Neoplasia maligna em que há infiltração da pele por grandes células neoplásicas com abundante citoplasma pálido e núcleos grandes com nucléolos proeminentes (células de Paget). Pode afetar o ânus, o pênis, o escroto e a vulva.
Introdução
O que você precisa saber de cara
A Doença de Paget extramamária é um tipo raro de câncer de pele que atinge principalmente adultos e idosos na região genital e anal. Ela costuma se manifestar por uma dermatite eczematoide (uma irritação avermelhada e descamativa na pele) e pela formação de nódulos ou feridas tumorais. O diagnóstico depende da análise microscópica da pele para identificar a presença das chamadas células de Paget. No SUS, existe um protocolo do Ministério da Saúde para Doença de Paget, mas ele é apenas um documento relacionado e não cobre esta forma extramamária específica.
Neoplasia maligna em que há infiltração da pele por grandes células neoplásicas com abundante citoplasma pálido e núcleos grandes com nucléolos proeminentes (células de Paget). Pode afetar o ânus, o pênis, o escroto e a vulva.
Tem tratamento?
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 2 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Genes associados
1 gene identificado com associação a esta condição.
Glioma
Gliomas are benign or malignant central nervous system neoplasms derived from glial cells. They comprise astrocytomas and glioblastoma multiforme that are derived from astrocytes, oligodendrogliomas derived from oligodendrocytes and ependymomas derived from ependymocytes.
Variantes genéticas (ClinVar)
58 variantes patogênicas registradas no ClinVar.
Vias biológicas (Reactome)
30 vias biológicas associadas aos genes desta condição.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
🇧🇷 Atendimento SUS — Doença de Paget extramamária
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Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.
Pesquisa ativa
Ensaios clínicos abertos e novidades científicas recentes
Ensaios em destaque
Com ensaio aberto em 3 países. O ponto verde marca onde há vaga agora.
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Outros ensaios clínicos
12 ensaios clínicos encontrados, 10 ativos.
Publicações mais relevantes
Efficacy of Radiation Therapy in 106 Patients with Extramammary Paget Disease: A Retrospective Study.
Extramammary Paget disease (EMPD) is rare among Asians, and the clinical value of radiation therapy for EMPD remains controversial. This retrospective study evaluated the efficacy of radiation therapy for EMPD. From January 2012 to December 2021, 106 patients with pathologically confirmed EMPD with complete follow-up data were treated at Peking University First Hospital. The primary treatment was surgery combined with adjuvant radiation therapy, with radical radiation therapy for inoperable patients. The main surgical method for the primary lesion was Mohs micrographic surgery, with a few patients undergoing wide local excision. The recommended postoperative and radical radiation therapy doses were 50 to 60 Gy and 60 to 70 Gy, respectively. The primary endpoint was disease-specific survival rate; secondary endpoints included local recurrence-free survival, lymph node metastasis, and distant metastasis rates. Survival rates were determined using the Kaplan-Meier method and tested with the log-rank test. Univariate and multivariate prognostic analyses were performed using the Cox regression model. The study population comprised 90 men and 16 women (male-to-female ratio, 5.6:1). The median age was 66 (range, 41-85) years. The median follow-up period was 66.5 (range, 5-139) months, with a median survival time of 132 months. The 1-, 3-, 5-, and 10-year disease-specific survival rates were 96%, 92%, 89%, and 89%, respectively; local recurrence-free survival rates were 99%, 99%, 95%, and 88%, respectively; lymph node metastasis rates were 3%, 5%, 9%, and 9%, respectively; and distant metastasis rates were 4%, 10%, 14%, and 17%, respectively. Univariate analysis revealed that the depth of invasion of the primary lesion was associated with local recurrence, with dermal involvement increasing the risk of local recurrence. Multivariate analysis revealed that the N and M stages were associated with disease prognosis. Radiation therapy is suitable for EMPD. Post-Mohs micrographic surgery radiation therapy provides excellent local control, and radical radiation therapy effectively controls tumors, including metastases.
Diagnostic and therapeutic challenges in mammary and extra-mammary Paget disease: a retrospective case series.
Mammary and extramammary Paget disease (PD; MPD, EMPD) is a rare neoplastic disorder characterised by intraepithelial infiltration of malignant cells with glandular differentiation. Diagnosis of PD remains challenging, as its clinical presentation frequently mimics inflammatory diseases or other cutaneous neoplasms. It is estimated that approximately 10-15% of EMPD cases and up to 50% of MPD cases are invasive at the time of definitive diagnosis, largely due to diagnostic delays that may last several years. In this retrospective case series, we analysed non-pigmented EMPD (n = 8) and MPD (n = 5) to evaluate the diagnostic performance of selected non-invasive imaging techniques, including dermoscopy, photodynamic diagnosis, and reflectance confocal microscopy, highlighting their limitations and advantages during the primary investigation and disease surveillance over several years. Furthermore, we analysed diagnostic challenges in advanced, metastatic, and overlapping cases. The study also summarises the outcomes of multiple-line therapeutic approaches applied in high-risk EMPD patients and compares these results with data available in the current literature.
Advances in the application of photodynamic diagnosis in Skin Tumors.
Photodynamic Diagnosis (PDD) is a non-invasive imaging technique. It relies on a photosensitizer that, when activated by a specific light source, causes metabolically active tissues like tumors to emit visible red fluorescence. PDD offers high sensitivity, high specificity, and real-time visualization. This article reviews advances in the use of PDD for common cutaneous tumors, including Keratinocyte Carcinoma and extramammary Paget disease (EMPD). In managing skin tumors, PDD can be used during preoperative, intraoperative, and postoperative stages. This helps optimize diagnosis and treatment. Preoperatively, PDD clearly delineates tumor margins and identifies subclinical lesions. It aids in planning surgical or photodynamic therapy-especially for multifocal or ill-defined lesions, such as Bowen's disease and actinic keratosis. Intraoperatively, PDD guides surgeons in determining excision boundaries in real-time. This increases the rate of negative margins (for example, up to 98.6% in cutaneous squamous cell carcinoma surgery) while reducing the number of surgical stages and tissue damage. Postoperatively, PDD can evaluate treatment response, monitor for residual disease, or detect early recurrence. This enables non-invasive, repeatable follow-up. Limitations of PDD include false positives due to inflammation, interference from nonspecific fluorescence, limited depth of fluorescence penetration, and variability in tumor tissue. Looking forward, new technologies such as nanotechnology, artificial intelligence, and multimodal imaging may broaden the use of fluorescence detection in dermatology.
Clinicopathological Analysis of 40 Cases of Extramammary Paget Disease: A Retrospective Study.
Extramammary Paget Disease (EMPD) is a rare intra-epithelial malignancy often misdiagnosed due to diverse clinical manifestations. This study retrospectively analyzed the clinicopathological features of 40 EMPD patients to provide references for clinical practice. Clinical data of 40 patients with pathologically confirmed EMPD were collected, including demographics, clinical features, histopathological and immunohistochemical findings, treatments, and follow-up outcomes. Of the 40 patients (27 males, 13 females; mean age 68.8 years), the most common onset location was the external genitalia (n = 33). Lesions primarily presented as erythema or red plaques (n = 38), with itching being the most common symptom (n = 26). Immunohistochemically, tumor cells were positive for CK7, CEA, and EMA, with positive rates of 70% for GCDFP-15 and 25% for CK20. Thirty-nine patients underwent surgical excision, and one received photodynamic therapy combined with radiotherapy. During follow-up after surgery, one patient with invasive features (tumor mass in the dermis) recurred but achieved remission after reoperation; the other 38 patients remained disease-free. Invasive EMPD was associated with a higher risk of recurrence. EMPD's varied presentations pose diagnostic challenges. Key diagnostic differentials include eczema, psoriasis, and melanoma, particularly for rare pigmented or depigmented variants. For clinicians, a high index of suspicion and prompt biopsy are crucial for early diagnosis. For pathologists, a comprehensive immunohistochemical panel is essential, while CK20's utility in distinguishing primary from secondary disease is limited and warrants further malignancy screening. Surgical excision remains the primary treatment, with alternative therapies effective for inoperable cases. Early diagnosis, appropriate treatment, and long-term follow-up are crucial for optimal prognosis.
Management of Extramammary Paget Disease of the Male Genital Region: A Narrative Review and Two Case Reports.
Background/Objectives: Extramammary Paget disease (EMPD) is a rare, slow-growing intraepithelial malignancy arising in apocrine gland-bearing skin, most commonly in the anogenital region. Although often confined to the epidermis, EMPD may be associated with synchronous or underlying malignancies and can progress to invasive disease, making early recognition and compre- hensive staging crucial. Methods: We review current knowledge on the epidemiology, molecular pathogenesis, diagnostic work-up and treatment of EMPD with a particular focus on male genital involvement, and illustrate key clinical issues with two cases of penoscrotal EMPD treated in our center. Clinically, EMPD typically presents as chronic erythematous, pruritic plaques that are frequently misdiagnosed as benign dermatoses, leading to diagnostic delays. Results: Histopathology with immunohistochemistry remains the diagnostic gold standard and guides the search for associated internal malignancies. Wide local excision and Mohs micrographic surgery are the mainstays of treatment, but recurrence is common owing to subclinical extension. Non-surgical modalities-including photodynamic therapy, topical imiquimod, radiotherapy and, in advanced disease, systemic chemotherapy, HER2 targeted agents and immune checkpoint inhibitors-provide additional options in selected patients. Conclusions: A multidisciplinary, biomarker driven approach is essential to individualize management and improve long-term outcomes in this challenging disease.
Publicações recentes
Genomic Alterations in Perianal and Scrotal Extramammary Paget Disease: A Retrospective Case Series.
The Spaghetti Technique of Modified Mohs Surgery in Extramammary Paget Disease.
Immunohistochemical evaluation of gastric-type markers in primary extramammary Paget disease: Differential MUC5AC expression between in situ and invasive lesions.
Common Vulvar Neoplasms-Benign and Malignant.
Efficacy of Radiation Therapy in 106 Patients with Extramammary Paget Disease: A Retrospective Study.
📚 EuropePMC325 artigos no totalmostrando 198
Efficacy of Radiation Therapy in 106 Patients with Extramammary Paget Disease: A Retrospective Study.
Advances in radiation oncologyDiagnostic and therapeutic challenges in mammary and extra-mammary Paget disease: a retrospective case series.
Photodiagnosis and photodynamic therapyPain and Scrotal Involvement are Associated with Misdiagnosis in Extramammary Paget Disease: A Retrospective Cohort Study.
Journal of the American Academy of DermatologyAdvances in the application of photodynamic diagnosis in Skin Tumors.
Photodiagnosis and photodynamic therapyClinicopathological Analysis of 40 Cases of Extramammary Paget Disease: A Retrospective Study.
Clinical, cosmetic and investigational dermatologyManagement of Extramammary Paget Disease of the Male Genital Region: A Narrative Review and Two Case Reports.
Journal of clinical medicineTRPS1 Expression Across the Spectrum of Paget Disease and Morphologic Mimics.
Applied immunohistochemistry & molecular morphology : AIMMTherapeutic Algorithm for Extramammary Paget Disease: Experience in a Dermatology Referral Center in Western Mexico.
CureusThe evolving role of TRPS1 in dermatopathology: insights from the past 4 years.
Journal of pathology and translational medicinePerianal Paget's Disease From Underlying Anal Adenocarcinoma: A Case Study.
CureusIntraepithelial Penile Lesions.
Advances in anatomic pathologyExtramammary Paget Disease Secondary to Axillary Apocrine Carcinoma: Highlighting the Diagnostic Utility of a Limited Immunohistochemical Panel in a Resource-Limited Setting.
CureusProstein (p501s) is expressed in primary extramammary Paget disease.
HistopathologyReal-World Efficacy of Chemotherapy in Patients With Advanced Unresectable Extramammary Paget Disease: A Retrospective Multicenter Study of 138 Japanese Patients.
The Journal of dermatologyDiagnostic Algorithm for Secondary Extramammary Paget Disease from Institutional Cases and Literature Review.
CancersClinicopathologic features and prognostic factors of surgically treated extramammary Paget disease of the vulva: A retrospective cohort analysis.
Journal of the American Academy of DermatologyExtramammary Paget's disease: when is enough, enough?
International journal of women's dermatologyHER2 in gynecological malignancies: Bridging the gap between standardized pathological assessment and precision therapy.
Critical reviews in oncology/hematologyRisk of Associated Visceral Malignancies in Patients With Extra-anogenital Extramammary Paget Disease.
Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.]Axillary Breast Cancer Masquerading as Extramammary Paget Disease after Bilateral Total Mastectomy: A Case Report.
Case reports in dermatologySuccessful Treatment of Primary Extramammary Paget Disease with a Combined Ablative Carbon Dioxide Laser and Topical Imiquimod.
Indian journal of dermatologySerum Nectin-4 as a Potential Prognostic Biomarker for Extramammary Paget Disease.
Experimental dermatologyPerianal Intestinal-Type Paget Disease With and Without Invasion, Unassociated With Internal Malignancy: A Distinct Form of Primary Perianal Adenocarcinoma.
Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc[Vaginal and Vulval Secondary Extramammary Paget's Disease Associated with Urothelial Carcinoma in Situ : A Case Report].
Hinyokika kiyo. Acta urologica JaponicaFactors Affecting Time to Surgery in Patients With Extramammary Paget Disease in the United States.
Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.]PRAME Expression in Mammary and Extramammary Paget Disease.
The American Journal of dermatopathologyTRPS1 Reliably Stains Benign and Malignant Lesions Arising Within Vulvar Anogenital Mammary-like Glands.
International journal of gynecological pathology : official journal of the International Society of Gynecological PathologistsDermatologic Oncology: The Past 50 Years.
Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.]Pigmented Extramammary Paget's Disease of the Axilla: Two Case Reports and a Literature Review.
Clinical, cosmetic and investigational dermatologySpiny Keratoderma in Association With Extramammary Paget Disease.
Mayo Clinic proceedingsJapanese Dermatological Association Guidelines: Clinical Questions of Guidelines for Extramammary Paget Disease 2025.
The Journal of dermatologyA Case of Umbilical Invasive Extramammary Paget Disease With a High Tumor Mutational Burden Successfully Treated With Pembrolizumab.
CureusSpontaneously Regressing Penile Extramammary Paget Disease without Recurrence of Primary Urothelial Carcinoma: A Case Report.
Case reports in oncologyThe role of HER2 pathway in vulvar paget's disease.
Critical reviews in oncology/hematologyA combined inferior epigastric artery perforator propeller flap with scrotal flap for the reconstruction of a massive penoscrotal defect:A case report.
Urology case reportsTrastuzumab monotherapy as maintenance treatment for metastatic HER2+ vulvar Paget disease: systematic review and case report.
Gynecologic oncology reportsExtramammary Paget Disease in the Peristomal Skin of an Ileal Conduit Reconstructed With a Pedicled Anterolateral Thigh Flap.
Plastic and reconstructive surgery. Global openMapping the potential for anti-PD-1 therapy in advanced rare skin carcinomas.
European journal of cancer (Oxford, England : 1990)Evolution of perianal extramammary Paget disease from an erythematous patch to an achromic patch.
Italian journal of dermatology and venereologyLine-field confocal optical coherence tomography-controlled cryotherapy for extramammary Paget disease: a case report.
Clinical and experimental dermatologySecondary extramammary Paget disease with presumed origin in the external ear's ceruminous gland.
The Journal of dermatologyTransurethral resection and oral mucosal grafting for urethral-involved extramammary Paget disease: A function-preserving approach.
The Journal of dermatologyTransperineal Vulvar Ultrasound: A Review of Normal and Abnormal Findings with a Proposed Standardized Methodology.
Diagnostics (Basel, Switzerland)Clinical characteristics and prognostic factors associated with in situ extramammary Paget disease in the United States.
Journal of the American Academy of DermatologyPhotodynamic Therapy: Clinical Applications in Dermatology.
Journal of the American Academy of DermatologyPulsed dye laser-activated photodynamic therapy for extramammary Paget disease.
Clinical and experimental dermatologyCause-specific survival of mohs micrographic surgery versus wide local excision in extramammary Paget disease: a retrospective cohort study.
Archives of dermatological researchA multicenter study on TROP2 as a potential targeted therapy for extramammary Paget disease in Japan.
Scientific reportsExtramammary Paget disease.
The British journal of dermatologyPerianal Paget Disease With an Enteric Phenotype and Associated In-Situ/Dysplastic Glandular Component: Report of a Potentially Novel Entity and Review of the Literature.
International journal of surgical pathologyThe diagnostic challenges of differentiating metastatic extramammary Paget disease and prostatic adenocarcinoma: A case report and review of the literature.
Urology case reportsTislelizumab plus chemotherapy in metastatic extramammary Paget disease after surgery: a case report.
Frontiers in oncologyRepair of Scrotal Wounds after Extensive Excision of Extramammary Paget Disease with Thick Split-thickness Skin Graft.
Plastic and reconstructive surgery. Global openA Territory-Wide Follow-Up of Primary and Secondary Extramammary Paget Disease of 2 Decades: Effects of Local Disease on Survival.
Dermatology (Basel, Switzerland)Beyond the Surface: Dermoscopic, Clinical, and Histopathological Insights Into Secondary Extramammary Paget Disease of the Glans Linked to Urothelial Carcinoma.
The American Journal of dermatopathologyExtramammary Paget's disease treated with of anti-programmed cell death protein 1 therapy after docetaxel therapy failure.
The Journal of dermatologyTertiary lymphoid structures associated with improved prognosis and reduced invasion in advanced extramammary Paget disease.
Clinical and experimental dermatologyInvasive acantholytic anaplastic extramammary Paget disease: A previously unreported neoplasm in the vulva and review of the literature.
Gynecologic oncology reportsGenomics predicting prognosis in metastatic extramammary Paget disease.
The British journal of dermatologyImpact of lesion location and pattern on the prognosis of genital extramammary Paget disease: a retrospective study.
The British journal of dermatologyMultiple Moat Modification of Mohs Micrographic Surgery for the Treatment of Anogenital Extramammary Paget Disease: Challenges and Considerations.
Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.]Simultaneous Occurrence of Two Ectopic Extramammary Paget Disease Lesions: A Case Report.
CureusAxillary extramammary Paget disease in a patient with a history of Birt-Hogg-Dubé syndrome.
JAAD internationalUtility of special AT-rich sequence-binding protein 2 (SATB2) immunohistochemistry as a marker for secondary perianal paget disease.
Virchows Archiv : an international journal of pathologyAssociation of genetic alterations with prognosis in extramammary Paget disease: insights into the involvement of somatic CDKN2A variants in patients with a poor prognosis.
The British journal of dermatologyThe Role of Line-Field Confocal Optical Coherence Tomography in Detecting Extramammary Paget Disease Recurrences: A Pilot Diagnostic Study.
Diagnostics (Basel, Switzerland)Case report: Pyrotinib in the treatment of advanced scrotal EMPD combined with sweat gland carcinoma.
Frontiers in oncologyEstablishment of a trastuzumab-resistant extramammary Paget disease model: loss of PTEN as a potential mechanism.
British journal of cancerMultiple-site primary extramammary Paget disease in a 77-year-old man.
CMAJ : Canadian Medical Association journal = journal de l'Association medicale canadienneScrotal Cancer Incidence Rates and Trends-United States, 1999-2020.
UrologyPedunculated Neoplasm of the Vulva.
The American Journal of dermatopathologySex disparities in surgery for extramammary Paget disease: a SEER population-based study.
International journal of dermatologyUnusual case of extramammary Paget disease of the oral mucosa: A case report and literature review.
Journal of cutaneous pathologyPreoperative Mapping Biopsies with Local Excision in the Treatment of Extramammary Paget Disease: A Retrospective Cohort Study, Systematic Review, and Individual Patient Data Analysis.
Dermatology (Basel, Switzerland)Assessment of HER2 status in extramammary Paget disease and its implication for disitamab vedotin, a novel humanized anti-HER2 antibody-drug conjugate therapy.
Frontiers of medicineExtramammary Paget disease. Part I. epidemiology, pathogenesis, clinical features, and diagnosis.
Journal of the American Academy of DermatologyEsophageal squamous cell carcinoma with pagetoid spread: a clinicopathologic study.
Virchows Archiv : an international journal of pathologyExtramammary Paget Disease of Oral Mucosa: Case Report with Literature Review.
Head and neck pathologyLoss of p16 Immunoexpression and Deletions of CDKN2A in the Progression of Extramammary Paget Disease: An Immunohistochemical and Genetic Study of 24 Invasive/Metastatic Cases.
The American Journal of dermatopathologyA rare case of metastatic extramammary Paget disease developing transfusion-related acute lung injury during chemotherapy.
The Journal of dermatologyExtramammary Paget disease. Part II. Evidence-based approach to management.
Journal of the American Academy of DermatologyCase report: A case of Paget disease outside the axillary breast.
MedicineTreatment of extramammary Paget disease with imiquimod in a real-life setting: a multicentre retrospective analysis in Spain.
Clinical and experimental dermatologyPaget's disease of the breast: Insights from imaging to guide surgical management.
American journal of surgeryNECTIN4-targeted antibody-drug conjugate is a potential therapeutic option for extramammary Paget disease.
Experimental dermatologyIdentification of a rare MET variant in three siblings with extramammary Paget disease.
Clinical and experimental dermatologyAnatomic Subtype Differences in Extramammary Paget Disease: A Meta-Analysis.
JAMA dermatologyPerianal extramammary Paget disease: our experience of two cases - a video vignette.
Colorectal disease : the official journal of the Association of Coloproctology of Great Britain and IrelandTRPS1 expression in non-melanocytic cutaneous neoplasms: an immunohistochemical analysis of 200 cases.
Journal of pathology and translational medicineFOXM1: a new therapeutic target of extramammary Paget disease.
Scientific reportsIncidence and survival rates of primary cutaneous malignancies in Korea, 1999-2019: A nationwide population-based study.
The Journal of dermatologyEribulin for patients with metastatic extramammary Paget disease: Study protocol for a single-arm phase II trial.
Experimental dermatologyA Review of the Dermatologic Clinical Applications of Topical Photodynamic Therapy.
Journal of cutaneous medicine and surgeryThe elusive extremities: a case of extramammary Paget disease.
BMJ case reportsA rare case of extramammary Paget disease in a young HIV-positive man.
JAAD case reportsImmediate Lymphatic Reconstruction Using a Handheld Fluorescence Imaging Device.
Plastic and reconstructive surgery. Global openMutational landscape of advanced extramammary Paget disease with comprehensive genomic profiling tests: A cohort study in a Japanese real-world setting.
Journal of the American Academy of DermatologyTopical Imiquimod in Primary Cutaneous Extramammary Paget's Disease: A Systematic Review.
CancersPathologically Confirmed Subcutaneous Fat and Lymphovascular Invasion of a Hypopigmented Area in Extramammary Paget Disease.
Annals of dermatologyUltrasonography for the Diagnosis and Follow-Up of Extramammary Paget Disease (EMPD): A Review.
Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in MedicineImpact of combining photodynamic diagnosis with reflectance confocal microscopy, on tumor margin detection and surgical outcomes in patients with extramammary Paget disease.
Indian journal of dermatology, venereology and leprologyA case of male Paget's disease of the breast.
Asian journal of surgeryTRPS1 expression in primary and secondary extramammary Paget diseases: An immunohistochemical analysis of 93 cases.
Human pathologyPhotodynamic Therapy for Primary Cutaneous Extramammary Paget Disease.
Dermatology practical & conceptualIdentification of Predictive Factors for Post-operative Recurrence and Clinical Outcomes of Primary Vulvar Extramammary Paget Disease.
In vivo (Athens, Greece)Hypopigmentation as a diagnostic clue in primary extramammary Paget disease: Case report and short literature review.
The Australasian journal of dermatologyScrotal Wall Metastasis from Adenocarcinoma of Unknown Origin, with Concurrent Extramammary Paget's Disease - a Case Report.
The Gulf journal of oncologyCommentary on "Reconstruction of a Large Extramammary Paget Disease Pelvic Defect".
Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.]Reconstruction of a Large Extramammary Paget Disease Pelvic Defect.
Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.]Transcriptional repressor GATA binding 1 (TRPS1) immunoexpression in normal skin tissues and various cutaneous tumors.
Journal of cutaneous pathologyElevated Risk of Visceral Malignant Neoplasms in Extramammary Paget Disease.
JAMA dermatologyPerianal Malignancies: A Review for the Dermatologic Surgeon.
Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.]Clinicopathological significance of androgen receptor expression in extramammary Paget disease: An analysis of 92 patients.
Pathology, research and practiceEXTRA, EXTRA, Treatment Approaches for EXTRAmammary Paget Disease.
Journal of drugs in dermatology : JDDA Simplified Algorithmic Approach to Vulvar Reconstruction According to Various Types of Vulvar Defects.
Annals of plastic surgeryTreatment of high tumor mutation burden metastatic extramammary Paget disease with an anti-PD-1 antibody.
The Journal of dermatologyExtramammary Paget Disease: a Therapeutic Challenge, for a Rare Entity.
Current oncology reportsPerianal extramammary Paget's disease with adenocarcinoma of perianal skin area, a case report.
Journal of surgical case reportsClaudin-4 Expression in the Distinction of Mammary and Extramammary Paget Disease From Morphologic Mimics: Diagnostic Utility and Pitfalls.
The American Journal of dermatopathologyThe role of sentinel lymph node biopsy in extramammary paget disease: a systematic review.
Archives of dermatological researchPrognostic Analysis of Patients With Extramammary Paget Disease Treated With Conservative Excision.
Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.]The utility of p63, CK7, and CAM5.2 staining in differentiating pagetoid intraepidermal carcinomas.
Journal of cutaneous pathologyEfficacy of chemotherapies for unresectable extramammary Paget disease: a single-centre retrospective study.
Clinical and experimental dermatologyPatient's Experience With Imiquimod for Extramammary Paget Disease.
Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.]Recurrent primary scrotal extramammary Paget disease: a case report and literature review.
Annals of the Royal College of Surgeons of EnglandExtramammary Paget disease of the buccal mucosa in an elderly patient.
GerodontologyTreatment Outcomes of Adjunct and Stand-alone Photodynamic Therapy in Patients With Extramammary Paget Disease: A Literature Review.
Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.]National screening practices in vulval extramammary Paget disease: a multicentre UK audit.
Clinical and experimental dermatologyTRPS1 expression is sensitive and specific for primary extramammary Paget disease.
HistopathologyEroded pigmented anogenital plaque in an elderly woman.
JAAD case reportsExtramammary Paget disease imaged by LC-OCT and treated with radiotherapy.
International journal of dermatologyPagetoid Spread in Basal Cell Carcinoma: Potential for Misdiagnosis.
The American Journal of dermatopathologyA retrospective case series of Mohs micrographic surgery and interdisciplinary management of female genital skin cancers: Local recurrence rates and patient-reported outcomes.
Journal of the American Academy of DermatologyHealth-related quality of life and associated factors among non-melanoma skin cancer patients: a cross-sectional study.
Annals of translational medicine'Get Data Out' Skin: national cancer registry incidence and survival rates for all registered skin tumour groups for 2013-2019 in England.
The British journal of dermatologyImmunohistochemical expression of TRPS1 in mammary Paget disease, extramammary Paget disease, and their close histopathologic mimics.
Journal of cutaneous pathologyClinical features of pagetoid spread: Analysis of perianal lesions associated with anal canal adenocarcinoma and perianal primary extramammary Paget disease.
The Journal of dermatologyResearch letter: A new fluorescent three-dimensional and deep-imaging technique for histological identification of individual tumor cells in extramammary Paget's disease.
Experimental dermatologyA Rare Collison of Vulvar Melanoma and Extramammary Paget Disease: Case Report and Review of the Literature.
The American Journal of dermatopathologyExtramammary paget disease of the penoscrotal region.
Pathology, research and practiceLichenoid lesions around the anus: An unusual site of Hailey-Hailey disease.
Clinical case reportsPagetoid Squamous Intraepithelial Neoplasia of the Vulva as a Mimicker of Vulvar Extramammary Paget Disease: Two Cases with Basal Layer Sparing.
International journal of surgical pathologyCutaneous Apocrine Adenocarcinoma of the Groin With Extramammary Paget Disease: Response to Dual HER2 Blockade With Trastuzumab and Pertuzumab.
Actas dermo-sifiliograficasSentinel lymph node biopsy in extramammary Paget disease: A 13-year institutional experience.
The Journal of dermatologyLocal Recurrence Rates of Extramammary Paget Disease Are Lower After Mohs Micrographic Surgery Compared With Wide Local Excision: A Systematic Review and Meta-Analysis.
Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.]Reconstruction of the male external genitalia in diverse disease processes: Our reconstructive algorithm, techniques, and experience.
Current urologyCell origin and genome profile difference of penoscrotum invasive extramammary Paget disease compared with its in situ counterpart.
Frontiers in oncologyPagetoid Dyskeratosis of the Hand With Parallel Ridge Pattern on Dermoscopy and Expression of Bcl-10 by Immunohistochemistry.
The American Journal of dermatopathologyCase Report: Prolonged clinical benefit with sequential trastuzumab-containing treatments in a patient with advanced extramammary Paget disease of the groin.
Frontiers in oncologyPerianal Paget disease associated with non-invasive colorectal adenoma.
Journal of cutaneous pathologyCompound Clear Cell Sarcoma: A Case Report With Ulceration and Pagetoid Scatter.
The American Journal of dermatopathologyNeutrophil-to-lymphocyte ratio is a potential prognostic biomarker for extramammary Paget disease: A retrospective study.
The Journal of dermatologyThe European Society of Gynaecological Oncology (ESGO), the International Society for the Study of Vulvovaginal Disease (ISSVD), the European College for the Study of Vulval Disease (ECSVD) and the European Federation for Colposcopy (EFC) Consensus Statements on Pre-invasive Vulvar Lesions.
Journal of lower genital tract diseaseTreatment of Metastatic Primary Extramammary Paget Disease With Combination Anlotinib and Tislelizumab: A Case Report and Review of the Literature.
Frontiers in medicineExtramammary Paget disease of the axilla and scrotum in a Caucasian man.
JAAD case reportsFluorescence kinetics study of twice laser irradiation based HpD-PDT for nonmelanoma skin cancer.
Lasers in surgery and medicineInvasive and non-invasive extramammary perianal Paget's disease: Understanding of carcinogenesis determines individualized treatment.
Cirugia espanolaAssessment of Extramammary Paget Disease by Two-Photon Microscopy.
Frontiers in medicinePenile extramammary Paget disease associated with urothelial carcinoma in situ: Case report and literature review.
Journal of cutaneous pathologyCK 8∕18: the key to differentiating intracutaneous lesions with pagetoid features.
Romanian journal of morphology and embryology = Revue roumaine de morphologie et embryologieBoron neutron capture therapy for urological cancers.
International journal of urology : official journal of the Japanese Urological AssociationDepigmentation of the vulvar area: Is it an alarming sign?
Clinical case reports"Clock mapping" prior to excisional surgery in vulvar Paget's disease: tailoring the surgical plan.
Archives of gynecology and obstetricsEvidence-Based Clinical Practice Guidelines for Extramammary Paget Disease.
JAMA oncologyLaparoscopic pelvic surgery after reconstructive surgery with vertical rectus abdominis muscle flap: A case report.
International journal of surgery case reportsUnusual lesion on the vulva of a postmenopausal woman.
JAAD case reportsReflectance Confocal Microscopy-Targeted Extramammary Paget Disease Before Mohs Micrographic Surgery.
Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.]Extramammary Paget's Disease in the Genital Area of a Male: A Case Report and Review of the Literature.
Frontiers in oncologyTreatment-refractory extramammary Paget disease responsive to radiotherapy.
American journal of obstetrics and gynecologyPoorly Differentiated Scrotal Carcinoma With Apocrine Immunophenotype.
The American Journal of dermatopathologyVulvar Extramammary Paget Disease Detected by Cytology for Cervical Cancer Screening: A Case Report and Literature Review.
The American journal of case reportsUnusual Manifestations of Vulval Paget Disease.
International journal of gynecological pathology : official journal of the International Society of Gynecological PathologistsGastrointestinal: Extramammary Paget disease of the esophagus.
Journal of gastroenterology and hepatologySuccessful treatment of a case of hormone receptor-positive metastatic extramammary Paget disease with tamoxifen.
Investigational new drugsAxillary Paget disease with a visible satellite: a case report and literature review.
Diagnostic pathologyParallel ridge pattern in palmar pagetoid dyskeratosis: a dermoscopic pitfall.
International journal of dermatologyPerianal Paget Disease: Different Entities With the Same Name.
CureusExtramammary Paget disease: five perianal case report and treatment options.
Journal of surgical case reportsIntradermal Band-like Lipomatous Metaplasia Can Be Associated With the Regression of Overlying Skin Neoplasms: A Clinicopathologic Study of 20 Cases.
The American Journal of dermatopathologyVulvar pagetoid urothelial intraepithelial neoplasia: a case report.
Acta chirurgica BelgicaPagetoid dyskeratosis in dermatopathology.
Anais brasileiros de dermatologiaAnogenital syringocystadenocarcinoma papilliferum in situ or anaplastic extramammary Paget disease? A unifying concept and review of the literature.
Journal of cutaneous pathologyIn vivo identification of tumor cells of the basal layer of the epidermis in an early lesion of extramammary Paget disease: A reflectance confocal microscopic analysis.
JAAD case reportsEctopic extramammary Paget disease in thoracic location.
Dermatology online journalHigh-Frequency Ultrasound for Evaluation of the Pathological Invasion Level of Extramammary Paget Disease.
Journal of ultrasound in medicine : official journal of the American Institute of Ultrasound in MedicineSurvival analysis of extramammary Paget's disease (EMPD) in a tertiary hospital in Taiwan.
World journal of surgical oncologyAberrant Melan-A Expression in Extramammary Paget Disease.
The American Journal of dermatopathologyTreatment of Metastatic Extramammary Paget Disease with Combination Ipilimumab and Nivolumab: A Case Report.
Case reports in oncologyUtility of photodynamic diagnosis plus reflectance confocal microscopy in detecting the margins of extramammary Paget disease.
Indian journal of dermatology, venereology and leprologyUsefulness of Topical Imiquimod 3.75% in Cytokeratin 7 Positive Extramammary Paget Disease of the Vulva: Towards Personalized Therapy.
Dermatology practical & conceptualTreatment of Extramammary Paget Disease and the Role of Reflectance Confocal Microscopy: A Prospective Study.
Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.]68Ga-FAPI and 18F-FDG PET/CT in Perineum Extramammary Paget Disease.
Clinical nuclear medicineLongitudinal multicentre retrospective cohort study of treatment outcomes in extramammary Paget disease.
The British journal of dermatologyVulvar Intraepithelial Neoplasia: A Review of the Disease and Current Management.
Obstetrical & gynecological surveyNeutrophil/lymphocyte ratio as a predictor of lymph node metastasis in extramammary Paget disease: A retrospective study.
Journal of the American Academy of DermatologyReflectance Confocal Microscopy as a Promising Adjunctive Tool for Treatment Planning in Extramammary Paget's Disease.
Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.]Hypopigmentation in Extramammary Paget Disease Is an Important Prognostic Factor for High Recurrence Rate and Poor Surgical Outcome.
Dermatologic surgery : official publication for American Society for Dermatologic Surgery [et al.][Extra Mammary Paget disease].
Annales de pathologieAssociações
Organizações que acompanham esta doença — pra ter apoio e orientação
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Doença com base genética
Um médico geneticista pode ajudar no diagnóstico de Doença de Paget extramamária e no aconselhamento genético da família.
Doenças relacionadas
Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico
Perguntas frequentes
O que as famílias mais perguntam sobre esta doença — cada resposta com a fonte de onde saiu
Existe um documento oficial para "Doença de Paget". Ele pode não cobrir esta forma específica da doença — confira no texto do protocolo antes de contar com ele.
É uma neoplasia maligna da pele com infiltração de grandes células de Paget com citoplasma abundante e pálido. Ela surge tipicamente em adultos e idosos, afetando áreas como o ânus, o pênis, o escroto e a vulva.
Referências
Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete
9 publicações do grafo RarasNet (PubMed) · 7 bases de dados. Títulos, periódicos e PMIDs vêm direto da fonte, sem intermediação de IA.
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Citar este verbete
Raras. (s.d.). Doença de Paget extramamária. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/doenca-de-paget-extramamaria
Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Doença de Paget extramamária
📋 Origem dos dados
Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.
- Doença rara (ontologia)
- fonte: Orphanet
- Identificador unificado
- fonte: MONDO
- Genética mendeliana
- fonte: OMIM
- Codificação WHO/SUS
- fonte: WHO ICD-10 / DATASUS
- CID-11 (futuro)
- fonte: WHO ICD-11
- NIH/GARD
- fonte: GARD (NIH)
- Indexação biomédica
- fonte: MeSH (NLM)
- Dado público estruturado
- fonte: Wikidata
- Ensaios clínicos
- fonte: ClinicalTrials.gov