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1.
Medicine (Baltimore) ; 100(5): e24485, 2021 Feb 05.
Artigo em Inglês | MEDLINE | ID: mdl-33592901

RESUMO

ABSTRACT: Paget disease is a complex disorder that can be identified in the breast (mammary Paget disease) or in other locations (extramammary Paget's disease) such as ano-genital skin (Paget disease of the vulva -PVD). This condition is associated with low mortality, but a late diagnosis and recurrence can negatively impact the prognosis. Therefore, the main objective of this study is to evaluate if the human epididymis protein 4 (HE4) and cancer antigen125 (CA125) can promote recognition of PVD in early stages and during the relapses.we have conducted a prospective, observational and laboratory-based study, that included 50 patients, whose 25 healthy women represented the control group and 25 PVD patients, which have been operated in our Oncology Institute, from May 2017 to September 2019. Both in the control group and in PVD patients, the CA-125 and HE4 were evaluated before surgery and after 6 months. Finally, a comparison of markers serum level, both between before/after surgery and with control group, and a ROC (Receiver Operating Characteristic) curve were performed.Dosing the markers in PVD patients, 3/25 (12%) showed a higher value of CA125 and 11/25 (44%) an increased HE4. In addition, after surgical treatment there were no statistically significant difference between levels of CA-125 (P = .3) and HE4 (P = .19). On the other hand, comparing HE4 in PVD patients with the control group, a statistically significant difference was found (P-value = .0036). Contrary, comparing CA-125 in PVD patients with the control group (P-value= .1969), no statistically significant difference was evidenced. Moreover, ROC (Receiver Operating Characteristic) curve showed low sensitivity and specificity for CA125 with area under curve (AUC) = 0.5608. Instead, the ROC curve of HE4 revealed a sensitivity and specificity of 76% and 88% respectively (AUC = 0.7408) using a cut-off at 90 pmol/L.Despite the limited cases, our data showed that CA125 is not a sensitive marker for PVD. On the other hand, in 44% of PVD we've seen an increase in HE4. So, this could be a starting point for further research that could confirm the possibility to use this marker in order to support PVD early identification.


Assuntos
Antígeno Ca-125/sangue , Doença de Paget Extramamária/diagnóstico , Doença de Paget Extramamária/patologia , Proteína 2 do Domínio Central WAP de Quatro Dissulfetos/análise , Idoso , Idoso de 80 Anos ou mais , Biomarcadores , Feminino , Humanos , Pessoa de Meia-Idade , Estudos Prospectivos , Curva ROC , Doenças da Vulva
2.
BMJ Case Rep ; 14(2)2021 Feb 04.
Artigo em Inglês | MEDLINE | ID: mdl-33541959

RESUMO

Perianal Paget disease (PPD) is a rare neoplastic condition defined by the presence of atypical Paget cells in the perianal skin, the aetiology of which remains largely unknown. It can be divided in primary forms, arising as an intraepithelial disease or manifestation of an underlying skin adenocarcinoma or secondary forms resulting from epidermotropic spread or metastasis of a concealed carcinoma. Indeed, because of its rarity, clear options regarding the treatment of these patients are yet to be clarified. A high level of suspicion is needed whenever dealing with any unhealed perianal skin lesions and, therefore, the need for close long-term follow-up must be highlighted. Herein, two cases of PPD, one primary and another secondary, treated at the same institution, are presented in an attempt to document the involved complexity and to bring further insight into the understanding of this entity.


Assuntos
Adenocarcinoma/patologia , Doença de Paget Extramamária/patologia , Neoplasias Cutâneas/patologia , Idoso , Canal Anal/patologia , Neoplasias Ósseas/patologia , Feminino , Humanos , Masculino , Neoplasias de Tecido Conjuntivo/patologia , Doença de Paget Extramamária/terapia , Períneo/patologia
3.
Anticancer Res ; 41(1): 219-226, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-33419816

RESUMO

BACKGROUND: Extramammary Paget's disease (EMPD) is a type of carcinoma that usually progresses slowly but may cause metastasis and subsequent death of patients. We investigated the relationship between the expression of programmed death-ligand 1 (PD-L1)/programmed death-ligand 2 (PD-L2) and stromal CD8+ tumor-infiltrating lymphocytes (TILs) in EMPD and clinicopathological findings, including prognosis. MATERIALS AND METHODS: We examined 47 cases of EMPD and performed immunohistochemical staining of formalin-fixed paraffin-embedded full-face sections. RESULTS: PD-L1 expression in tumor cells was observed in 13 cases (27.7%) while PD-L2 expression was observed in 21 cases (44.7%). The cumulative postoperative recurrence-free rate in the group with positivity for PD-L1 and/or PD-L2 with a low CD8+ TIL count was significantly lower than that of the corresponding group with a high CD8+ TIL count and of the PD-L1- and PD-L2-negative group (p=0.026). CONCLUSION: The expression of PD-L1/PD-L2 in tumor cells was shown to be a factor for poor prognosis.


Assuntos
Antígeno B7-H1/genética , Biomarcadores Tumorais , Expressão Gênica , Doença de Paget Extramamária/genética , Doença de Paget Extramamária/mortalidade , Proteína 2 Ligante de Morte Celular Programada 1/genética , Idoso , Idoso de 80 Anos ou mais , Antígeno B7-H1/metabolismo , Linfócitos T CD8-Positivos/imunologia , Linfócitos T CD8-Positivos/metabolismo , Linfócitos T CD8-Positivos/patologia , Feminino , Humanos , Imuno-Histoquímica , Estimativa de Kaplan-Meier , Linfócitos do Interstício Tumoral/imunologia , Linfócitos do Interstício Tumoral/metabolismo , Linfócitos do Interstício Tumoral/patologia , Masculino , Pessoa de Meia-Idade , Doença de Paget Extramamária/imunologia , Doença de Paget Extramamária/patologia , Prognóstico , Proteína 2 Ligante de Morte Celular Programada 1/metabolismo
4.
Medicine (Baltimore) ; 99(51): e23443, 2020 Dec 18.
Artigo em Inglês | MEDLINE | ID: mdl-33371071

RESUMO

BACKGOUND: This study aims to identify the expression of lipoma preferred partner (LPP) in Paget disease (PD) and to further understand the pathogenesis of PD. METHODS: Tissue microarray was used to evaluate the expression of LPP by immunohistochemistry in 40 PD patients. The results of LPP expression were combined with clinical and histopathological characteristics. Patient files were analyzed retrospectively. RESULTS: Twenty-one cases were mammary Paget disease (MPD) and 19 extramammary Paget disease (EMPD) involving the vulva, scrotum, and penis. LPP was expressed in PD and this expression was significantly greater in MPD versus EMPD (P = .031). The expression of LPP in MPD was significantly related with age (P = .009) and expression of Ki-67 (P = .011). No statistically significant differences were observed in LPP expression as related to sex, body location, and time of PD diagnosis. CONCLUSIONS: While LPP is expressed in both MPD and EMPD, the intensity of this expression is greater in MPD. LPP expression is positively correlated with Ki-67 and is more prevalent in middle-aged versus senior MPD patients. Further research is needed to determine its potential role in tumorigenesis and distribution.


Assuntos
Proteínas do Citoesqueleto/biossíntese , Proteínas com Domínio LIM/biossíntese , Neoplasias/patologia , Doença de Paget Extramamária/patologia , Doença de Paget Mamária/patologia , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Neoplasias da Mama/patologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias Penianas/patologia , Estudos Retrospectivos , Neoplasias Testiculares/patologia , Análise Serial de Tecidos , Neoplasias Vulvares/patologia
5.
Int J Mol Sci ; 22(1)2020 Dec 27.
Artigo em Inglês | MEDLINE | ID: mdl-33375467

RESUMO

Vulvar cancer (VC) is a rare neoplasm, usually arising in postmenopausal women, although human papilloma virus (HPV)-associated VC usually develop in younger women. Incidences of VCs are rising in many countries. Surgery is the cornerstone of early-stage VC management, whereas therapies for advanced VC are multimodal and not standardized, combining chemotherapy and radiotherapy to avoid exenterative surgery. Randomized controlled trials (RCTs) are scarce due to the rarity of the disease and prognosis has not improved. Hence, new therapies are needed to improve the outcomes of these patients. In recent years, improved knowledge regarding the crosstalk between neoplastic and tumor cells has allowed researchers to develop a novel therapeutic approach exploiting these molecular interactions. Both the innate and adaptive immune systems play a key role in anti-tumor immunesurveillance. Immune checkpoint inhibitors (ICIs) have demonstrated efficacy in multiple tumor types, improving survival rates and disease outcomes. In some gynecologic cancers (e.g., cervical cancer), many studies are showing promising results and a growing interest is emerging about the potential use of ICIs in VC. The aim of this manuscript is to summarize the latest developments in the field of VC immunoncology, to present the role of state-of-the-art ICIs in VC management and to discuss new potential immunotherapeutic approaches.


Assuntos
Carcinoma de Células Escamosas/imunologia , Imiquimode/farmacologia , Imunoterapia/métodos , Melanoma/imunologia , Tumores Neuroendócrinos/imunologia , Doença de Paget Extramamária/imunologia , Neoplasias Vulvares/imunologia , Adjuvantes Imunológicos/farmacologia , Adjuvantes Imunológicos/uso terapêutico , Alphapapillomavirus/imunologia , Antineoplásicos/farmacologia , Antineoplásicos/uso terapêutico , Carcinoma de Células Escamosas/tratamento farmacológico , Carcinoma de Células Escamosas/patologia , Feminino , Humanos , Imiquimode/uso terapêutico , Melanoma/tratamento farmacológico , Melanoma/patologia , Tumores Neuroendócrinos/tratamento farmacológico , Tumores Neuroendócrinos/patologia , Doença de Paget Extramamária/tratamento farmacológico , Doença de Paget Extramamária/patologia , Prognóstico , Neoplasias Vulvares/tratamento farmacológico , Neoplasias Vulvares/patologia , Neoplasias Vulvares/virologia
6.
Oncogene ; 39(36): 5867-5875, 2020 09.
Artigo em Inglês | MEDLINE | ID: mdl-32724160

RESUMO

Although the prognosis of advanced extramammary Paget's disease (EMPD) is poor, there have been no preclinical research models for the development of novel therapeutics. This study aims to establish a preclinical research model for EMPD. We transplanted EMPD tissue into immunodeficient NOD/Scid mice. Histopathological and genetic analyses using a comprehensive cancer panel were performed. For in vivo preclinical treatments, trastuzumab, lapatinib, docetaxel, or eribulin were administered to patient-derived xenograft (PDX) models. Tissue transplanted from the EMPD patient was enlarged in NOD/Scid mice and was transplanted into further generations. Both the transplantation of PDX into nu/nu mice and the reanimation of the cryopreserved xenografted tumors in NOD/Scid mice were successful. We also established an EMPD-PDX-derived primary cell culture. Histopathologically, the xenografted tumors were positive for CK7, which was consistent with the patient's tumors. Genetically, the pathogenic mutation ERBB2 S310F was detected in the patient's tumors (primary intraepidermal lesion, metastatic lymph node) and was observed in the xenografted tumors even after continued passages. The xenografted tumors responded well to trastuzumab and lapatinib therapy. Also, cytotoxic agents (docetaxel and eribulin) were effective against the xenografted tumors. This PDX model (EMPD-PDX-H1) could be a powerful tool for the research and development of EMPD treatments.


Assuntos
Substituição de Aminoácidos , Mutação , Doença de Paget Extramamária/genética , Doença de Paget Extramamária/patologia , Receptor ErbB-2/genética , Idoso , Animais , Antineoplásicos Imunológicos/farmacologia , Linhagem Celular Tumoral , Análise Mutacional de DNA , Modelos Animais de Doenças , Feminino , Humanos , Camundongos , Terapia de Alvo Molecular , Doença de Paget Extramamária/tratamento farmacológico , Receptor ErbB-2/antagonistas & inibidores , Ensaios Antitumorais Modelo de Xenoenxerto
7.
Diagn Pathol ; 15(1): 29, 2020 Mar 24.
Artigo em Inglês | MEDLINE | ID: mdl-32209119

RESUMO

BACKGROUND: Perianal Paget's disease (PPD) is rare and mostly described in clinical literature as case reports or small series. METHODS: We investigated the clinicopathologic and immunohistochemical features of PPD in a total of 13 cases retrieved from multiple academic institutions. RESULTS: The median age at diagnosis was 75 (range 50-86) years. Males were predominant with a male to female ratio of 2.25:1. Four (30.8%) cases were classified as primary PPD due to lack of synchronous or metachronous underlying malignancies, while nine (69.2%) were classified as secondary PPD with concurrent invasive adenocarcinoma (n = 8) or tubular adenoma with high-grade dysplasia (n = 1). Immunohistochemically, there is no differential expression of CK7 or CK20 in Paget's cells between primary and secondary PPD; however, GCDFP-15 was only positive in primary PPD (3/3 vs. 0/6, P = 0.012), while CDX2 was only positive in secondary PPD (0/3 vs. 7/7, P = 0.008), suggesting different cell origin. All patients received local surgical resection with or without adjuvant therapy. After a median follow-up of 47 months, one patient with secondary PPD (7.7%) died of disease progression from underlying adenocarcinoma. CONCLUSIONS: PPD occurs in elderly patients with male predominance and is frequently associated with underlying malignancies. Differential expression of CDX2 and GCDFP-15 may help distinguishing primary vs. secondary PPD, which is important for management as the presence of an underlying malignancy impacts clinical course and prognosis. Surgical excision remains the major treatment strategy for PPD. Long-term follow-up is required to monitor the disease recurrence and metastasis.


Assuntos
Neoplasias do Ânus/patologia , Doença de Paget Extramamária/patologia , Neoplasias Cutâneas/patologia , Idoso , Idoso de 80 Anos ou mais , Biomarcadores Tumorais/análise , Feminino , Humanos , Imuno-Histoquímica , Masculino , Pessoa de Meia-Idade , Neoplasias Primárias Múltiplas/patologia
9.
J Invest Dermatol ; 140(3): 583-592, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-31513804

RESUMO

The molecular weight of methotrexate (MTX) makes cutaneous penetration difficult. Oxygen flow could enhance the skin permeation of MTX diluted in the proprietary LP3 carrier system. This pilot study aims to assess the efficacy, safety, and tolerance of oxygen flow-assisted LP3-MTX3% for treating superficial skin cancers. Patients with superficial basal cell carcinoma (n = 12), extramammary Paget disease (n = 5), classic mycosis fungoides (MF; n = 10), and folliculotropic MF (n = 6) were included in the study and were treated with four weekly applications of oxygen flow-assisted LP3-MTX3%. Photographs and biopsies were performed before and one month after treatment. At one month after treatment, the mean superficial basal cell carcinoma erythema-crusting-thickness clinical score, the extramammary Paget disease erythema-oozing-scaling/hyperkeratosis-pain/pruritus clinical score, and the modified composite assessment of index lesion severity classic MF and folliculotropic MF scores were improved by 77.5% ± 17.1% (P < 0.0001), 66.7% ± 22.9% (P = 0.011), 51.3% ± 32.2% (P = 0.0007), and 27.8% ± 32.0% (P = 0.086), respectively. At one month after treatment, histology revealed partial and total clearances for superficial basal cell carcinoma (1/12, 11/12), extramammary Paget disease (4/5, 1/5), classic MF (8/10, 2/10), and folliculotropic MF (6/6, 0/6). Tolerance was excellent and no pain was observed. MTX was never detectable in serum at baseline and 1, 2, 3, 8, 24, 48, and 72 hours post-treatment. In conclusion, the interesting therapeutic efficacy of oxygen flow-assisted LP3-MTX3% for treating superficial basal cell carcinoma, extramammary Paget disease, and MF lesions prompts further studies on a larger scale.


Assuntos
Carcinoma Basocelular/tratamento farmacológico , Portadores de Fármacos/química , Metotrexato/administração & dosagem , Micose Fungoide/tratamento farmacológico , Oxigênio/administração & dosagem , Doença de Paget Extramamária/tratamento farmacológico , Neoplasias Cutâneas/tratamento farmacológico , Administração Cutânea , Idoso , Idoso de 80 Anos ou mais , Biópsia , Carcinoma Basocelular/diagnóstico , Carcinoma Basocelular/patologia , Feminino , Humanos , Masculino , Metotrexato/efeitos adversos , Metotrexato/farmacocinética , Pessoa de Meia-Idade , Micose Fungoide/diagnóstico , Micose Fungoide/patologia , Doença de Paget Extramamária/diagnóstico , Doença de Paget Extramamária/patologia , Permeabilidade , Projetos Piloto , Índice de Gravidade de Doença , Pele/metabolismo , Pele/patologia , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/patologia , Distribuição Tecidual , Resultado do Tratamento
10.
Gynecol Oncol ; 157(1): 146-150, 2020 04.
Artigo em Inglês | MEDLINE | ID: mdl-31780234

RESUMO

OBJECTIVE: To evaluate the clinicopathological characteristics, management and prognosis of patients with vulvar extramammary Paget disease of the vulva (EMPD). MATERIALS AND METHODS: The U.S National Cancer Database was accessed and patients diagnosed between 2004 and 2015 with microscopically confirmed vulvar EMPD were selected. Overall survival (OS) was calculated for patients diagnosed between 2004 and 2014, who had at least one month of follow-up. Five year OS rates were calculated following generation of Kaplan-Meier curves while comparisons were made with the log-rank test. RESULTS: A total of 2602 patients were identified. Median age at diagnosis was 72 years (range 31-90 years) and the majority were of White race (92%), without any co-morbidities (80.9%). Personal history of another tumor was present in 36.9% of patients. In situ EMPD was diagnosed in 994 cases (38.2%) and the majority (95.1%) were managed with local excision or vulvectomy. Five-year OS was 85.8%, while presence of positive margins was not associated with worse OS (p = 0.38). Invasive EMPD was diagnosed in 1608 (61.8%) patients. Staging information was available for 1172 patients, 75.3% had early stage disease. Most patients underwent surgical treatment (91.6%); 53.6% had positive margins. Performance of lymphadenectomy was infrequent (6.8%). Moreover, immunotherapy (4.5%), chemotherapy (1.5%) and radiation therapy (2.2%) were rarely employed in the management of invasive EMPD. Patients with early stage disease (n = 766) had better OS compared to those with advanced stage (n = 278) (5-yr OS rates were 84.3% and 73.6% respectively, p = 0.015) while presence of positive margins was not associated with worse OS (p = 0.35). CONCLUSIONS: Extramammary Paget disease is a rare vulvar tumor. Surgical excision is the main treatment option while other modalities are rarely employed. Overall survival rates are encouraging.


Assuntos
Doença de Paget Extramamária/cirurgia , Neoplasias Vulvares/cirurgia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Excisão de Linfonodo/estatística & dados numéricos , Pessoa de Meia-Idade , Doença de Paget Extramamária/mortalidade , Doença de Paget Extramamária/patologia , Prognóstico , Estados Unidos/epidemiologia , Neoplasias Vulvares/mortalidade , Neoplasias Vulvares/patologia , Vulvectomia/estatística & dados numéricos
13.
Diagn Pathol ; 14(1): 125, 2019 Nov 07.
Artigo em Inglês | MEDLINE | ID: mdl-31699107

RESUMO

BACKGROUND: Vulvar extramammary Paget disease is a rare chronic condition, that presents with non-specific symptoms such as pruritus and eczematous lesions. Because most of these lesions are noninvasive, the distinction between primary and secondary Paget disease is crucial to management. CASE PRESENTATION: We report an unusual case of vulvar Paget disease associated with massive dermal vascular embolization, cervicovaginal involvement and metastasis to inguinal and retroperitoneal lymph nodes. The intraepithelial vulvar lesion had a classical appearance and was accompanied by extensive component of dermal lymphovascular tumor emboli, similar to those observed in inflammatory breast carcinoma. Immunohistochemical analysis revealed that the lesion was secondary to high-grade urothelial cell carcinoma. The patient had a history of superficial low-grade papillary urothelial carcinoma of the bladder, which had appeared 2 years before the onset of vulvar symptoms. CONCLUSIONS: Eczematoid vulvar lesions merit careful clinical examination and biopsy, including vulva mapping and immunohistochemistry. The information obtained may help to define and classify a particular presentation of Paget disease. Noninvasive primary lesions do not require the same aggressive approaches required for the treatment of invasive and secondary disease.


Assuntos
Carcinoma de Células de Transição/patologia , Embolia/etiologia , Doença de Paget Extramamária/patologia , Neoplasias Urológicas/patologia , Neoplasias do Colo do Útero/patologia , Neoplasias Vulvares/patologia , Biomarcadores Tumorais/análise , Carcinoma de Células de Transição/diagnóstico , Feminino , Humanos , Pessoa de Meia-Idade , Gradação de Tumores , Doença de Paget Extramamária/diagnóstico , Neoplasias Urológicas/diagnóstico , Neoplasias Vulvares/diagnóstico
15.
Dis Colon Rectum ; 62(11): 1283-1293, 2019 11.
Artigo em Inglês | MEDLINE | ID: mdl-31567917

RESUMO

BACKGROUND: Extramammary Paget's disease is an uncommon intraepidermal adenocarcinoma with poorly defined clinical implications. OBJECTIVE: The purpose of this research was to estimate the risk of second primary neoplasms in patients with extramammary Paget's disease. DESIGN: This was a retrospective analysis of the Surveillance, Epidemiology, and End Results Registry (1973-2014). SETTINGS: The study included population-based cancer registries from the United States. PATIENTS: Patients who were diagnosed with anogenital Paget's disease were included. MAIN OUTCOME MEASURES: Risk of second primary development was measured. RESULTS: We identified 108 patients with anal Paget's disease, 421 patients with male genital (scrotum or penis) Paget's, and 1677 patients with female genital (vagina or vulva) Paget's. Median follow-up time was 5.9 years. The risk of developing colorectal adenocarcinoma was 18.5% for patients with anal Paget's disease. Eighty percent of colorectal adenocarcinoma diagnoses were synchronous (within 2 mo) to anal Paget's diagnoses, whereas metachronous tumors occurred at a median time of 2.4 years. Of patients with anal Paget's disease, 8.3% developed an anal adenocarcinoma or nonsmall cell cancer. In male patients with genital Paget's, the risk of proximal genitourinary malignancy was 9.7%, scrotal or testicular adenocarcinoma was 0.4%, and penile or scrotal squamous carcinoma was 1.7%. In female patients with genital Paget's, the risk of proximal genitourinary malignancy was 3.0%, vaginal or vulvar adenocarcinoma was 1.4%, and vaginal or vulvar squamous neoplasm was 1.0%. Five-year overall survival was 59.7%, 73.5%, and 80.7% in patients with anal, male genital, and female genital Paget's (p < 0.001). LIMITATIONS: The registry did not record surveillance schedule, provider specialty, or nonprocedural therapies for extramammary Paget's disease. CONCLUSIONS: In the largest published cohort of patients with extramammary Paget's disease, patients with anal Paget's had a much higher risk of both proximal and local neoplasms as compared with patients with genital Paget's. Patients with anal Paget's also experienced worse survival as compared with those with purely genital Paget's. See Video Abstract at http://links.lww.com/DCR/B20. ALTO RIESGO DE NEOPLASIAS PROXIMALES Y LOCALES EN 2206 PACIENTES CON ENFERMEDAD DE PAGET EXTRAMAMARIA ANOGENITAL:: La enfermedad de Paget extramamaria es un adenocarcinoma intraepidérmico poco frecuente con implicaciones clínicas poco definidas.Estimar el riesgo de segundas neoplasias primarias en pacientes con enfermedad de Paget extramamaria.Análisis retrospectivo del Registro de Vigilancia, Epidemiología y Resultados Finales (1973-2014).Registros de base poblacional en cáncer de los Estados Unidos.Pacientes que fueron diagnosticados con enfermedad de Paget anogenital.Riesgo de desarrollo un cáncer primario adicional.Se identificaron 108 pacientes con Paget anal, 421 pacientes con Paget genital masculino (escroto o pene) y 1677 pacientes con Paget genital femenino (vagina o vulva). Tiempo mediano de seguimiento fue de 5,9 años. El riesgo de desarrollar adenocarcinoma colorrectal fue del 18,5% para los pacientes con Paget anal. El ochenta por ciento de los diagnósticos de adenocarcinoma colorrectal fueron sincrónicos (dentro de los 2 meses) a los diagnósticos de Paget anal, mientras que los tumores metacrónicos ocurrieron en un tiempo promedio de 2,4 años. De los pacientes con Paget anal, el 8.3% desarrolló un adenocarcinoma anal o cáncer de células no pequeñas. En los pacientes masculinos con Paget genital, el riesgo de malignidad genitourinaria proximal fue del 9,7%, el adenocarcinoma escrotal o testicular fue del 0,4% y el carcinoma escamoso del pene o escroto fue del 1,7%. En pacientes femeninas con Paget genital, el riesgo de malignidad genitourinaria proximal fue de 3.0%, el adenocarcinoma vaginal o vulvar fue de 1.4% y la neoplasia escamosa vaginal o vulvar fue de 1.0%. La supervivencia general a cinco años fue del 59.7%, 73.5% y 80.7% en pacientes con anal, genital masculino y genital femenino, respectivamente (p <0.001).El registro no señalo el cronograma de vigilancia, la especialidad del proveedor o las terapias sin procedimiento para la enfermedad de Paget extramamaria.En la cohorte más grande publicada de pacientes con enfermedad de Paget extramamaria, los pacientes con Paget anal demostraron un riesgo mucho mayor de neoplasias proximales y locales en comparación con los pacientes con Paget genital. Los pacientes con Paget anal además demostraron una peor supervivencia en comparación con aquellos con Paget aislada genital. Vea el Resumen del Video en http://links.lww.com/DCR/B20.


Assuntos
Adenocarcinoma , Neoplasias do Ânus , Neoplasias dos Genitais Femininos , Neoplasias dos Genitais Masculinos , Segunda Neoplasia Primária , Doença de Paget Extramamária , Adenocarcinoma/epidemiologia , Adenocarcinoma/patologia , Assistência ao Convalescente/estatística & dados numéricos , Idoso de 80 Anos ou mais , Neoplasias do Ânus/epidemiologia , Neoplasias do Ânus/patologia , Epiderme/patologia , Feminino , Neoplasias dos Genitais Femininos/epidemiologia , Neoplasias dos Genitais Femininos/patologia , Neoplasias dos Genitais Masculinos/epidemiologia , Neoplasias dos Genitais Masculinos/patologia , Humanos , Masculino , Massachusetts/epidemiologia , Estadiamento de Neoplasias , Segunda Neoplasia Primária/epidemiologia , Segunda Neoplasia Primária/patologia , Doença de Paget Extramamária/epidemiologia , Doença de Paget Extramamária/patologia , Sistema de Registros/estatística & dados numéricos , Estudos Retrospectivos , Medição de Risco , Análise de Sobrevida
16.
Medicine (Baltimore) ; 98(41): e17018, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31593076

RESUMO

Paget's disease can arise in the breast (mammary Paget disease) or in other locations (extramammary Paget disease) such as anogenital skin in both males and females (Paget disease of the vulva [PDV]). Underlying adenocarcinoma can be found in some cases. This study aims to report clinical aspects, surgical procedures, outcomes, and recurrences of patients with PDV.A retrospective chart review was conducted on patients with pathologically confirmed diagnosis of PDV managed at the Department of Obstetrics and Gynecology, University of Bari, and the "Giovanni Paolo II" National Cancer Institute in Bari, between 1998 and 2018.Records of 24 cases of PDV were examined. Median age of the patients at diagnosis was 69.3 (range 38-84), diagnosis of synchronous cancer was made in 2 cases and in 2 other cases of metachronous disease. Three patients had previously been diagnosed with other oncological diseases. All patients underwent surgery including wide local excision (6), simple vulvectomy (8), and extended vulvectomy (10). Lymphadenectomy was performed in 2 cases and reconstructions with advancement flaps in 7 cases. Four patients were found to have invasive disease and 1 had inguinal node involvement. Positive margins were found in 11 patients. Wound dehiscence and urethral stenosis were found in 4 and 1 case each. Eight recurrences (33.33%) were observed, regardless of positive surgical margins.PDV has a low rate of malignancy but a high rate of recurrence. It should be diagnosed early to avoid repeated surgery and to reduce symptoms and morbidity.


Assuntos
Adenocarcinoma/patologia , Doença de Paget Extramamária/patologia , Neoplasias Vulvares/patologia , Adenocarcinoma/cirurgia , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Humanos , Pessoa de Meia-Idade , Recidiva Local de Neoplasia/patologia , Recidiva Local de Neoplasia/cirurgia , Doença de Paget Extramamária/cirurgia , Estudos Retrospectivos , Vulva/patologia , Vulva/cirurgia , Neoplasias Vulvares/cirurgia
18.
Hautarzt ; 70(9): 670-676, 2019 Sep.
Artigo em Alemão | MEDLINE | ID: mdl-31482274

RESUMO

Extramammary Paget's disease (EPD) is a rare, slowly growing, cutaneous adenocarcinoma with an incidence of 0.1-2.4 per 1,000,000 inhabitants. Histologically, EPD is characterized by the presence of epidermal Paget's cells, similarly to mammary Paget's disease. The EPD is typically divided into primary EPD (type I) and secondary EPD (type II associated with colorectal carcinoma and type III associated with urogenital carcinoma). From a clinical point of view, EPD is unspecific commonly mimicking chronic inflammatory skin disorders. This unspecific clinical picture can impede and delay the diagnosis of EPD. The treatment of choice for local EPD is the micrographically controlled excision. The extent of the infiltration of adnexal structures should be histologically determined prior to topical therapies, such as imiquimod and superficial ablative therapy. The complete excision of the tumor can be challenging due to ill-defined borders. In the metastatic stage the EPD has a poor prognosis. Controlled clinical trials for systemic treatment are still lacking.


Assuntos
Adenocarcinoma/patologia , Doença de Paget Extramamária/patologia , Neoplasias Cutâneas/patologia , Adenocarcinoma/cirurgia , Humanos , Doença de Paget Extramamária/cirurgia , Neoplasias Cutâneas/cirurgia
19.
J. coloproctol. (Rio J., Impr.) ; 39(3): 262-264, June-Sept. 2019. ilus
Artigo em Inglês | LILACS | ID: biblio-1040332

RESUMO

ABSTRACT Paget's disease most commonly affects the breast. Extramammary involvement is rare, and the most commonly affected sites are the vulva, anus, perianal region, and axilla. The disease may progress to invasive adenocarcinoma or synchronous cancers. Due to the lack of distinctive features and nonspecific presenting symptoms, Paget's disease may be misdiagnosed as other conditions, thus delaying the correct diagnosis. We report a case of extramammary Paget's disease in the perianal region that initially presented as an irregular, circumferential, scaling lesion with eczematous eruptions. Immunology and immunohistochemistry confirmed the diagnosis. Although surgery is the standard treatment, the patient opted for pelvic radiotherapy associated with radiosensitizing chemotherapy.


RESUMO A doença de Paget acomete mais comumente a mama. Os focos extramamários são raros e os locais mais habitualmente acometidos são vulva, ânus, região perianal e axila. A patologia envolve a evolução da doença para adenocarcinoma invasivo ou neoplasias sincrônicas. Devido à singularidade e sua aparência inespecífica, a doença pode ser confundida com outras comorbidades, retardando o diagnóstico. O objetivo foi relatar um caso de doença de Paget extramamária na região perianal, a qual inicialmente apresentou lesão circunferencial com erupção eczematosa, descamativa e irregular. A confirmação diagnóstica foi por meio de exames imuno-histoquímico e imunológico. O tratamento padrão da doença é cirúrgico, porém optou-se por radioterapia pélvica associada à quimioterapia radiossensibilizante.


Assuntos
Humanos , Feminino , Neoplasias do Ânus , Doença de Paget Extramamária/tratamento farmacológico , Radioterapia , Neoplasias Cutâneas , Doença de Paget Extramamária/diagnóstico , Doença de Paget Extramamária/patologia
20.
Pathol Int ; 69(10): 619-625, 2019 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-31397510

RESUMO

We report a case of cutaneous squamous cell carcinoma (CSCC), initially coexisting with invasive extramammary Paget's disease (EMPD), in the scrotum of an 84-year-old man. The patient initially had a rash and pruritus before presenting with a pedunculated scrotal mass surrounded by widespread erythema. He underwent total gastrectomy for adenocarcinoma 1 year previously and had been receiving TS-1 (Tegafur/Gimeracil/Oteracil pottasium) orally. Histopathologically, the tumor consisted of invasive SCC, with invasive EMPD in the erythematous region. From the clinical presentation and histopathological findings, we assumed that CSCC developed in the background of the EMPD. The CSCC metastasized to several inguinal lymph nodes and to the brain in the following years. While the histogenesis of each of the tumors remains to be elucidated, the fact that the CSCC rather than the EMPD metastasized to a distant site in this patient is to be noted for future treatment considerations.


Assuntos
Neoplasias Encefálicas/patologia , Carcinoma de Células Escamosas/patologia , Doença de Paget Extramamária/patologia , Neoplasias Cutâneas/patologia , Adenocarcinoma/patologia , Idoso de 80 Anos ou mais , Neoplasias Encefálicas/diagnóstico , Carcinoma de Células Escamosas/diagnóstico , Humanos , Linfonodos/patologia , Masculino , Doença de Paget Extramamária/diagnóstico , Neoplasias Cutâneas/diagnóstico
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