,ensures stone clearance,and accelerates pediatric recovery.展开更多
Pancreatic cystic lesions are being increasingly detected,mainly due to the widespread use of cross-sectional imaging.The reported prevalence ranges from 13%to 18%in asymptomatic individuals.These lesions display a br...Pancreatic cystic lesions are being increasingly detected,mainly due to the widespread use of cross-sectional imaging.The reported prevalence ranges from 13%to 18%in asymptomatic individuals.These lesions display a broad histologic spectrum,from benign pseudocysts to premalignant mucinous cystic neoplasms and invasive carcinomas.Although many classification and management strategies exist,the natural history of numerous pancreatic cystic lesions remains incompletely understood,contributing to significant clinical uncertainty.Current diagnostic tools,including computed tomography,magnetic resonance imaging,endoscopic ultrasound,and cyst fluid analysis,are constrained by either suboptimal sensitivity or high costs.Cytology,while specific when positive,suffers from low sensitivity.Biochemical markers such as carcinoembryonic antigen,amylase,and glucose can help in cyst differentiation,whereas molecular testing(e.g.,KRAS,GNAS,RNF43 mutations)provides additional diagnostic and prognostic value.However,the application of molecular diagnostics is still restricted in routine practice due to costs,access issues,and a lack of standardization.This diagnostic uncertainty leads to both overtreatment and undertreatment.Some patients undergo unnecessary surgeries for benign lesions,which exposes them to procedural risks and long-term consequences.Others may experience delays in interventions for high-risk cysts and missing opportunities for cancer prevention.Additionally,prolonged,and often unnecessary surveillance burdens patients and healthcare systems psychologically and financially.In this minireview,we present a comprehensive overview of the classification,diagnostic approach,and management of pancreatic cystic lesions,incorporating recent evidence and current international guidelines(Fukuoka,American Gastroenterological Association,European).We also highlight the limitations of existing strategies and emerging tools such as radiomics,next-generation sequencing,and novel biomarkers.Additionally,we emphasize the urgent need for cost-effective,accurate,and accessible diagnostic pathways.A more refined risk stratification approach is essential to optimize outcomes,reduce healthcare waste,and improve the quality of life for patients with pancreatic cystic lesions.展开更多
BACKGROUND Eosinophilic solid and cystic(ESC)renal cell carcinoma(RCC),a unique and emerging subtype of RCC,has an indolent nature;in some rare instances,it may exhibit metastatic potential.Current cases are inadequat...BACKGROUND Eosinophilic solid and cystic(ESC)renal cell carcinoma(RCC),a unique and emerging subtype of RCC,has an indolent nature;in some rare instances,it may exhibit metastatic potential.Current cases are inadequate to precisely predict the clinical outcome of ESC RCC and determine treatment choices.CASE SUMMARY Herein,we report two patients with ESC RCC.Patient 1 was a young woman with classical pathological characteristics.Patient 2 was a 52-year-old man with multifocal metastases,involving the pulmonary hilar and mediastinal lymph nodes,liver,brain,mesosternum,vertebra,rib,femur,and symphysis pubis.Awareness of ESC RCC,along with its characteristic architecture and immunophenotype,would contribute to making a definitive diagnosis,even on core biopsy samples.CONCLUSION The discovery of ESC RCC molecular signatures may provide new therapeutic strategies in the future.展开更多
Pancreatic cystic neoplasms present a complex diagnostic scenario encompassing low-and high-grade malignancies.Their prevalence varies widely,notably increasing with age,reaching 75%in individuals older than 80 years....Pancreatic cystic neoplasms present a complex diagnostic scenario encompassing low-and high-grade malignancies.Their prevalence varies widely,notably increasing with age,reaching 75%in individuals older than 80 years.Accurate diagnosis is crucial,as errors occur in approximately one-third of resected cysts discovered incidentally.Various imaging modalities such as computed tomography,magnetic resonance imaging,and endoscopic techniques are available to address this challenge.However,risk stratification remains problematic,with guideline inconsistencies and diagnostic accuracy varying according to cyst type.This review proposed a stepwisemanagement approach,considering patient factors,imaging results,and specific features.This patient-centered model offers a structured framework for optimizing the care of individuals with pancreatic cystic neoplasms.展开更多
文摘Mucinous cystic neoplasms of the liver(MCN-L)are rare cystic lesions characterized by mucin-producing epithelium and ovarian-like stroma.Although they constitute fewer than 5%of hepatic cystic lesions,MCN-L poses significant diagnostic challenges due to overlapping features with other cystic lesions and their potential for malignant transformation.Early recognition and definitive surgical intervention are therefore critical to ensure optimal patient outcomes.A literature review was conducted to summarize epidemiology,clinical presentation,diagnostic modalities,and management strategies for MCN-L.Additionally,from 2019 to 2025,9 patients with MCN-L were identified at our center.Clinical data and outcomes were retrospectively analyzed.MCN-L predominantly affects middle-aged women and presents as large,multiloculated cystic lesions without biliary communication.The revised 2010 World Health Organization classification emphasizes the presence of ovarian-like stroma for definitive diagnosis.Contrast-enhanced computed tomography or magnetic resonance imaging are often suggestive but not pathognomonic,reinforcing the need for histopathological confirmation.MCN-L remains a diagnostic and therapeutic challenge due to its resemblance to other cystic liver lesions.Complete surgical resection is the treatment of choice to prevent recurrence and malignant transformation,reinforcing the importance of early intervention.Further research is needed to improve diagnostic accuracy and refine management strategies.
文摘BACKGROUND Acinar cystic transformation(ACT)of the pancreas is a rare non-neoplastic transformation of the pancreas.Adult women are the majority of patients with ACT,but few cases have been reported in pediatric patients.Given that there are currently no guidelines for the treatment of ACT,current treatment is based primarily on expert opinions and clinical experiences.Here,we report the case of the youngest child with ACT to date.Additionally,a literature review on pediatric ACT cases was performed to summarize previous clinical experience and treatment methods.CASE SUMMARY A 1-year-old Chinese girl presented with progressive abdominal distension for 6 months.A detailed consultation revealed an uneventful history.The patient showed no signs of fever or abdominal pain and had a good appetite and normal feces.A mass of about 20 cm×10 cm×10 cm in size was detected in the abdomen.Both abdominal ultrasound and computed tomography examination revealed a multilocular cystic mass about 21.7 cm×16.8 cm×8.9 cm in size.At first,due to the large size and the possible retroperitoneal origin of the cyst,a total resection of the lesion was not possible.A single-port laparoscopic lymphangioma puncture and Pingyangmycin injection were performed in March 2023.One month after surgery,the abdominal cyst rapidly enlarged to its pre-operative size.After consulting with the experts in the angiology department and interventional department,sclerotherapy combined with oral sirolimus was performed in May 2023.After confirming that the tumor was not sensitive to sclerotherapy combined with oral sirolimus,our surgical team performed tumor reduction in August 2023.This surgery confirmed that the polycystic mass originated from the head of the pancreas,and pathological and immunohistochemical findings diagnosed pancreatic ACT.The patient showed no signs of cyst lesions after 6 months of follow-up and remains in good health up to the time of this report.CONCLUSION ACT is a rare non-neoplastic transformation of the pancreas,more rarely seen in children.Manifestation and examinations show no specificity for diagnosis,and final diagnosis is mainly based on histological findings.To reach a specific diagnosis and rule out malignancy is a priority in clinical practice,and repeated biopsy or radical surgery should be considered before malignancy is ruled out.However,once a diagnosis of ACT is made,a conser-vative treatment with consecutive follow-up is recommended until symptoms present or obvious enlargement occurs because ACT is considered a slow-growing and benign tumor.
基金Supported by the National Key Research and Development Program of China,‘Clinical Performance Verification of Radial Ultrasound Endoscope’,No.2017YFC0109804the National High Level Hospital Clinical Research Funding,No.2022-PUMCH-A-076.
文摘BACKGROUND As a heterogeneous group of lesions,pancreatic cystic lesions(PCLs)vary enormously in malignant potential,mandating different treatment strategies.Despite significant advances in diagnostic imaging and laboratory tests,the accurate diagnosis of PCLs remains challenging,leading to overtreatment or delayed/missed surgical timing in patients with PCLs.CASE SUMMARY We present a case of a 64-year-old female patient in whom an asymptomatic,incidental cystic mass was found in the pancreatic tail on a routine abdominal ultrasound.After a comprehensive work-up with laboratory examinations,contrast-enhanced computed tomography,magnetic resonance imaging,and magnetic resonance cholangiopancreatography,a pancreatic pseudocyst was suspected.Subsequent endoscopic ultrasound with fine-needle aspiration and needle-based confocal laser endomicroscopy supported a benign diagnosis.Follow-up computed tomography and magnetic resonance imaging examinations five months later showed significant cyst shrinkage without any abnormalities.However,three years after being lost to follow-up,the patient was readmitted and diagnosed with pancreatic adenocarcinoma with multiple metastases,suggesting that the initial lesion was a mucinous cystic neoplasm misdiagnosed as a pan-creatic pseudocyst.CONCLUSION Comprehensive integration of all available information(e.g.,cyst features,abnormal imaging findings,cyst biochemistry,clinical history,and patient demographics)rather than over-reliance on imaging or endoscopic findings is pivotal to diagnosing PCLs,and patients with concerning features should undergo strict surveillance.
基金supported by the National Natural Science Foundation of China(grant No.82171588)the Fundamental Research Funds for the Central Institutes(grant No.2023GJZD01).
文摘Individuals with congenital absence of the vas deferens(CAVD)may transmit cystic fibrosis(CF)-causing variants of the cystic fibrosis transmembrane conductance regulator(CFTR)gene to their offspring through assisted reproductive technology(ART).We aimed to delineate the spectrum and estimate the prevalence of CF-causing variants in Chinese individuals with CAVD through a cohort analysis and meta-analysis.CFTR was sequenced in 145 Chineseindividuals with CAVD.CFTR variants were classified as CF-causing or non-CF-causing variants regarding clinical significance.A comprehensive genotype analysis was performed in Chinese individuals with CAVD,incorporating previous studies and our study cohort.The prevalence of CF-causing variants was estimated through meta-analysis.In our cohort,56 differentCFTR variants were identified in 108(74.5%)patients.Twenty variants were categorized as CF-causing and were detected in 28(19.3%)patients.A comprehensive genotype analysis of 867 patients identified 174 differentCFTR variants.Sixty-four were classified as CF-causing variants,56.3%of which had not been previously reported in Chinese patients with CF.Meta-analysis showed that 14.8%(95%confidence interval[CI]:11.0%-18.9%)CAVD cases harbored one CF-causing variant,and 68.6%(95%CI:65.1%-72.0%)CAVD cases carried at least one CFTR variant.Our study underscores the urgent need for extensiveCFTR screening,including sequencing of whole exons and flanking regions and detection of large rearrangements and deep intronic CF-causing variants,in Chinese individuals with CAVDbefore undergoing ART.The established CF-causing variants spectrum may aid in the development of genetic counseling strategies and preimplantation diagnosis to prevent the birth of a child with CF.
文摘BACKGROUND Malignant transformation(MT)of mature cystic teratoma(MCT)has a poor prognosis,especially in advanced cases.Concurrent chemoradiotherapy(CCRT)has an inhibitory effect on MT.CASE SUMMARY Herein,we present a case in which CCRT had a reduction effect preoperatively.A 73-year-old woman with pyelonephritis was referred to our hospital.Computed tomography revealed right hydronephrosis and a 6-cm pelvic mass.Endoscopic ultrasound-guided fine-needle biopsy(EUS-FNB)revealed squamous cell carci-noma.The patient was diagnosed with MT of MCT.Due to her poor general con-dition and renal malfunction,we selected CCRT,expecting fewer adverse effects.After CCRT,her performance status improved,and the tumor size was reduced;surgery was performed.Five months postoperatively,the patient developed dis-semination and lymph node metastases.Palliative chemotherapy was ineffective.She died 18 months after treatment initiation.CONCLUSION EUS-FNB was useful in the diagnosis of MT of MCT;CCRT suppressed the disea-se and improved quality of life.
基金supported by the Special Project for the Construction of Innovative Provinces in Hunan Province,China(2020SK2013)。
文摘Objective:In recent years,the incidence and detection rate of pancreatic cystic lesions(PCLs)have increased significantly.Endoscopic ultrasound(EUS)plays an indispensable role in the diagnosis and differential diagnosis of PCLs.However,evidence comparing the diagnostic performance of EUS-guided fine-needle aspiration(EUS-FNA)and fine-needle biopsy(FNB)remains limited.This study aims to compare the diagnostic yield,adequacy of tissue acquisition,and safety between EUS-FNA and EUS-FNB in evaluating PCLs to inform clinical practice.Methods:A retrospective review was conducted on patients with PCLs who underwent either EUS-FNA or EUS-FNB between January 2014 and August 2021.The diagnostic yield,tissue acquisition adequacy,and incidence of adverse events were compared between the 2 groups.Results:A total of 90 patients with PCLs were included(52 in the FNA group and 38 in the FNB group).The diagnostic yield was similar between the FNA and FNB groups(94.2%vs 94.7%,P>0.05).The adequacy of tissue acquisition was 71.2%in the FNA group and 81.6%in the FNB group(P>0.05).No statistically significant difference was observed in the incidence of adverse events between the 2 groups(P>0.05).Conclusion:Both EUS-FNA and EUS-FNB demonstrate equally high diagnostic yields and tissue adequacy in PCLs,with excellent safety profiles.Both methods are safe and effective diagnostic tools for evaluating PCLs.
文摘BACKGROUND Neonatal screening(NS)is a public health policy to identify genetic pathologies such as cystic fibrosis(CF),sickle cell disease,and other diseases.Sickle cell disea-se is the comprehensive term for a group of hemoglobinopathies characterized by the presence of hemoglobin S.CF is an autosomal recessive multisystemic disease with pathophysiology involving deleterious mutations in the transmembrane re-gulatory gene that encodes a protein that regulates the activity of chloride and sodium channels in the cell surface epithelium.NS is crucial for early diagnosis and management,which ensures a better quality of life.AIM To report a case of the coexistence of sickle cell anemia(SCA)and CF and perform an integrative literature review.METHODS This is an observational study and a review of the literature focusing on two rare genetic pathologies identified simultaneously in NS from the perspective of a clinical case.The authors identified only 5 cases of SCA associated with CF.No clinical trials or review articles were identified considering the rarity of the coexistence of these two pathologies.RESULTS Herein,the authors reported the case of a girl who after undergoing NS on day 8 of life was diagnosed with SCA with an alteration in the dosage of immunoreactive trypsin.The diagnosis of CF was confirmed by the Coulometry Sweat Test.The rarity of the co-occurrence of these two severe genetic pathologies(CF and SCA)is a challenge for medical science.CONCLUSION This study adds to the few case reports present in the literature that highlight the identification of two severe diseases via NS.
基金Supported by the National Natural Science Foundation of China,No.82360111Xinjiang Science and Technology Department-Leading Talents in Technological Innovation-High-Level Leading Talents,No.2022TSYCLJ0034+1 种基金State Key Laboratory for The Cause and Control of High Incidence in Central Asia Jointly Constructed by The Ministry and The Province,No.SKL-HIDCA-2023-2 and No.SKLHIDCA-2024-22Xinjiang Uygur Autonomous Region Graduate Innovation Program,No.XJ2024G153.
文摘BACKGROUND Alveolar and cystic echinococcoses are lethal zoonotic diseases caused by Echinococcus multilocularis and Echinococcus granulosus infections,leading to alveolar echinococcosis(AE)or cystic echinococcosis(CE),respectively.No study has hitherto reported effective treatment approaches for AE or CE with concurrent hepatorenal involvement.AIM To investigate the feasibility and efficacy of simultaneous combined surgery(SCS)as a comprehensive treatment approach for patients with hepatorenal echinococcosis.METHODS Clinical datasets of hepatorenal AE(n=10)and CE(n=11)patients were retrospectively collected and systematically analyzed.The SCS approach was introduced,and surgical outcomes,complications,and prognoses were documented in detail.RESULTS The SCS approach incorporated hybridized techniques,including partial hepatectomy,partial or total nephrectomy,ex vivo liver resection and autotransplantation,and total or subtotal cystectomy with endocystectomy.Radical SCS was achieved in 100%of AE patients and 63.6%of CE patients.All surgeries were completed without intraoperative complications.The short-term complication rate was 28.6%(Clavien-Dindo classification:AE-1 IIIb,3 IIIa;CE-2 II),while the long-term complication rate was 4.8%(Clavien-Dindo classification:AE-1 IIIb).Patients were followed up for a median of 37 months(AE:6-81 months;CE:34-123 months),with no reported deaths or disease relapses.CONCLUSION CS appears to be a feasible and effective treatment method for patients with hepatorenal involvement of AE or CE.It fulfills the management criteria for advanced AE or CE cases,aiming to maximize patient benefits.
基金National Natural Science Foundation of China,No.82200686Shaanxi Provincial People's Hospital Incubation Project,No.2022YJY-14.
文摘BACKGROUND Acute cholecystitis due to unintended cystic artery embolism is an uncommon and mostly self-limiting complication after transarterial chemoembolization procedure for treatment of hepatocellular carcinoma.Usually,conservative management is sufficient for complete recovery of patients who develop this complication.If conservative treatment is ineffective,urgent surgical inter-vention may be required to prevent the progression of complications.CASE SUMMARY This article reports a rare and serious case of acute cholecystitis complicated by gallbladder necrosis and biliary peritonitis,which was initially treated conservatively but eventually necessitated emergency laparotomy.The patient initially presented with equivocal symptoms of fever and upper abdominal pain and distention,which worsened at the two weeks mark along with emergence of signs of peritonitis.This was managed by emergency laparotomy and cholecystostomy,allowing rapid symptom relief.The patient ultimately discharged and succumbed to advanced liver cancer 11 months after diagnosis.CONCLUSION After cholecystostomy,the patient showed symptom relief and was discharged,surviving 11 months post-stage IIIB liver cancer diagnosis.
文摘BACKGROUND Leiomyomas or fibroids commonly originate from the uterus;extrauterine leiomyomas are rare and most often arise from the broad ligament.Diagnosing broad ligament leiomyomas becomes particularly challenging when they undergo degenerative changes because their clinical and radiological features often mimic those of ovarian tumors.We report a rare case of a giant broad ligament fibroid with cystic degeneration,which was initially mistaken for an ovarian mass.CASE SUMMARY A 49-year-old woman presented with mild abdominal distension and pain as the only symptoms.Upon abdominal examination,a large mass measuring approximately 30 cm and extending from the pelvic cavity to just below the xiphoid process was identified.Both transvaginal ultrasound and contrast-enhanced computed tomography suggested an ovarian origin of the mass.However,laparotomy confirmed that the mass originated from the right broad ligament.The mass was separated from the uterus and bilateral ovaries,with no involvement of the uterus or ovaries.The mass was completely resected with respecting the patient’s desire to retain her uterus and adnexa.Postoperative histopathological examination confirmed leiomyoma with cystic degeneration.CONCLUSION Broad ligament myomas mimic ovarian tumors;accurate diagnosis and careful operation are critical to avoid complications and ensure safety.
文摘BACKGROUND Cystic lymphangioma is a rare hamartoma that is especially found in the adult gastrointestinal tract.In the early stage,most patients are asymptomatic;after the onset of symptoms,there is often no specificity regarding symptoms.CASE SUMMARY Here we report the endoscopic diagnosis and treatment of an adult patient with cystic lymphangioma of the ascending colon.One patient who came to our hospital with“dull pain in the left lower abdomen for 2 days”was initially misdiagnosed with a colon cyst according to endoscopy and then underwent endoscopic submucosal dissection.The final pathological results suggested cystic lymphangioma.One year later,no recurrence was found on re-examination via colonoscopy.CONCLUSION Cystic lymphangioma in the gastrointestinal tract rarely occurs in adults and is easily misdiagnosed or missed.Endoscopy,imaging,histology,and immunohistochemical staining are useful for diagnosis.Surgical resection is the preferred treatment.
基金Supported by National Key R&D Program of China(No.2023YFC2410203)Beijing Hospitals Authority Clinical medicine Development of Special Funding Support(No.ZLRK202503).
文摘Adenoid cystic carcinoma of the lacrimal gland(LGACC)is the most common type of malignant epithelial tumor of the lacrimal gland,which is characterized by a high recurrence rate,perineural invasion,and a propensity to metastasize to distant sites.Due to its unclear pathogenesis,LGACC has a poor prognosis and a high mortality rate.In recent years,a range of radiotherapy and chemotherapy have been clinically applied,leading to a shift in the treatment approach for LGACC.This article discussed the advances being made in the treatment of LGACC and provides readers with an overview of the impact of LGACC treatment modalities on patient survival and prognostic levels.
基金Supported by the Beijing Municipal Science and Technology Commission,No.Z191100006619002Haiyou Health High-Caliber Talent Project,No.202412the Research Unit of Minimally Invasive Pediatric Surgery on Diagnosis and Treatment,Chinese Academy of Medical Sciences,No.2021RU015.
文摘BACKGROUND Choledochal cysts(CC)and cystic biliary atresia(CBA)present similarly in early infancy but require different treatment approaches.While CC surgery can be delayed until 3-6 months of age in asymptomatic patients,CBA requires intervention within 60 days to prevent cirrhosis.AIM To develop a diagnostic model for early differentiation between these conditions.METHODS A total of 319 patients with hepatic hilar cysts(<60 days old at surgery)were retrospectively analyzed;these patients were treated at three hospitals between 2011 and 2022.Clinical features including biochemical markers and ultrasonographic measurements were compared between CC(n=274)and CBA(n=45)groups.Least absolute shrinkage and selection operator regression identified key diagnostic features,and 11 machine learning models were developed and compared.RESULTS The CBA group showed higher levels of total bile acid,total bilirubin,γ-glutamyl transferase,aspartate aminotransferase,and alanine aminotransferase,and direct bilirubin,while longitudinal diameter of the cysts and transverse diameter of the cysts were larger in the CC group.The multilayer perceptron model demonstrated optimal performance with 95.8% accuracy,92.9% sensitivity,96.3% specificity,and an area under the curve of 0.990.Decision curve analysis confirmed its clinical utility.Based on the model,we developed user-friendly diagnostic software for clinical implementation.CONCLUSION Our machine learning approach differentiates CC from CBA in early infancy using routinely available clinical parameters.Early accurate diagnosis facilitates timely surgical intervention for CBA cases,potentially improving patient outcomes.
文摘Objective: This study aimed to demonstrate the feasibility of laparoscopic-resection of the abdominal cystic lymphangiomas in Pediatric Surgery and describe the morbidity associated to this management in Queen fabiola children’s university hospital (HUDERF) in Brussels. Methods: We retrospectively conducted a study at the Pediatric Surgery Department of HUDERF, Brussels. The studied period was from January 1, 2014, to January 1, 2024;a span of 10 years. All patients with a confirmed diagnosis of cystic lymphangiomas and who underwent laparoscopic surgery were included in our study. Those who have been operated exclusively by open surgery have been excluded. The technique involved either total laparoscopic resection or laparoscopic-assisted with extra-abdominal resection of the tumor. Parameters that were studied included age, sex, weight, symptoms, preoperative diagnosis, imaging assessment, location, size of the tumor, type of mass, surgical procedure, duration of the surgery, conversion to open surgery, morbidity, and histopathology. Data were analyzed using Microsoft Office Excel 2010 and SPSS. Results: We retrieved 10 files of patients presenting with abdominal cystic lymphangiomas within two patients underwent exclusive open surgery and have excluded from our study. Then our sample was constituted with 8 patients. The mean age of the patients was 6.4 years (standard deviation: 3.6 years, range: 1 to 11 years). Male patients were predominant. The mean weight was 26.7 kg (standard deviation: 14.7 kg, range: 10 to 55 kg). The most common symptom was abdominal pain. Preoperative diagnosis of abdominal cystic lymphangioma was made in 8 cases. Abdominal ultrasound was performed in all patients. MRI was done in 5 patients, and CT scan in 2 patients. All patients presented a multicystic mass. Pure laparoscopic resection of the cyst was done in 2 cases. Laparoscopic-assisted resection in 4 cases (with extra-peritoneal with small bowel resection and mesenteric detorsion in 1 case), and conversion in 2 cases due to the complex location of the cyst. After a follow-up period of 5 years, morbidity was noted in one patient (Patient 3) who developed postoperative bowel obstruction 1 month post-surgery. This patient was re-operated on with a favorable clinical outcome following conventional small bowel resection and anastomosis. The other patients (1, 2, 4, 5, 6, 7 and 8) had a simple clinical course, and no recurrence was observed in our series. Conclusion: Laparoscopic-resection of the abdominal cystic lymphangiomas is feasible in Pediatric Surgery. As minimally invasive surgery it gives many advantages even for complex abdominal cystic lymphangiomas with less morbidity as shown in our series.
文摘BACKGROUND Pancreatic cystic lesions are common in patients eligible for solid organ transplan-tation.It has been shown that the need for immunosuppression after organ transplantation increases the rate of malignancies in organ recipients.However,the impact of immunosuppression on pancreatic cystic lesions is yet unknown.AIM To evaluate the prevalence of pancreatic cystic lesions and the risk of cyst progression in immunosuppressed patients.METHODS A systematic literature search was performed in relevant databases.Studies reporting either on the prevalence and/or the incidence of pancreatic cyst progression compared to a control group were implemented in the first systematic review and meta-analysis on this topic.RESULTS The prevalence of pancreatic cystic lesions was comparable with 7%(95%CI:5%-11%)in the immunosuppressed cohort and 9%(95%CI:5%-16%)in the control cohort.The mean cyst size increase in the immunosuppression group was 3.2 mm(range 1.0-5.2mm)compared to 3.5 mm(1.0-6.9)in the control group(standar-dized mean difference 0.0 mm,95%CI:-0.3-0.2 mm,P=0.72).There was also no significant increase in the development of resection criteria or worrisome features under immunosuppression either[relative risk 1.1(fixed effect model),1.2(ran-dom effects model),P=0.61].CONCLUSION Immunosuppression does not increase the prevalence of pancreatic cystic lesions,nor does it increase the risk of cyst progression in terms of cyst size and development of resection criteria.Therefore,pancreatic cystic lesions in transplant candidates should not be a contraindication for solid organ transplantation.
文摘BACKGROUND Adenoid cystic carcinoma(ACC)of the Bartholin’s gland represents an exceptionally rare malignancy with limited documented cases in the medical literature.It typically manifests as a solid mass that clinically warrants suspicion for neoplastic processes.CASE SUMMARY This case series details two cases of primary ACC cases involving the Bartholin’s gland treated with radical surgical resection of the vaginal lesions.Notably,divergent therapeutic approaches resulted in contrasting prognoses:The patient receiving adjuvant radiotherapy following surgery maintained disease-free status with no locoregional recurrence or metastatic progression through 58 months of surveillance.Conversely,the non-radiated patient experienced disease recurrence within 18 months postoperatively.CONCLUSION Our findings suggest that postoperative radiation therapy may significantly decrease local recurrence rates in Bartholin’s gland ACC,potentially influencing long-term disease control.This comparative outcome analysis underscores the importance of integrating adjuvant radiotherapy integration into treatment protocols for this rare malignancy.
文摘BACKGROUND The management of pediatric choledocholithiasis is complicated by anatomical constraints and the subsequent risks of conventional therapies requiring external drainage.This case report introduces a novel laparoscopic microincision tech-nique at the cystic duct confluence,designed to eliminate T-tube dependence,minimize ductal trauma,and expedite recovery,which are critical priorities for active children.We present this innovation to address unmet pediatric-specific surgical needs and demonstrate its feasibility as a tailored,minimally invasive solution for choledocholithiasis in children.A 12-year-old girl with a 5-year history of recurrent upper abdominal pain was diagnosed with choledocholithiasis,cholelithiasis,and biliary pancreatitis based on imaging and laboratory tests.After failed conservative management,laparo-scopic cholecystectomy with a microincision at the cystic duct confluence enabled choledochoscopic extraction of seven stones without T-tube placement.Primary closure using absorbable sutures with cystic duct confluence preserved biliary integrity.Postoperatively,liver function and amylase levels normalized by day 3,and abdominal ultrasonography confirmed no complications.The patient promptly resumed normal activity with no recurrence observed at the 16-month follow-up visit.This approach avoids external drainage,minimizes ductal manipulation,and optimizes recovery,which are key advantages for pediatric patients.CONCLUSION Microincision at the cystic duct confluence safely eliminates T-tubes,ensures stone clearance,and accelerates pediatric recovery.
文摘Pancreatic cystic lesions are being increasingly detected,mainly due to the widespread use of cross-sectional imaging.The reported prevalence ranges from 13%to 18%in asymptomatic individuals.These lesions display a broad histologic spectrum,from benign pseudocysts to premalignant mucinous cystic neoplasms and invasive carcinomas.Although many classification and management strategies exist,the natural history of numerous pancreatic cystic lesions remains incompletely understood,contributing to significant clinical uncertainty.Current diagnostic tools,including computed tomography,magnetic resonance imaging,endoscopic ultrasound,and cyst fluid analysis,are constrained by either suboptimal sensitivity or high costs.Cytology,while specific when positive,suffers from low sensitivity.Biochemical markers such as carcinoembryonic antigen,amylase,and glucose can help in cyst differentiation,whereas molecular testing(e.g.,KRAS,GNAS,RNF43 mutations)provides additional diagnostic and prognostic value.However,the application of molecular diagnostics is still restricted in routine practice due to costs,access issues,and a lack of standardization.This diagnostic uncertainty leads to both overtreatment and undertreatment.Some patients undergo unnecessary surgeries for benign lesions,which exposes them to procedural risks and long-term consequences.Others may experience delays in interventions for high-risk cysts and missing opportunities for cancer prevention.Additionally,prolonged,and often unnecessary surveillance burdens patients and healthcare systems psychologically and financially.In this minireview,we present a comprehensive overview of the classification,diagnostic approach,and management of pancreatic cystic lesions,incorporating recent evidence and current international guidelines(Fukuoka,American Gastroenterological Association,European).We also highlight the limitations of existing strategies and emerging tools such as radiomics,next-generation sequencing,and novel biomarkers.Additionally,we emphasize the urgent need for cost-effective,accurate,and accessible diagnostic pathways.A more refined risk stratification approach is essential to optimize outcomes,reduce healthcare waste,and improve the quality of life for patients with pancreatic cystic lesions.
文摘BACKGROUND Eosinophilic solid and cystic(ESC)renal cell carcinoma(RCC),a unique and emerging subtype of RCC,has an indolent nature;in some rare instances,it may exhibit metastatic potential.Current cases are inadequate to precisely predict the clinical outcome of ESC RCC and determine treatment choices.CASE SUMMARY Herein,we report two patients with ESC RCC.Patient 1 was a young woman with classical pathological characteristics.Patient 2 was a 52-year-old man with multifocal metastases,involving the pulmonary hilar and mediastinal lymph nodes,liver,brain,mesosternum,vertebra,rib,femur,and symphysis pubis.Awareness of ESC RCC,along with its characteristic architecture and immunophenotype,would contribute to making a definitive diagnosis,even on core biopsy samples.CONCLUSION The discovery of ESC RCC molecular signatures may provide new therapeutic strategies in the future.
文摘Pancreatic cystic neoplasms present a complex diagnostic scenario encompassing low-and high-grade malignancies.Their prevalence varies widely,notably increasing with age,reaching 75%in individuals older than 80 years.Accurate diagnosis is crucial,as errors occur in approximately one-third of resected cysts discovered incidentally.Various imaging modalities such as computed tomography,magnetic resonance imaging,and endoscopic techniques are available to address this challenge.However,risk stratification remains problematic,with guideline inconsistencies and diagnostic accuracy varying according to cyst type.This review proposed a stepwisemanagement approach,considering patient factors,imaging results,and specific features.This patient-centered model offers a structured framework for optimizing the care of individuals with pancreatic cystic neoplasms.
