2023
Colorectal Carcinoma With Sarcomatoid Components
Golconda U, McHugh K, Allende D, Collins K, Henn P, Lacambra M, Bejarano P, Groisman G, Loughrey M, Monappa V, Zhang X, Hornick J, Gonzalez R. Colorectal Carcinoma With Sarcomatoid Components. The American Journal Of Surgical Pathology 2023, 48: 465-474. PMID: 38155543, DOI: 10.1097/pas.0000000000002172.Peer-Reviewed Original ResearchConceptsSarcomatoid componentColorectal carcinomaUndifferentiated carcinomaTumor-infiltrating lymphocytesNodal metastasisAbdominal painDistant metastasisRare subtypeSMARCA4 lossTumor buddingHeterologous elementsRepair protein expressionRectosigmoid regionSpindle cellsGastrointestinal bleedingKeratin-positivePoor prognosisFollow-upCarcinomaMismatch repair protein expressionMolecular testingAdvanced stageTumorLoss of mismatch repair protein expressionProtein expressionLarotrectinib efficacy and safety in adult patients with tropomyosin receptor kinase fusion sarcomas
Kummar S, Shen L, Hong D, McDermott R, Keedy V, Casanova M, Demetri G, Dowlati A, Melcón S, Lassen U, Leyvraz S, Liu T, Moreno V, Patel J, Patil T, Mallick A, Sousa N, Tahara M, Ziegler D, Norenberg R, Arvis P, Brega N, Drilon A, Tan D. Larotrectinib efficacy and safety in adult patients with tropomyosin receptor kinase fusion sarcomas. Cancer 2023, 129: 3772-3782. PMID: 37769113, PMCID: PMC11265530, DOI: 10.1002/cncr.35036.Peer-Reviewed Original ResearchConceptsTropomyosin receptor kinaseNTRK gene fusionsAdult patientsFusion sarcomaAdverse eventsData cutoffGrade 3 treatment-emergent adverse eventsTropomyosin receptor kinase fusion cancerCohort of adult patientsClinical management of adult patientsExtended survival benefitSafety of larotrectinibManagement of adult patientsGastrointestinal stromal tumorsSoft tissue sarcomasTreatment of patientsFirst-in-classGene fusionsInvestigator-assessedNTRK genesReceptor kinaseStromal tumorsSurvival benefitPediatric patientsSolid tumorsPot1b −/− tumors activate G-quadruplex-induced DNA damage to promote telomere hyper-elongation
Takasugi T, Gu P, Liang F, Staco I, Chang S. Pot1b −/− tumors activate G-quadruplex-induced DNA damage to promote telomere hyper-elongation. Nucleic Acids Research 2023, 51: 9227-9247. PMID: 37560909, PMCID: PMC10516629, DOI: 10.1093/nar/gkad648.Peer-Reviewed Original ResearchConceptsDNA damage responseDamage responseReplication protein A (RPA) complexDependent DNA damage responseTelomere length homeostasisTelomere maintenance mechanismLength homeostasisTelomerase recruitmentPOT1 proteinsHuman POT1Mouse genomeLength maintenanceFunction disruptsReplicative immortalityTelomeresPOT1 mutationsDNA damageHuman cancersLonger telomeresPOT1bMaintenance mechanismsSerial transplantationA complexesSimilar mechanismMutationsOutcomes After Preoperative Chemoradiation With or Without Pazopanib in Non-Rhabdomyosarcoma Soft Tissue Sarcoma: A Report From Children's Oncology Group and NRG Oncology
Weiss A, Chen Y, Scharschmidt T, Xue W, Gao Z, Black J, Choy E, Davis J, Fanburg-Smith J, Kao S, Kayton M, Kessel S, Lim R, Million L, Okuno S, Ostrenga A, Parisi M, Pryma D, Randall R, Rosen M, Shulkin B, Terezakis S, Venkatramani R, Zambrano E, Wang D, Hawkins D, Spunt S. Outcomes After Preoperative Chemoradiation With or Without Pazopanib in Non-Rhabdomyosarcoma Soft Tissue Sarcoma: A Report From Children's Oncology Group and NRG Oncology. Journal Of Clinical Oncology 2023, 41: 4842-4848. PMID: 37523624, PMCID: PMC10852395, DOI: 10.1200/jco.23.00045.Peer-Reviewed Original ResearchConceptsNon-rhabdomyosarcoma soft tissue sarcomasSoft tissue sarcomasRegimen BPreoperative chemoradiationRegimen ATissue sarcomasPathological responseMedian survivor follow-upComplete pathologic response ratePathological response rateEvent-free survivalPhase II studyPrimary end pointChildren's Oncology GroupIntent-to-treat analysisSurvivor follow-upNon-rhabdomyosarcomasPreoperative radiotherapyPrimary resectionOverall survivalOncology GroupII studyClinical trial updateNRG OncologyEligible patientsPhase II study of nivolumab and salvage nivolumab/ipilimumab in treatment-naïve patients with advanced non-clear cell renal cell carcinoma (HCRN GU16-260-Cohort B)
Atkins M, Jegede O, Haas N, McDermott D, Bilen M, Stein M, Sosman J, Alter R, Plimack E, Ornstein M, Hurwitz M, Peace D, Signoretti S, Denize T, Cimadamore A, Wu C, Braun D, Einstein D, Catalano P, Hammers H. Phase II study of nivolumab and salvage nivolumab/ipilimumab in treatment-naïve patients with advanced non-clear cell renal cell carcinoma (HCRN GU16-260-Cohort B). Journal For ImmunoTherapy Of Cancer 2023, 11: e004780. PMID: 36948504, PMCID: PMC10040058, DOI: 10.1136/jitc-2022-004780.Peer-Reviewed Original ResearchConceptsNivolumab/ipilimumabObjective response rateNon-clear cell renal cell carcinomaTreatment-naïve patientsCell renal cell carcinomaProgressive diseaseRenal cell carcinomaNivolumab monotherapyStable diseaseCell carcinomaAdvanced non-clear cell renal cell carcinomaMedian progression-free survivalTreatment-related adverse eventsDeath ligand 1 (PD-L1) expressionLigand 1 expressionPhase II studyProgression-free survivalWeeks of treatmentSymptomatic disease progressionEligible patientsSalvage therapyB patientsII studySalvage treatmentSarcomatoid featuresUpdate of pediatric bone tumors—other mesenchymal tumors of bone, hematopoietic neoplasms of bone, and WHO classification of undifferentiated small round cell sarcomas of bone
Wang A, Maloney E, Al-Dasuqi K, Irshaid L, Ahmed A, Haims A, Porrino J. Update of pediatric bone tumors—other mesenchymal tumors of bone, hematopoietic neoplasms of bone, and WHO classification of undifferentiated small round cell sarcomas of bone. Skeletal Radiology 2023, 52: 1443-1463. PMID: 36656343, DOI: 10.1007/s00256-023-04286-8.Peer-Reviewed Original ResearchConceptsUndifferentiated small round cell sarcomaSmall round cell sarcomasBone tumorsRound cell sarcomasPediatric populationCell sarcomaMesenchymal tumorsHematopoietic neoplasmsWorld Health Organization classificationGiant cell-rich tumorsPediatric bone tumorsOsteogenic tumorsOrganization classificationVascular tumorsNotochordal tumorsTumorsGenetic sequencing techniquesChondrogenic tumorsCertain lesionsSoft tissueBoneSarcomaNeoplasmsSeparate manuscriptDetailed update
2022
Impact of early detection on cancer curability: A modified Delphi panel study
Schwartzberg L, Broder M, Ailawadhi S, Beltran H, Blakely L, Budd G, Carr L, Cecchini M, Cobb P, Kansal A, Kim A, Monk B, Wong D, Campos C, Yermilov I. Impact of early detection on cancer curability: A modified Delphi panel study. PLOS ONE 2022, 17: e0279227. PMID: 36542647, PMCID: PMC9770338, DOI: 10.1371/journal.pone.0279227.Peer-Reviewed Original ResearchConceptsBlood testsLikelihood of benefitCancer typesEarly detectionEarly cancer detectionSolid tumorsTotal cancer incidenceAmerican Joint CommitteeLow cure rateRAND/UCLAPotential clinical benefitCancer detectionDelphi panel studyLong cancerClinical benefitCure ratePotential benefitsCancer curabilityMost cancer typesCancer incidenceHigh curabilityJoint CommitteeTreat cancersExpert consensusPanel consensuscircCsnk1g3- and circAnkib1-regulated interferon responses in sarcoma promote tumorigenesis by shaping the immune microenvironment
Piras R, Ko E, Barrett C, De Simone M, Lin X, Broz M, Tessaro F, Castillo-Martin M, Cordon-Cardo C, Goodridge H, Di Vizio D, Batish M, Lawrenson K, Chen Y, Chan K, Guarnerio J. circCsnk1g3- and circAnkib1-regulated interferon responses in sarcoma promote tumorigenesis by shaping the immune microenvironment. Nature Communications 2022, 13: 7243. PMID: 36433954, PMCID: PMC9700836, DOI: 10.1038/s41467-022-34872-8.Peer-Reviewed Original ResearchConceptsNon-coding RNA speciesExonic circular RNAsViral RNA sensorsRNA speciesInterferon-related genesSpecific circRNAsCircular RNAsSarcoma cellsCircRNAsRNA sensorsCancer progressionFunctional contributionInterferon responseSoft tissue sarcoma cellsImmune cell recruitmentPro-tumorigenic microenvironmentCellsTumor growthSarcoma growthTumor cellsPro-inflammatory elementsCell recruitmentActivationPro-inflammatory factorsMicroenvironmentFeasibility study of clinical target volume definition for soft-tissue sarcoma using muscle fiber orientations derived from diffusion tensor imaging
Shusharina N, Liu X, Coll-Font J, Foster A, Fakhri G, Woo J, Bortfeld T, Nguyen C. Feasibility study of clinical target volume definition for soft-tissue sarcoma using muscle fiber orientations derived from diffusion tensor imaging. Physics In Medicine And Biology 2022, 67: 155013. PMID: 35817048, PMCID: PMC9344976, DOI: 10.1088/1361-6560/ac8045.Peer-Reviewed Original ResearchConceptsClinical target volumeSoft tissue sarcomasHealthy volunteersClinical target volume definitionClinical target volume delineationRight thighGross tumor volumeTarget volume definitionEcho-planar acquisitionsTarget volumeAuto-segmentationTumor volumeVolume definitionSarcoma patientsTissue sarcomasCT planeConvolutional neural networkT2-weightedCancer CenterPlanar acquisitionImaging sessionFat suppressionMR imagingSarcomaAnatomical orientationPrimary Mesenchymal Tumors of the Thyroid Gland: A Modern Retrospective Cohort Including the First Case of TFE3-Translocated Malignant Perivascular Epithelioid Cell Tumor (PEComa)
Zhang L, Lubin D, Sinard JH, Dickson BC, Antonescu CR, Wu H, Panni RZ, Dogan S, Untch BR, Ghossein RA, Xu B. Primary Mesenchymal Tumors of the Thyroid Gland: A Modern Retrospective Cohort Including the First Case of TFE3-Translocated Malignant Perivascular Epithelioid Cell Tumor (PEComa). Head And Neck Pathology 2022, 16: 716-727. PMID: 35218514, PMCID: PMC9424363, DOI: 10.1007/s12105-022-01428-7.Peer-Reviewed Original ResearchConceptsMalignant perivascular epithelioid cell tumorPerivascular epithelioid cell tumorEpithelioid cell tumorPrimary mesenchymal tumorsMesenchymal tumorsThyroid glandCell tumorsPeripheral nerve sheath tumorsNerve sheath tumorsSmall case seriesBiphasic synovial sarcomaOnly case reportsSolitary fibrous tumorAnaplastic thyroid carcinomaMulti-centric cohortFirst caseRetrospective cohortEpithelioid hemangioendotheliomaSheath tumorsTertiary centerCase seriesClinicopathologic featuresFemale patientsSynovial sarcomaCase reportGlobal assessment of IRF8 as a novel cancer biomarker
McQuaid DC, Panse G, Wang WL, Pinkus GS, Katz SG, Xu ML. Global assessment of IRF8 as a novel cancer biomarker. Human Pathology 2022, 122: 1-10. PMID: 35085599, PMCID: PMC10621657, DOI: 10.1016/j.humpath.2022.01.004.Peer-Reviewed Original ResearchConceptsBlastic plasmacytoid dendritic cell neoplasmInterferon regulatory factor 8Myeloid sarcomaDifferential diagnosisPlasmacytoid dendritic cell neoplasmDesmoplastic small round cell tumorSmall round cell tumorTriple-negative breast cancerStrong uniform expressionDendritic cell neoplasmBroad differential diagnosisNegative breast cancerRound cell tumorAbsence of CD34IHC expression patternsAcute monocytic leukemiaNovel immunohistochemical markerTypes of cancerRegulatory factor 8Cancer Genome AtlasNovel cancer biomarkersRetrospective studySynovial sarcomaCell tumorsPleomorphic sarcoma
2021
Deep learning-based GTV contouring modeling inter- and intra- observer variability in sarcomas
Marin T, Zhuo Y, Lahoud R, Tian F, Ma X, Xing F, Moteabbed M, Liu X, Grogg K, Shusharina N, Woo J, Lim R, Ma C, Chen Y, El Fakhri G. Deep learning-based GTV contouring modeling inter- and intra- observer variability in sarcomas. Radiotherapy And Oncology 2021, 167: 269-276. PMID: 34808228, PMCID: PMC8934266, DOI: 10.1016/j.radonc.2021.09.034.Peer-Reviewed Original ResearchConceptsGross tumor volumeRadiation therapy treatment planningGross tumor volume contoursGross tumor volume delineationTherapy treatment planningIntra-observer variabilityConsensus contoursGTV contoursPre-operative CT imagesSoft tissue sarcomasRadiation oncologistsTumor volumeBone sarcomasTreatment planningAccurate contoursCT imagesDelineation procedureSarcomaSoft tissueConfidence levelRadiationPatientsHausdorff distanceMultiple contoursX-rayNUTM1-Rearranged Neoplasms—A Heterogeneous Group of Primitive Tumors with Expanding Spectrum of Histology and Molecular Alterations—An Updated Review
Luo W, Stevens T, Stafford P, Miettinen M, Gatalica Z, Vranic S. NUTM1-Rearranged Neoplasms—A Heterogeneous Group of Primitive Tumors with Expanding Spectrum of Histology and Molecular Alterations—An Updated Review. Current Oncology 2021, 28: 4485-4503. PMID: 34898574, PMCID: PMC8628659, DOI: 10.3390/curroncol28060381.Peer-Reviewed Original ResearchConceptsGenome-wide histone modificationsPost-meiotic spermatidsExpression of oncogenesTumor suppressor geneTranscription regulationHistone modificationsBromodomain proteinsNuclear proteinsRNA sequencingFusion proteinProtein productsSuppressor geneFusion partnerGenesSitu hybridizationDifferent clinical coursesProteinSkin adnexal tumorsPrimitive morphologyOncogenic driversInhibitor therapyClinical courseMolecular alterationsPrimitive tumorHematologic malignanciesDistinct functions of POT1 proteins contribute to the regulation of telomerase recruitment to telomeres
Gu P, Jia S, Takasugi T, Tesmer VM, Nandakumar J, Chen Y, Chang S. Distinct functions of POT1 proteins contribute to the regulation of telomerase recruitment to telomeres. Nature Communications 2021, 12: 5514. PMID: 34535663, PMCID: PMC8448735, DOI: 10.1038/s41467-021-25799-7.Peer-Reviewed Original ResearchConceptsDNA damage responseTelomerase recruitmentPOT1 proteinsDamage responseATR-dependent DNA damage responseNon-homologous end-joining DNA repair pathwayRecruitment of telomeraseC-strand fillAmino acidsDNA repair pathwaysUnique amino acidsTEN1 (CST) complexTelomere extensionCTC1-STN1Stable heterodimerRepair pathwaysC-terminusDistinct functionsPOT1bPOT1aTelomeresC-strandG-strandTPP1ProteinNCCN Guidelines® Insights: Uterine Neoplasms, Version 3.2021.
Abu-Rustum NR, Yashar CM, Bradley K, Campos SM, Chino J, Chon HS, Chu C, Cohn D, Crispens MA, Damast S, Diver E, Fisher CM, Frederick P, Gaffney DK, George S, Giuntoli R, Han E, Howitt B, Huh WK, Lea J, Mariani A, Mutch D, Nekhlyudov L, Podoll M, Remmenga SW, Reynolds RK, Salani R, Sisodia R, Soliman P, Tanner E, Ueda S, Urban R, Wethington SL, Wyse E, Zanotti K, McMillian NR, Motter AD. NCCN Guidelines® Insights: Uterine Neoplasms, Version 3.2021. Journal Of The National Comprehensive Cancer Network 2021, 19: 888-895. PMID: 34416706, DOI: 10.6004/jnccn.2021.0038.Peer-Reviewed Original ResearchProton radiotherapy for recurrent or metastatic sarcoma with palliative quad shot
Lee A, Kang J, Bernstein H, Marqueen K, Neal B, Kelly C, Dickson M, Tsai C, Tap W, Singer S, Alektiar K, Lee N. Proton radiotherapy for recurrent or metastatic sarcoma with palliative quad shot. Cancer Medicine 2021, 10: 4221-4227. PMID: 34085781, PMCID: PMC8267151, DOI: 10.1002/cam4.3646.Peer-Reviewed Original ResearchConceptsLocal progression-free survivalMetastatic sarcomaSystemic therapyQUAD ShotOverall survivalUnivariate analysisPalliative responseMedian local progression-free survivalGrade 3 acute toxicityFurther systemic therapyProgression-free survivalConcurrent systemic therapyProton therapyCommon histologyMedian followProton RTTreatment optionsRadiation therapyPatientsLocal controlSarcomaTherapyRegimenProton radiotherapyAcute toxicitySystematic review of charged-particle therapy for chordomas and sarcomas of the mobile spine and sacrum.
Pennington Z, Ehresman J, Elsamadicy AA, Shin JH, Goodwin CR, Schwab JH, Sciubba DM. Systematic review of charged-particle therapy for chordomas and sarcomas of the mobile spine and sacrum. Neurosurgical FOCUS 2021, 50: e17. PMID: 33932924, DOI: 10.3171/2021.2.focus201059.Peer-Reviewed Original ResearchConceptsDisease-free survivalProgression-free survivalDisease-specific survivalOverall survivalLocal controlMobile spineLong-term local controlAcute toxicityManagement of sarcomasEn bloc resectionCharged-particle therapyUse of protonsFull-text screeningDefinitive radiotherapyAdjuvant radiationDefinitive radiationSurgical cohortSurgical groupPrimary sarcomaUnresectable tumorsBloc resectionNegative marginsRadiation toxicityConventional radiotherapyHigh riskRadical Exenteration of the Skull Base for End-Stage, Locally Advanced Sinonasal Malignancies: Challenging the Dictum of Unresectability
Yeung JT, Caminer DM, Young IM, Sughrue ME, Teo C. Radical Exenteration of the Skull Base for End-Stage, Locally Advanced Sinonasal Malignancies: Challenging the Dictum of Unresectability. World Neurosurgery 2021, 150: e102-e107. PMID: 33647490, DOI: 10.1016/j.wneu.2021.02.092.Peer-Reviewed Original ResearchMeSH KeywordsAdolescentAdultCarcinomaCarcinoma, Adenoid CysticChildEsthesioneuroblastoma, OlfactoryFemaleHumansMaleMaxillary Sinus NeoplasmsMiddle AgedNasopharyngeal CarcinomaNeoplasm StagingNeurofibrosarcomaNeurosurgical ProceduresNose NeoplasmsOsteosarcomaParanasal Sinus NeoplasmsProgression-Free SurvivalSalvage TherapySarcomaSkull BaseSquamous Cell Carcinoma of Head and NeckSurvival RateYoung AdultConceptsAdvanced sinonasal cancerSinonasal cancerPerioperative complicationsSalvage surgeryIntracranial involvementSingle-center retrospective reviewAdvanced sinonasal malignanciesAdvanced sinonasal tumorsDetails of chemotherapyRadical salvage surgerySalvage surgical resectionRole of surgeryPrior surgeryOverall survivalPatient demographicsSurgical resectionRetrospective reviewAggressive diseasePalliative careSinonasal malignanciesSurvival outcomesSurgical approachTreatment optionsIntracranial invasionSinonasal tumorsEvaluation of head and neck soft tissue sarcoma 8th edition pathologic staging system and proposal of a novel stage grouping system
Lee NCJ, Eskander A, Miccio JA, Park HS, Shah C, Rutenberg M, Hosni A, Husain ZA. Evaluation of head and neck soft tissue sarcoma 8th edition pathologic staging system and proposal of a novel stage grouping system. Oral Oncology 2021, 114: 105137. PMID: 33422859, DOI: 10.1016/j.oraloncology.2020.105137.Peer-Reviewed Original ResearchConceptsNeck soft tissue sarcomasTumor size cutNational Cancer DatabaseSoft tissue sarcomasMultivariable analysisTissue sarcomasFive-year overall survivalNeoadjuvant therapy patientsT1-3 tumorsEnd Results (SEER) databaseAdjacent structuresEvaluation of headPaucity of dataNCDB cohortSEER cohortPrimary surgeryOverall survivalWorsened survivalAdverse prognosticatorPrognostic differencesResults databaseStaging systemT4 classificationTumor stageCancer Database
2020
A Phase 1b Study Evaluating the Safety, Tolerability, and Immunogenicity of CMB305, a Lentiviral-Based Prime-Boost Vaccine Regimen, in Patients with Locally Advanced, Relapsed, or Metastatic Cancer Expressing NY-ESO-1
Somaiah N, Chawla SP, Block MS, Morris JC, Do K, Kim JW, Druta M, Sankhala KK, Hwu P, Jones RL, Gnjatic S, Kim-Schulze S, Tuballes K, Yishak M, Lu H, Yakovich A, Meulen J, Chen M, Kenney RT, Bohac C, Pollack SM. A Phase 1b Study Evaluating the Safety, Tolerability, and Immunogenicity of CMB305, a Lentiviral-Based Prime-Boost Vaccine Regimen, in Patients with Locally Advanced, Relapsed, or Metastatic Cancer Expressing NY-ESO-1. OncoImmunology 2020, 9: 1847846. PMID: 33312760, PMCID: PMC7714520, DOI: 10.1080/2162402x.2020.1847846.Peer-Reviewed Original ResearchConceptsNY-ESO-1Prime-boost vaccine regimenVaccine regimenImmune responseNY-ESO-1 immune responsesNY-ESO-1 antibodyNY-ESO-1 proteinToll-like receptor agonistsInjection site painOral metronomic cyclophosphamidePhase 1b studyRecombinant protein boostCommon adverse eventsDisease control ratePrime-boost vaccineT cell responsesDose-escalation designSoft tissue sarcomasLentiviral vectorsMetronomic cyclophosphamideAdverse eventsMetastatic diseaseOverall survivalProgressive diseaseStudy entry
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