简介：AbstractCentral nervous system (CNS) tumors represent the most deadly cancer in pediatric age group. In China, thousands of children are diagnosed with CNS tumors every year. Despite the improving socioeconomic status and availability of medical expertise within the country, unique challenges remain for the delivery of pediatric neuro-oncology service. In this review, we discuss the existing hurdles for improving the outcome of children with CNS tumors in China. Need for precise disease burden estimation, lack of intra- and inter-hospital collaborative networks, high probability of treatment abandonment, along with financial toxicities from treatment represent the key challenges that Chinese healthcare providers encounter. The tremendous opportunities for advancing the status of pediatric neuro-oncology care in and beyond the country are explored.

简介：BACKGROUND&OBJECTIVE:Themajorityofintramedullaryspinalcordtumors(IMSCT)arelow-gradegliomas.RadicalresectionforIMSCTsremainschallenging.Recently,improvedneuroimagingandadvancedmicrosurgicaltechniquehavemadegreatsuccessinsurgicalmanagementoftheintramedullaryspinalcordtumors.METH-ODS&RESULTS:Twenty-ninepatientswithintramedullaryspinalcordtumorsweretreatedbyradicalresectiondur-ingthepast4yearsinourinstitute.Thehistologicalresultswereasfollows:12ependymomas,4astrocytomas,4heman-gioblastomas,4epidermoids,1cavernoma,2lipomas,2metastatics.Agross-totalresection(>95%)wasachievedin25surgicalprocedures.Subtotalresections(80-95%)wereperformedin4cases.Therewasnosurgicaldeath.Whencomparingthepreoperativeand3-monthpostoperativefunctionalgrades,12patientswerestable14improved,and3deteriorated.Patientswitheithernodeficitoronlymilddeficitbeforesurgerywererarelyimpairedbytheproce-dure,reinforcingtheimportanceofearlydiagnosisandtreatment.Themajordeterminantoflong-termsurvivalwashistologi-calcompositionofthetumor.PatientsinwhomanIMSCTwasonlypartiallyresected(<80%)faredsignificantlyworse.CONCLUSIONS:Thelong-termsurvivalandqualityoflifeforpatientswithlow-gradegliomastreatedbyradi-calresectionaloneiscomparableorsuperiortominimalresectionplusradiotherapy.Theoptimaltherapyforpatientswithhigh-gradegliomaisyettobedetermined.Forbenignlesion,suchashemangioblastomaandcavernomacouldbecuredbytotalresectionofthetumor.Forlipomaandepidermoid,fibrousadhesionstothecordmaketotalremovaldifficult,andthus,removalisnotthegoalofsurgery.Thecarbondioxidelaserisparticularlyusefulduringsurgeryforthislesion.

简介：ＡＦＡＭＩＬＩＡＬ４６ＸＹＧＯＮＡＤＡＬＤＹＳＧＥＮＥＳＩＳＡＮＤＨＩＧＨＩＮＣＩＤＥＮＣＥＯＦＥＭＢＲＹＯＮＩＣＧＯＮＡＤＡＬＴＵＭＯＲＳＥｈＺｈｅｎｇ；鄂征；ＬｉｕＷｅｉｌｉ；刘伟莉（ＤｅｐａｒｔｍｅｎｔｏｆＣｙｔｏｇｅｎｅｔｉｃｓ，Ｂｅｉｊｉｎ...

简介：Objective:Toevaluatetheshort-termoutcomesofvideo-assistedthoracicsurgery(VATS)forthoracictumors.Methods:Thedataof1,790consecutivepatientswereretrospectivelyreviewed.ThesepatientsunderwentVATSpulmonaryresections,VATSesophagectomies,andVATSresectionsofmediastinaltumorsorbiopsiesattheCancerInstitute&Hospital,ChineseAcademyofMedicalSciencesbetweenJanuary2009andJanuary2012.Results:Therewere33patientsconvertedtoopenthoracotomy(OT,1.84%).Theoverallmorbidityandmortalityratewas2.79%(50/1790)and0.28%(5/1790),respectively.TheoverallhospitalizationandchesttubedurationwereshorterintheVATSlobectomygroup(n=949)thanintheopenthoracotomy(OT)lobectomygroup(n=753).Therewerenosignificantdifferencesinmorbidityrate,mortalityrateandoperationtimebetweenthetwogroups.Intheesophagealcancerpatients,nosignificantdifferencewasfoundinthenumberofnodaldissection,chesttubeduration,morbidityrate,mortalityrate,andhospitallengthofstaybetweentheVATSesophagectomygroup(n=81)andopenesophagectomygroup(n=81).However,theoperationtimewaslongerintheVATSesophagectomygroup.Inthethymomapatients,therewasnosignificantdifferenceinthechesttubeduration,morbidityrate,mortalityrate,andhospitallengthofstaybetweentheVATSthymectomygroup(n=41)andopenthymectomygroup(n=41).However,theoperationtimewaslongerintheVATSgroup.ThemediantumorsizeintheVATSthymectomygroupwascomparablewiththatintheOTgroup.Conclusions:Inearly-stage(I/II)non-smallcelllungcancerpatientswhounderwentlobectomies,VATSiscomparablewiththeOTapproachwithsimilarshort-termoutcomes.Inpatientswithresectableesophagealcancer,VATSesophagectomyiscomparablewithOTesophagectomywithsimilarmorbidityandmortality.VATSthymectomyforMasaokastageIandIIthymomaisfeasibleandsafe,andtumorsizeisnotcontraindicated.Longerfollow-upsareneededtodete

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简介：AIM:Toevaluatetheclinicalusefulnessofendoscopicultrasonography(EUS)forthediagnosisoftheinvasiondepthofulcerativecolitis-associatedtumors.METHODS:Thestudygroupcomprised13patientswith16ulcerativecolitis(UC)-associatedtumorsforwhichthedepthofinvasionwaspreoperativelyestimatedbyEUS.Thelesionswerethenresectedendoscopicallyorbysurgicalcolectomyandwereexaminedhistopathologically.Themeanageofthesubjectswas48.2±17.1years,andthemeandurationofUCwas15.8±8.3years.Twolesionsweretreatedbyendoscopicresectionandtheother14lesionsbysurgicalcolectomy.ThedepthofinvasionofUCassociatedtumorswasestimatedbyEUSusinganultrasonicprobeandwasevaluatedonthebasisofthedeepestlayerwithnarrowingorruptureofthecolonicwall.RESULTS:ThediagnosisofUC-associatedtumorsbyEUSwascarcinomafor13lesionsanddysplasiafor3lesions.Theinvasiondepthofthecarcinomaswasintramucosalfor8lesions,submucosalfor2,themuscularispropriafor2,andsubserosalfor1.Eleven(69%)ofthe16lesionsaroseintherectum.Themacroscopicappearancewasthelaterallyspreadingtumor-non-granulartypefor4lesions,sessiletypefor4,laterallyspreadingtumor-granulartypefor3,semipedunculatedtype(Isp)for2,type1for2,andtype3for1.ThedepthofinvasionwascorrectlyestimatedbyEUSfor15lesions(94%)butwasmisdiagnosedasintramucosalfor1carcinomawithhigh-gradesubmucosalinvasion.The2lesionstreatedbyendoscopicresectionwereintramucosalcarcinomaanddysplasia,andbothwerediagnosedasintramucosallesionsbyEUS.CONCLUSION:EUSprovidesagoodestimationoftheinvasiondepthofUC-associatedtumorsandmaythusfacilitatetheselectionoftreatment.

简介：Objective:Toanalyzetheinfluencingfactorsforradiation-inducedheartdisease(RIHD)inapanelofcaseswithmalignantthymictumorstreatedbyradiotherapy.Methods:52consecutivepatientsweretreatedbyradiotherapyformalignantthymictumor(14atMasaokastageII,23atstageIIIand15atstageIV).Treatmentincludedradical(in20),postoperative(in14),preoperative(in2)andpalliative(in16)radiotherapy.Theconventionaltwo-dimension(2D)radiationtherapywasperformedinforty-sevenpatientsandthree-dimension(3D)conformalradiationtherapyhasbeenusedin5patientssinceOctober2000.Thetotaltumordoserangedfrom10Gyto84.5Gy(medianof55Gy).Chemotherapywasgivenintwenty-fivepatientsbeforeorafterradiotherapy.Theresultsoffollowing-upcouldbeobtainedfromthedatabaseandupdatedwhereappropriated.Thedosevolumehistogram(DVH)ofheartinradiotherapyforallpatientswasanalyzedfortheeffectivevolumedoseofheart.Result:Themedianfollowing-upwas14months(rangedfrom0.6to111.3months)inthestudy.RIHDwasobservedinsevenpatients.CardiactoxicityofthesesevenpatientswereevaluatedasSOMAgrade1-3.Themediantwo-thirdeffectivevolumedoseofheartwas47.2Gy(rangedfrom8.3Gyto70.1Gy)forconventional2Dradiotherapy,whichcorrelatedwiththymictumordose(P<0.0001).Themediantwo-thirdeffectivevolumedoseofheartwas35.3Gy(rangedfrom13Gyto38.7Gy)for3Dconformalradiotherapy.Theeffectivevolumedosesofheartweredecreasedbyusing3Dconformalradiotherapy(P=0.048).Asignificantassociationbetweencardiactoxicityandeffectivevolumedoseofheartwasfoundinthisstudy(P<0.0001).Cardiactoxicityaccountedfor10.4%and4.1%ofpatientsreceivingandnotreceivingadjuvantchemotherapy,respectively,andoccurredearlierinradiochemotherapygroup(P=0.0528).Multivariateanalysissuggestedthatcardiactoxicitywassignificantlyinfluencedbytheeffectivevolumedoseofheartandchemotherapy.C

简介：AbstractGastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasm of the digestive system and are not sensitive to traditional chemotherapy. Therefore, historically, surgical resection was the only effective therapy. However, the emergence of tyrosine kinase inhibitors (TKIs) has revolutionized the treatment of GISTs, because they target c-Kit and PDGF receptor-α (PDGFRA), which are important in GIST development and progression. As research into c-Kit and PDGFRA continues, an increasing number of different TKIs are being used in the clinical setting. This review aims to discuss the current state of chemotherapy for the treatment of GISTs with different genotypes.

简介：AbstractThere has been a rising trend in the incidence and prevalence of non-functioning pancreatic neuroendocrine tumors (NFPanNETs). While a significant number of the newly diagnosed NFPanNETs are asymptomatic, a majority of patients will present with liver metastasis (LM) at the time of diagnosis. Surgical resection remains the only curative treatment, especially for localized NFPanNETs. While a majority of small NFPanNETs are indolent, some are not. This heterogeneity in tumor biology presents the surgeon with the unique challenge of determining which patient will benefit from surgery, given the morbidity of pancreatic surgery. There has been a recent push for a more aggressive approach to the care of these patients, given the emergence of data supporting such measures. However, the risk of over or under treatment has generated immense debate amongst experts in the field. The heterogeneity of current practice guidelines and institutional practices around the world is a reflection of the disparate opinion on the management of NFPanNET. In this review, we set out to examine the evidence regarding some of the most controversial and challenging aspects of the surgical treatment of NFPanNET. We evaluate the following questions; should patients with small NFPanNETs ≤ 2 cm in size be resected; should patients with metastatic NFPanNETs undergo surgical debulking, and should there be resection of the primary tumor in the setting of non-resectable metastatic disease?Although there are currently no Level 1 data to answer these questions conclusively, we believe that the current literature supports a more aggressive approach to the management of NFPanNET.

简介：AbstractBackground:Despite the recent large number of studies comparing endoscopic and laparoscopic resection for small gastrointestinal stromal tumors (GISTs) (diameter ≤5 cm), the results remain conflicting. The objective of this work was to perform a cumulative meta-analysis to assess the advantages and disadvantages of endoscopic resection vs. laparoscopic resection.Methods:The meta-analysis followed the Preferred Reporting Items for Systematic Reviews and Meta-Analyses statement. We searched medical databases up to January 2020. Meta-analytical random or fixed effects models were used in pooled analyses. Meta-regression, cumulative meta-analyses, and sub-group analyses were performed to improve the accuracy of the conclusion. Sensitivity analyses were applied to assess the robustness of the results.Results:A total of 12 cohort studies with 1383 participants comparing endoscopic resection and laparoscopic resection were identified, while three cohort studies with 167 participants comparing endoscopic resection and laparoscopic and endoscopic cooperative surgery were found. We found that endoscopic resection had shorter operation times (weighted mean difference [WMD]= -27.1 min, 95% confidence interval [CI]: -40.8 to -13.4 min) and lengths of hospital stay (WMD = -1.43 days, 95% CI: -2.31 to -0.56 days) than did laparoscopic resection. The results were stable and reliable. There were no significant differences in terms of blood loss, hospitalization costs, incidence of complications or recurrence rates. For tumor sizes 2 to 5 cm, endoscopic resection increased the risk of positive margins (relative risk [RR] = 5.78, 95% CI: 1.31-25.46). Although operation times for endoscopic resection were shorter than those of laparoscopic and endoscopic cooperative surgery (WMD = -41.03 min, 95% CI: -59.53 to -22.54 min), there was a higher incidence of complications (RR = 4.03, 95% CI: 1.57-10.34).Conclusions:In general, endoscopic resection is an alternative method for gastric GISTs ≤5 cm. Laparoscopic and endoscopic cooperative surgery may work well in combination. Further randomized controlled trials are recommended to validate or update these results.

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简介：AbstractObjective:This study was performed to evaluate the clinical and epidemiological characteristics of inpatients with malignant skin tumors in western Inner Mongolia during the past 10 years.Methods:We collected the clinical data of inpatients with histopathologically diagnosed malignant skin tumors admitted to the Affiliated Hospital of Inner Mongolia Medical University from June 2008 to December 2018. Morbidity was compared using the Chi-square test.Results:In total, 340 inpatients with malignant skin tumors were evaluated, including 163 (47.94%) patients with basal cell carcinoma, 134 (39.41%) with squamous cell carcinoma, 5 (1.47%) with malignant melanoma, 21 (6.18%) with Bowen disease, and 6 (1.76%) with Paget’s disease. Four (1.18%) patients had metastatic skin cancer and seven (2.06%) had other malignant skin tumors. The patients comprised 132 (38.8%) men and 208 (61.1 %) women, and there were no statistically significant sex-related differences among the skin malignancies (x2 = 5.006, P > 0.05). Among the 340 patients, 314 (92.4%) were of Han nationality and 26 (7.6%) were of ethnic minorities. Statistically significant differences were found in the various types of malignant skin tumors between the Han and minority groups (x2 = 19.446, P < 0.05). Among the selected patients, 61.76% were farmers and herdsmen, and the sites mainly affected were the head, face, neck, trunk, limbs, and vulva.Conclusion:The incidence of malignant skin tumors in western Inner Mongolia has substantially increased during the past decade, especially in the past 2 years. Therefore, we should increase the awareness of prevention and treatment of malignant skin tumors to achieve early diagnosis and treatment effects.

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简介：AbstractPancreatic neuroendocrine tumors (PNETs) are a rare group of neoplasms originating from the endocrine pancreas. PNETs are classified as functional or non-functional tumors. PNETs are more often diagnosed at a higher stage with distant metastases or advanced locoregional disease. The majority of individuals with hepatic metastases will ultimately die of liver failure; therefore, the treatment of liver tumor burden is critical to providing a survival impact. While surgical resection remains the only chance of cure for disease confined to the pancreas or for locoregional disease, the treatment of advanced or metastatic PNETs is more complex and often requires a multimodal approach. This review focuses on treatment options for well and moderately differentiated PNETs with metastatic disease to the liver. These include surgery, liver-directed therapies including ablative and intra-arterial therapies, and systemic therapies such as somatostatin analogues, targeted therapies, chemotherapy, and peptide receptor radionuclide therapy. Developing an individualized treatment strategy requires careful assessment of liver tumor burden and predicted biological behavior. Aggressive surgical resection of hepatic metastases secondary to PNET primary tumors is associated with improved survival in multiple retrospective studies. General goals of treatment for metastatic disease include prolonging overall survival and progression free survival, improving quality of life, and control of symptoms.

简介：Superficialnon-ampullaryduodenalepithelialtumor(SNADET)isdefinedasasporadictumorthatisconfinedtothemucosaorsubmucosathatdoesnotarisefromVater’spapilla,anditincludesadenomaandadenocarcinoma.Recentdevelopmentsinendoscopictechnology,suchashigh-resolutionendoscopyandimage-enhancedendoscopy,mayincreasethechancesofdetectingSNADETlesions.However,becauseSNADETisrare,littleisknownaboutitspreoperativeendoscopicdiagnosis.TheuseofendoscopicresectionforSNADET,whichhasnoriskofmetastasis,isincreasing,buttheincidenceofcomplications,suchasperforation,issignificantlyhigherthaninanyotherpartofthedigestivetract.Apreoperativediagnosisisrequiredtodistinguishbetweenlesionsthatshouldbefollowedupandthosethatrequiretreatment.Retrospectivestudieshaverevealedcertainendoscopicfindingsthatsuggestmalignancy.Inrecentyears,severalnewimagingmodalitieshavebeendevelopedandexploredforrealtimediagnosisoftheselesiontypes.EstablishinganendoscopicdiagnostictooltodifferentiatebetweenadenomaandadenocarcinomainSNADETlesionsisrequiredtoselectthemostappropriatetreatment.ThisreviewdescribesthecurrentstateofknowledgeaboutpreoperativeendoscopicdiagnosisofSNADETs,suchasduodenaladenomaandduodenaladenocarcinoma.Newerendoscopictechniques,includingmagnifyingendoscopy,mayhelptoguidethesediagnostics,buttheiradditionaladvantagesremainunclear,andfurtherstudiesarerequiredtoclarifytheseissues.

简介：AbstractGastric carcinoma is extremely rare in pregnancy and the prognosis for this malignancy tends to be dismal. We herein describe a case of gastric cancer in pregnancy to alert clinicians to this rare possibility. A 29-year-old woman developed abdominal spastic pain and diarrhea during the 26 weeks of gestation and her condition was confusing and hard to recognize. The patient was initially misdiagnosed as enterogastritis and inflammatory intestinal obstruction and was finally confirmed by exploratory laparotomy with intestinal and peritoneum metastases. Because the disease was not detected early enough and progressed rapidly, the pregnancy was terminated by cesarean section at 30 weeks of gestation, and then followed by systemic chemotherapy, but eventually succumbed to the lethal pneumonia. Therefore, it is of great significance to alert clinicians to note this rare possibility and to consider the differential diagnosis of this disease in pregnant women with a long course of gastrointestinal symptoms that cannot be explained by pregnancy alone, and cancer should be suspected and tested with sophisticated diagnostic procedures.

简介：AbstractImportance:Cancer is the main cause of death by disease in children. Children experience the highest incidence of cancer in the first year of life. However, there is no comprehensive registration system for children with tumors in China.Objective:To summarize the diagnosis and treatment of infant cancer and analyze the status of standardized diagnosis and management among several treatment centers in Beijing, China, thereby providing evidence to guide further clinical research.Methods:From January 1, 2010 to December 31, 2019, patients with newly diagnosed infantile malignant solid tumors were admitted to six large tertiary pediatric solid tumor diagnosis and treatment centers in Beijing. The epidemiology, clinical features, and therapeutic effects of tumors in these patients were analyzed retrospectively. All patients were followed up until March 31, 2020.Results:In total, 938 patients were enrolled in this study. There were 530 boys (56.5%) and 408 girls (43.5%); the median age was 6.0 months (range, 0-12.0 months). The three most common tumors were retinoblastoma in 366 patients (39.0%), neuroblastoma in 266 patients (28.4%), hepatoblastoma in 133 patients (14.2%), and central nervous system tumors in 52 patients (5.5%). The estimated 5-year overall survival rate was 81.3% ± 1.8%, and the 5-year event-free survival rate was 71.8% ± 2.9%. The 5-year overall survival rates of non-rhabdomyosarcoma soft tissue sarcoma, neuroblastoma, and retinoblastoma were 100%, 88% ± 2.2%, and 86.9% ±2.1%, respectively. The 5-year event-free survival rates were 81.1% ± 2.7% for neuroblastoma, 81.6% ± 9.8% for non-rhabdomyosarcoma soft tissue sarcoma, and 72.7% ± 14.1% for extracranial malignant germ cell tumors.Interpretation:The three most common infantile malignant solid tumors were retinoblastoma, neuroblastoma, and hepatoblastoma. Multidisciplinary combined diagnosis and treatment is needed for infantile tumors.

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简介：Circulatingfreenucleicacids;cellfreeDNAandcirculatingmicro-RNA,arefoundintheplasmaofpatientswithhematologicandsolidmalignanciesatlevelshigherthanthatofhealthyindividuals.InpatientswithhematologicmalignancycellfreeDNAreflectstheunderlyingtumormutationalprofile,whilstmicro-RNAsreflectgeneticinterferencemechanismswithinatumorandpotentiallythesurroundingmicroenvironmentandimmuneeffectorcells.Thesecirculatingnucleicacidsofferapotentiallysimple,non-invasive,repeatableanalysisthatcanaidindiagnosis,prognosisandtherapeuticdecisionsincancertreatment.

简介：AbstractImportance:Pediatric hypervascular primary airway tumors are progressive, fatal lesions with a low incidence, and the disease is often more serious than that in adults.Objective:To evaluate the clinical efficacy and safety of interventional therapeutic bronchoscopy combined with conservative treatment and bronchial arterial embolization in children with primary airway tumors.Methods:We retrospectively analyzed the clinical data of four pediatric patients with hypervascular primary airway tumor between 2017 and 2019 at Beijing Children’s Hospital.Results:Two patients were low-grade bronchial mucoepidermoid carcinoma, one patient was pleomorphic adenoma, and one was bronchial leiomyoma. Interventional therapeutic bronchoscopy combined with bronchial arterial embolization was used for treatment (all four patients received general anesthesia). The tumors were safely resected in all patients via interventional bronchoscopy. There were no severe complications related to the procedures. All patients were followed up for 5-12 months, and one low-grade bronchial mucoepidermoid carcinoma recurred.Interpretation:Interventional therapeutic bronchoscopy combined with bronchial arterial embolization appears to be a safe and efficient therapeutic method associated with less trauma and fewer complications, including no serious adverse events, in children with hypervascular primary airway tumors without bronchus wall infiltration.