2025
Treatment of Extraosseous Giant Cell Tumor of Bone and Calcitriol-Mediated Hypercalcemia With Denosumab in Paget Disease
Magvanjav O, Bergwitz C. Treatment of Extraosseous Giant Cell Tumor of Bone and Calcitriol-Mediated Hypercalcemia With Denosumab in Paget Disease. JCEM Case Reports 2025, 3: luaf031. PMID: 40110572, PMCID: PMC11920620, DOI: 10.1210/jcemcr/luaf031.Peer-Reviewed Original ResearchBone-specific alkaline phosphataseGiant cell tumorCell tumorsPaget's diseaseCalcitriol-mediated hypercalcemiaOsteoclast-like giant cellsDose of denosumabTreated with bisphosphonatesReduced tumor sizeOne-time doseElevated calcitriolParathyroid autonomyPolyostotic PDBStromal mononuclear cellsPelvic massSymptomatic hypercalcemiaTumor regrowthTumor sizeParathyroid hormoneKidney injuryNormal calciumMononuclear cellsDenosumabHypercalcemiaLong-term management option
2024
Lesions of Nipple and Axilla
Andrejeva L, Venkateswaran J, Vidhun R, Killelea B, Brownson K, Singh K. Lesions of Nipple and Axilla. 2024, 269-295. DOI: 10.1007/978-3-031-65711-5_10.Peer-Reviewed Original ResearchSquamous metaplasia of lactiferous ductsMammary duct ectasiaIntraductal papillomaSubareolar tissueDuct ectasiaMalignant lesionsNipple adenomaSubareolar ductsCase of incomplete excisionPaget's diseaseMammographically occult malignanciesBenign epithelial lesionsIntraductal epithelial proliferationRisk of recurrenceTreatment of choicePeriareolar regionOccult malignancyNegative marginsIncomplete excisionSurgical excisionSyringomatous adenomaBreast carcinomaInvasive carcinomaNegative mammogramEpithelial lesionsRecommended guidelines for screening for underlying malignancy in extramammary Paget's disease based on anatomic subtype
Kibbi N, Owen J, Worley B, Alam M, Group E, Wang J, Harikumar V, Downing M, Aasi S, Aung P, Barker C, Bolotin D, Bordeaux J, Cartee T, Chandra S, Cho N, Choi J, Chung K, Cliby W, Dorigo O, Eisen D, Fujisawa Y, Golda N, Halfdanarson T, Iavazzo C, Jiang S, John E, Kanitakis J, Keimig E, Khan A, Linos E, Kim J, Kuzel T, Lawrence N, Leitao M, MacLean A, Maher I, Mittal B, Nehal K, Ozog D, Pettaway C, Rajan N, Ross J, Rossi A, Servaes S, Solomon M, Thomas V, Tolia M, Voelzke B, Waldman A, Wong M, Zhou Y, Brackett A, Poon E, Ahmed A. Recommended guidelines for screening for underlying malignancy in extramammary Paget's disease based on anatomic subtype. Journal Of The American Academy Of Dermatology 2024, 92: 261-268. PMID: 39401611, DOI: 10.1016/j.jaad.2024.07.1531.Peer-Reviewed Original ResearchExtramammary Paget's diseaseSecondary extramammary Paget's diseaseVulvar extramammary Paget diseasePerianal extramammary Paget's diseaseUrine cytologyAnatomic subtypesComputed tomographyPaget's diseaseProstate-specific antigen testingHigh-risk featuresOrgan-specific testsMultidisciplinary expert panelUnderlying malignancySystematic follow-upFollow-upAdenocarcinomaAntigen testAnatomical locationInclusion criteriaCytologyRetrospective dataScreening testSubtypesScreening algorithmRecommendation statementsVulvar Cancer, Version 3.2024, NCCN Clinical Practice Guidelines in Oncology.
Abu-Rustum N, Yashar C, Arend R, Barber E, Bradley K, Brooks R, Campos S, Chino J, Chon H, Crispens M, Damast S, Fisher C, Frederick P, Gaffney D, Gaillard S, Giuntoli R, Glaser S, Holmes J, Howitt B, Kendra K, Lea J, Lee N, Mantia-Smaldone G, Mariani A, Mutch D, Nagel C, Nekhlyudov L, Podoll M, Rodabaugh K, Salani R, Schorge J, Siedel J, Sisodia R, Soliman P, Ueda S, Urban R, Wethington S, Wyse E, Zanotti K, McMillian N, Espinosa S. Vulvar Cancer, Version 3.2024, NCCN Clinical Practice Guidelines in Oncology. Journal Of The National Comprehensive Cancer Network 2024, 22: 117-135. PMID: 38503056, DOI: 10.6004/jnccn.2024.0013.Peer-Reviewed Original ResearchConceptsNCCN Clinical Practice GuidelinesVulvar cancerClinical practice guidelinesCell carcinomaHistologically squamous cell carcinomaSystemic therapy optionsSquamous cell carcinomaBasal cell carcinomaExtramammary Paget's diseasePractice guidelinesVerrucous carcinomaGynecologic malignanciesHuman papillomavirusVulvar neoplasiaTherapy optionsPaget's diseaseInflammatory conditionsRisk factorsCigarette smokingCarcinomaCancerIncreasing ageHistologyNCCNVulvar
2023
Therapeutic developments for valosin-containing protein mediated multisystem proteinopathy
Boock V, Roy B, Pfeffer G, Kimonis V. Therapeutic developments for valosin-containing protein mediated multisystem proteinopathy. Current Opinion In Neurology 2023, 36: 432-440. PMID: 37678339, DOI: 10.1097/wco.0000000000001184.Peer-Reviewed Original ResearchConceptsInclusion body myopathyPotential therapeutic targetValosin-containing proteinPaget's diseasePreclinical modelsTherapeutic approachesPotential therapyControl trialRare diseaseTherapeutic targetBody myopathyVivo modelFrontotemporal dementiaVCP mutationsMultisystem proteinopathyPathway involvementDiseaseTherapyMitochondrial dysfunctionTherapeutic developmentGene therapyMissense mutationsFunction activityATPase activityRCTs
2022
The role of HER2 as a therapeutic biomarker in gynaecological malignancy: potential for use beyond uterine serous carcinoma
Talia K, Banet N, Buza N. The role of HER2 as a therapeutic biomarker in gynaecological malignancy: potential for use beyond uterine serous carcinoma. Pathology 2022, 55: 8-18. PMID: 36503635, DOI: 10.1016/j.pathol.2022.11.004.Peer-Reviewed Original ResearchConceptsHuman epidermal growth factor receptor 2Uterine serous carcinomaRole of HER2Serous carcinomaClinical trialsHER2 overexpressionPrognostic biomarkerTherapeutic targetHigh-grade endometrioid carcinomaEpidermal growth factor receptor 2Vulvar Paget's diseaseGrowth factor receptor 2Time of diagnosisFuture clinical trialsGynaecological cancer careClinical trial dataHER2 protein overexpressionFactor receptor 2P53 mutant tumorsRecurrent diseaseSurvival benefitGynaecological malignanciesPaget's diseaseClinical benefitEndometrial carcinomaEvidence-Based Clinical Practice Guidelines for Extramammary Paget Disease
Kibbi N, Owen J, Worley B, Wang J, Harikumar V, Downing M, Aasi S, Aung P, Barker C, Bolotin D, Bordeaux J, Cartee T, Chandra S, Cho N, Choi J, Chung K, Cliby W, Dorigo O, Eisen D, Fujisawa Y, Golda N, Halfdanarson T, Iavazzo C, Jiang S, Kanitakis J, Khan A, Kim J, Kuzel T, Lawrence N, Leitao M, MacLean A, Maher I, Mittal B, Nehal K, Ozog D, Pettaway C, Ross J, Rossi A, Servaes S, Solomon M, Thomas V, Tolia M, Voelzke B, Waldman A, Wong M, Zhou Y, Arai N, Brackett A, Ibrahim S, Kang B, Poon E, Alam M. Evidence-Based Clinical Practice Guidelines for Extramammary Paget Disease. JAMA Oncology 2022, 8: 618-628. PMID: 35050310, DOI: 10.1001/jamaoncol.2021.7148.Peer-Reviewed Original ResearchConceptsExtramammary Paget's diseaseSecondary extramammary Paget's diseaseClinical practice guidelinesNonsurgical treatmentPaget's diseaseMalignant neoplasmsPractice guidelinesEvidence-based clinical practice guidelinesInvasive extramammary Paget's diseaseCarbon dioxide laser therapyClinical care recommendationsInternal malignant neoplasmsMultiple skin biopsiesDistant metastatic diseaseLymph node dissectionHigh-risk tumorsCare of adultsSuperior management approachCurative intentNode dissectionProspective registryMetastatic diseaseSentinel lymphSurgical resectionTumor characteristicsDevelopment of a standard of care for patients with valosin-containing protein associated multisystem proteinopathy
Korb M, Peck A, Alfano LN, Berger KI, James MK, Ghoshal N, Healzer E, Henchcliffe C, Khan S, Mammen PPA, Patel S, Pfeffer G, Ralston SH, Roy B, Seeley WW, Swenson A, Mozaffar T, Weihl C, Kimonis V. Development of a standard of care for patients with valosin-containing protein associated multisystem proteinopathy. Orphanet Journal Of Rare Diseases 2022, 17: 23. PMID: 35093159, PMCID: PMC8800193, DOI: 10.1186/s13023-022-02172-5.Peer-Reviewed Original ResearchConceptsStandard of careAmyotrophic lateral sclerosisCharcot Marie Tooth diseaseMultisystem proteinopathyFrontotemporal dementiaSupportive therapyValosin-containing proteinMarie Tooth diseaseExpert consensus recommendationsRare inherited disorderInclusion body myopathyMulti-gene panel sequencingSingle-gene testingNeuromuscular centerAppropriate pharmacotherapyMultidisciplinary carePaget's diseaseSecondary complicationsVCP diseaseMultisystem involvementMovement disordersPatient advocacy organizationsPhysical therapyPractice guidelinesConsensus recommendations
2021
Treatment of Metastatic Extramammary Paget Disease with Combination Ipilimumab and Nivolumab: A Case Report
Guercio BJ, Iyer G, Kidwai WZ, Lacouture ME, Ghafoor S, Rossi AM, Assis DN, Chen YB, Busam KJ, Janjigian YY, Jhaveri K, Feldman DR, Capozzi A, Figueroa V, Bajorin DF, Rosenberg JE, Hollmann TJ, Funt SA. Treatment of Metastatic Extramammary Paget Disease with Combination Ipilimumab and Nivolumab: A Case Report. Case Reports In Oncology 2021, 14: 430-438. PMID: 33790764, PMCID: PMC7983595, DOI: 10.1159/000514345.Peer-Reviewed Original ResearchMetastatic extramammary Paget's diseaseExtramammary Paget's diseaseStandard therapyPaget's diseaseTreatment responsePrimary cutaneous extramammary Paget diseaseCutaneous extramammary Paget's diseaseHigh PD-L1 expressionHigh tumor mutation burdenCycles of ipilimumabDurable partial responseImmune checkpoint inhibitionImmune checkpoint inhibitorsPD-L1 expressionCheckpoint inhibitor immunotherapyRare clinical entityTumor mutation burdenMetastatic tumor tissueCombination ipilimumabCheckpoint inhibitorsMetastatic diseaseCytotoxic chemotherapyPartial responseCheckpoint inhibitionClinical entity
2020
Paget disease of the breast: A national retrospective analysis of the US population
Sisti A, Huayllani MT, Restrepo DJ, Boczar D, Advani P, Lu X, Spaulding AC, Ball CT, McLaughlin SA, Forte AJ. Paget disease of the breast: A national retrospective analysis of the US population. Breast Disease 2020, 39: 119-126. PMID: 32390594, DOI: 10.3233/bd-200439.Peer-Reviewed Original ResearchConceptsNational Cancer DatabasePaget's diseaseComplete mastectomyPartial mastectomyCommon treatmentSurvival rateRegional lymph node metastasisBlack race/ethnicityHigher cancer stageNational retrospective analysisOverall survival rateLymph node metastasisLarger tumor sizeHigh tumor gradeRace/ethnicityMedian ageNode metastasisFemale patientsShorter survivalTumor sizeCancer DatabaseCancer stageTumor gradeTreatment characteristicsMetastatic cancer
2017
Traumatic Rib Injury: Patterns, Imaging Pitfalls, Complications, and Treatment
Talbot BS, Gange CP, Chaturvedi A, Klionsky N, Hobbs SK, Chaturvedi A. Traumatic Rib Injury: Patterns, Imaging Pitfalls, Complications, and Treatment. RadioGraphics 2017, 37: 160100. PMID: 28186860, DOI: 10.1148/rg.2017160100.Peer-Reviewed Original ResearchConceptsRib injuriesRib fracturesAbdominal solid organ injuriesPediatric nonaccidental traumaPrimary osseous neoplasmsSolid organ injuryAcute vascular injuryCare of patientsEmergency department settingOrgan-specific injuryPrinciples of managementPulmonary contusionFlail chestMetastatic diseasePathologic fracturePulmonary lacerationThoracic traumaTrauma complicationsPaget's diseaseSignificant morbidityNonaccidental traumaVascular injuryBifid ribsDepartment settingExtrapleural hematoma
2015
Bone turnover markers in Paget’s disease of the bone: A Systematic review and meta-analysis
Al Nofal A, Altayar O, BenKhadra K, Qasim Agha O, Asi N, Nabhan M, Prokop L, Tebben P, Murad M. Bone turnover markers in Paget’s disease of the bone: A Systematic review and meta-analysis. Osteoporosis International 2015, 26: 1875-1891. PMID: 26037791, DOI: 10.1007/s00198-015-3095-0.Peer-Reviewed Original ResearchConceptsBone turnover markersBone turnover marker concentrationsTurnover markersDisease activityDisease of bonePaget's diseaseBone ALPBone scintigraphyMeta-analysisConcentrations of bone turnover markersIntroductionPaget’s disease of boneMeta-analysis searching MEDLINEUrine N-terminal telopeptideProcollagen type 1 amino-terminal propeptideSystematic reviewBone-specific alkaline phosphataseMarkers of disease activityCorrelation of disease activityPresence of liver diseaseDisease patientsCochrane Central Register of Controlled TrialsQuantitative bone scintigraphyC-terminal telopeptideCentral Register of Controlled TrialsN-terminal telopeptideRisk stratification in extramammary Paget disease
Cohen JM, Granter SR, Werchniak AE. Risk stratification in extramammary Paget disease. Clinical And Experimental Dermatology 2015, 40: 473-478. PMID: 26011765, DOI: 10.1111/ced.12690.Peer-Reviewed Original ResearchConceptsExtramammary Paget's diseaseRisk stratificationPaget's diseaseUncommon intraepithelial adenocarcinomaImportant prognostic informationDepth of invasionIntraepithelial adenocarcinomaPatient ageExtracutaneous sitesLymph nodesPrognostic factorsPoor prognosisMucin 5ACPrognostic informationKi-67Perianal regionHigh riskLesion siteClinical practiceApocrine glandsBody sitesTumor geneticsReticular dermisCyclin D1Treatment planning
2010
Lesions of the Vulva and Vagina
Chhieng D, Hui P. Lesions of the Vulva and Vagina. Current Clinical Pathology 2010, 15-34. DOI: 10.1007/978-1-60761-164-6_2.Peer-Reviewed Original ResearchSquamous cell carcinomaCell carcinomaPrimary clear cell carcinomaVulvar squamous cell carcinomaLow-risk HPV subtypesIntrauterine DES exposureHigh-risk HPVExtramammary Paget's diseaseMalignant mesenchymal tumorSquamous intraepithelial neoplasiaCommon primary malignancyClear cell carcinomaPreinvasive conditionsVulvar cancerHPV subtypesPrimary malignancyDES exposureLichen sclerosusIntraepithelial neoplasiaPaget's diseaseSquamous carcinomaWidespread metastasesAggressive angiomyxomaCondyloma acuminatumMesenchymal tumorsHER-2/NEU overexpression in vulvar Paget disease: the Yale experience
Richter CE, Hui P, Buza N, Silasi DA, Azodi M, Santin AD, Schwartz PE, Rutherford TJ. HER-2/NEU overexpression in vulvar Paget disease: the Yale experience. Journal Of Clinical Pathology 2010, 63: 544. PMID: 20418225, DOI: 10.1136/jcp.2010.077446.Peer-Reviewed Original ResearchConceptsVulvar Paget's diseasePaget's diseaseNeu overexpressionOutcome dataHER-2/neu expressionHER-2/neuEfficacy of trastuzumabExtensive reconstructive proceduresHigh recurrence rateMedical record searchInteresting treatment optionSurgical treatmentRecurrence rateTreatment optionsNeu expressionClinical trialsReconstructive proceduresTissue microarrayPatientsRecord searchTumor samplesYale experienceDiseaseNeuStaining results
2005
Mohs micrographic surgery: established uses and emerging trends.
Pennington BE, Leffell DJ. Mohs micrographic surgery: established uses and emerging trends. Oncology 2005, 19: 1165-71; discussion 1171-2, 1175. PMID: 16255133.Peer-Reviewed Original ResearchConceptsSquamous cell carcinomaBasal cell carcinomaMohs micrographic surgeryCell carcinomaMicrographic surgeryCommon cutaneous neoplasmsConventional surgical excisionExtramammary Paget's diseaseMicrocystic adnexal carcinomaMerkel cell carcinomaCutaneous neoplasmsPaget's diseaseResidual tumorSurgical excisionAdnexal carcinomaCommon cancerCutaneous tumorsSurgical techniqueDermatofibrosarcoma protuberansCarcinomaDeep marginTissue conservationSurgeryTumorsDisease
2002
Liposomal Doxorubicin for Treatment of Metastatic Chemorefractory Vulvar Adenocarcinoma
Huang GS, Juretzka M, Ciaravino G, Kohler S, Teng NN. Liposomal Doxorubicin for Treatment of Metastatic Chemorefractory Vulvar Adenocarcinoma. Gynecologic Oncology 2002, 87: 313-318. PMID: 12468332, DOI: 10.1006/gyno.2002.6830.Peer-Reviewed Original ResearchConceptsVulvar adenocarcinomaLiposomal doxorubicinPelvic lymph node dissectionGroin lymph nodesLymph node dissectionExtramammary Paget's diseaseYears of survivalWarrants further investigationBulky involvementGroin irradiationInitial therapyNode dissectionMultiagent chemotherapyRadical vulvectomyGynecologic malignanciesMetastatic diseaseLymph nodesInitial diagnosisMetastatic lesionsPaget's diseaseDramatic regressionRare entityDoxil treatmentEffective treatmentSubcutaneous lesions
1993
Mice Transgenic for HTLV-I LTR-tax Exhibit Tax Expression in Bone, Skeletal Alterations, and High Bone Turnover
Ruddle N, Li C, Horn W, Santiago P, Troiano N, Jay G, Horowitz M, Baron R. Mice Transgenic for HTLV-I LTR-tax Exhibit Tax Expression in Bone, Skeletal Alterations, and High Bone Turnover. Virology 1993, 197: 196-204. PMID: 8212554, DOI: 10.1006/viro.1993.1580.Peer-Reviewed Original ResearchConceptsHigh bone turnoverHTLV-I infectionBone turnoverMice transgenicSkeletal alterationsAdult T-cell leukemiaMonths of ageSpindle-shaped cellsT-cell leukemiaBone cell activityPaget's diseaseViral etiologyOsteoclast numberBone resorptionPathogenetic mechanismsBone diseaseCell leukemiaBone marrowCell activitySkeletal changesTax mRNASevere skeletal abnormalitiesHost factorsDiseaseSkeletal abnormalities
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