ABSTRACT
Extramammary Paget disease (EMPD) is a kind of rare clinical malignant tumor in the skin. Surgical treatment is the main treatment method, and the Mohs microsurgery is considered the first choice which can control the incision margin accurately and reduce the recurrence rate. Due to the insidious and multifocal nature of the disease, the postoperative recurrence rate is still high. The other treatment methods include radiation therapy, chemotherapy, targeted therapy, photodynamic therapy, local drug therapy and laser therapy. Further research on the progress of EMPD treatment is helpful for the management and treatment of patients in clinical practice.
ABSTRACT
RESUMEN La enfermedad de Paget extramamaria (EPEM) perianal es un adenocarcinoma intraepitelial del margen anal de muy baja frecuencia. La forma primaria representa una neoplasia de origen apocrino, mientras que la forma secundaria representa una diseminación pagetoide de una neoplasia maligna en general anorectal. Debido a su similitud clínica con otras patologías de presentación frecuente, se requiere de un alto índice de sospecha para diagnosticarla. La resección quirúrgica amplia o la cirugía micrográfica de Mohs se considera comúnmente el pilar del tratamiento con altas tasas de recurrencia. Estudios previos han demostrado buena respuesta en la EPEM vulvar con imiquimod 5% en crema, pero su eficacia no ha sido bien descrita en la afectación perianal. En este artículo presentamos un caso de EPEM perianal primario con sobreinfección con HPV, al que se le indicó tratamiento tópico con imiquimod.
ABSTRACT Perianal extramammary Paget disease is a very low frequency intraepithelial adenocarcinoma of the anal margin. The primary form represents a neoplasm of apocrine origin, while the secondary form represents a pagetoid spread of a generally anorectal malignant neoplasm. Due to its clinical similarity with other pathologies with frequent presentation, a high index of suspicion is required to diagnose it. Wide surgical resection or Mohs micrographic surgery is commonly considered the mainstay of treatment with high recurrence rates. Previous studies have shown a good response in vulvar extramammary Paget disease with imiquimod 5% cream, but its efficacy has not been well described in perianal involvement. In this article we present a case of primary perianal extramammary Paget disease with HPV superinfection, which received topical treatment with imiquimod.
ABSTRACT
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.
Subject(s)
Humans , Female , Anus Neoplasms , Paget Disease, Extramammary/drug therapy , Radiotherapy , Skin Neoplasms , Paget Disease, Extramammary/diagnosis , Paget Disease, Extramammary/pathologyABSTRACT
Abstract Extramammary Paget disease is a rare neoplastic condition that more commonly affects postmenopausal Caucasian women. Although the vulvar area is the most frequently affected location, it corresponds solely to 1 to 2% of all vulvar malignancies. A 72-year-old female patient was observed in our outpatient clinic with a 2-year history of an erythematous and pruritic plaque on the vulva. Histopathology and immunohistochemistry studies were compatible with extramammary Paget disease of the vulva. Associated neoplastic conditions were excluded. Due to multiple relapses, the patient was submitted to three surgical interventions, including a total vulvectomy, and to external radiotherapy. The present case illustrates the chronic and recurrent nature of extramammary Paget disease despite aggressive procedures as well as the challenge in obtaining tumor-free resection margins.
Subject(s)
Humans , Female , Aged , Vulvar Neoplasms/pathology , Paget Disease, Extramammary/pathology , Vulvectomy , Imiquimod/therapeutic use , Neoplasm Recurrence, Local/pathology , Antineoplastic Agents/therapeutic use , Vulvar Neoplasms/therapy , Treatment Outcome , Paget Disease, Extramammary/therapy , Plastic Surgery ProceduresABSTRACT
Advanced extramammary Paget's disease does not have a standardized treatment guideline as its incidence is low and has been rarely reported in literature. Here we describe a case of metastatic extramammary Paget's disease successfully treated with topical 5-fluorouracil (5-FU) and systemic pemetrexed. The therapy was safe without any appreciable adverse effects like diarrhea, rash, neutropenia or fatigue; maintaining remission for more than 6 months. Thus, we propose 5-FU and pemetrexed as the first-line therapy for advanced extramammary Paget's disease, especially for aged patients with unresectable skin lesions.
ABSTRACT
RESUMEN: La enfermedad de Pagetextramamaria es un adenocarcinoma intraepitelial,extremadamente raro. Su diagnóstico suele ser tardío pudiendo demorarse hasta tres años. Debemos hacer un diagnóstico diferencial con otras enfermedades dermatológicas, que pueden cursar igual. El diagnóstico definitivo es histopatológico. La cirugía es el tratamiento de elección, siendo la cirugía micrográfica de Mohs la más empleada. Existen tratamientos tópicos tales como el imiquimod, que pueden ser útiles. En general el pronóstico suele ser favorable.
SUMMARY: Extramammary Paget disease is an intraephithelial adenocarcinoma very rare. Its diagnosis is usually late and may take up to 3 years. We must make a differential diagnosis with other dermatological diseases that can occur equally. The definitive diagnosis is histopatological. Surgery is the gold standard treatment, Mohs micrographic surgery being the most used. There are topical treatments such as imiquimod that may be useful. In general, the prognosis is usually favorable.
ABSTRACT
Ectopic extramammary Paget's disease (EMPD) is a rare variant of EMPD that develops in non-apocrine regions. We present a 70-year-old man in whom ectopic EMPD affected the lower back. Initially, erythematous scaly plaques with several papules on the back were suggestive of Bowen's disease. However, the biopsy specimen and immunohistochemical study results were consistent with EMPD features. The lesion was resected with a 1-cm safety margin and covered with a full thickness local skin graft. Ectopic EMPD is so rare that only few cases of EMPD in a non-apocrine region have been reported. We considered this case of EMPD on the back to be interesting since there has been no previous case reported in this location in Korea.
Subject(s)
Aged , Humans , Biopsy , Bowen's Disease , Korea , Paget Disease, Extramammary , Skin , TransplantsABSTRACT
No abstract available.
Subject(s)
Hyperpigmentation , Paget Disease, Extramammary , ScrotumABSTRACT
No abstract available.
Subject(s)
Anal Canal , Paget Disease, Extramammary , Radiotherapy , VulvaABSTRACT
La enfermedad de Paget extramamaria es un adenocarcinoma intraepitelial, que ocurre en sitios ricos en glándulas apocrinas, como: vulva, axila, región anogenital y escroto en el varón. Presentamos el caso de una paciente, con una lesión eritemato-descamativa en región perianal, de seis meses de evolución, con buena respuesta al tratamiento y favorable evolución.
Extramammry Paget's disease is an intraepithelial adenocarcinoma that occurs in areas rich apocrine glands, such as the: vulva, axilla, anogenital area and scrotum in man. We present the clinical case in a patient with an erythematous exudative lesión in perineal area over the last six months with good response to treatment. The patient evolved favorably.
ABSTRACT
Extramammary Paget disease (EMPD) is a rare cutaneous neoplasm. Perianal Paget disease (PPD) is a subset of EMPD manifesting perianal lesions. Two cases of PPD in Severance Hospital are described in this article. A 65-year-old female and 78-year-old male patients visited our institution because of an unhealed perianal skin lesion despite treatment for a long period with topical agents. PPD was diagnosed by skin biopsies in both cases, and the patients underwent surgical treatment. Clinical manifestations, preoperative work-ups, and surgical treatments including different reconstruction methods are described in detail. As only sporadic PPD cases have been reported and no standard treatment has been established, we hope that our experience could contribute to improving the diagnosis and treatment of PPD patients.
Subject(s)
Aged , Female , Humans , Male , Biopsy , Diagnosis , Hope , Paget Disease, Extramammary , SkinABSTRACT
Extramammary Paget disease (EMPD) is a rare cutaneous neoplasm. Perianal Paget disease (PPD) is a subset of EMPD manifesting perianal lesions. Two cases of PPD in Severance Hospital are described in this article. A 65-year-old female and 78-year-old male patients visited our institution because of an unhealed perianal skin lesion despite treatment for a long period with topical agents. PPD was diagnosed by skin biopsies in both cases, and the patients underwent surgical treatment. Clinical manifestations, preoperative work-ups, and surgical treatments including different reconstruction methods are described in detail. As only sporadic PPD cases have been reported and no standard treatment has been established, we hope that our experience could contribute to improving the diagnosis and treatment of PPD patients.
Subject(s)
Aged , Female , Humans , Male , Biopsy , Diagnosis , Hope , Paget Disease, Extramammary , SkinABSTRACT
Abstract Extramammary Paget disease (EPD) is a rare malign neoplasm that may affect the vulva and has manifestations common to benign diseases such as itching, pain and eczema. This leads to delay in diagnosis and consequent worse prognosis. The definitive diagnosis is obtained by biopsy of the vulva, which shows Paget cells. The treatment of choice is wide excision with margins, which leads to sequelae, functional and aesthetic. Recurrence is common. This article reports the case of a 48-year-old female patient with history of vulvar itching for the past 2 years, who had been submitted to various treatments for benign pathologies. The patient was biopsied and was diagnosed with extensive EPD, being submitted to vulvectomy. This article aims to draw attention to the need for biopsy of pruritic vulvar lesions that do not respond to usual treatment.
Resumo A doença de Paget extramamária (DPEM) é uma neoplasia maligna incomum que pode acometer a vulva, e que tem manifestações comuns a doenças benignas como prurido, dor e eczema. Isto leva a atraso no diagnóstico e, consequente, piora do prognóstico. O diagnóstico de certeza se dá pela biópsia de vulva que evidencia células de Paget. O tratamento de escolha é a excisão com margens amplas, que leva a sequela funcional e estética. A recidiva é frequente. Este artigo relata o caso de uma paciente do sexo feminino, de 48 anos, com história de prurido vulvar há 2 anos com diversos tratamentos para patologias benignas. A biopsia da paciente confirmou diagnóstico de DPEM extensa sendo submetida a vulvectomia. Este artigo visa chamar a atenção para a necessidade de biópsia das lesões vulvares pruriginosas que não respondem ao tratamento habitual.
Subject(s)
Humans , Female , Middle Aged , Paget Disease, Extramammary , Vulvar Neoplasms , Paget Disease, Extramammary/pathology , Paget Disease, Extramammary/surgery , Vulvar Neoplasms/pathology , Vulvar Neoplasms/surgeryABSTRACT
Treatment of perianal and vulvar extramammary Paget disease (EMPD), rare intraepithelial malignancies, is often challenging because of its potential to spread into the anal canal. However, there is still no consensus regarding the optimal resection margin within the anal canal. Between 2004 and 2014, six patients (three with perianal EMPD and three with vulvar EMPD) in which the spread of Paget cells into the anal canal was highly suspected were referred to our department. To evaluate the disease extent within the anal canal, preoperative mapping biopsy of the anal canal was performed in five out of six patients. Two patients were positive for Paget cells within the anal canal (one at the dentate line and the other at 0.5 cm above the dentate line), whereas in three patients, Paget cell were present only in the skin of the anal verge. Using 1 cm margin within the anal canal from the positive biopsy sites, we performed anal-preserving wide local excision (WLE), and negative resection margins within the anal canal were confirmed in all five patients. The remaining one patient with perianal EMPD did not undergo mapping biopsy of the anal canal because preoperative colonoscopy revealed that the Paget cells had spread into the lower rectum. Therefore, WLE with abdominoperineal resection was performed. During the median follow-up period of 37.3 months, no local recurrence was observed in all patients. Our small case series suggest the usefulness of mapping biopsy of the anal canal for the treatment of perianal and vulvar EMPD.
Subject(s)
Humans , Anal Canal , Biopsy , Colonoscopy , Consensus , Follow-Up Studies , Paget Disease, Extramammary , Rectum , Recurrence , SkinABSTRACT
Treatment of perianal and vulvar extramammary Paget disease (EMPD), rare intraepithelial malignancies, is often challenging because of its potential to spread into the anal canal. However, there is still no consensus regarding the optimal resection margin within the anal canal. Between 2004 and 2014, six patients (three with perianal EMPD and three with vulvar EMPD) in which the spread of Paget cells into the anal canal was highly suspected were referred to our department. To evaluate the disease extent within the anal canal, preoperative mapping biopsy of the anal canal was performed in five out of six patients. Two patients were positive for Paget cells within the anal canal (one at the dentate line and the other at 0.5 cm above the dentate line), whereas in three patients, Paget cell were present only in the skin of the anal verge. Using 1 cm margin within the anal canal from the positive biopsy sites, we performed anal-preserving wide local excision (WLE), and negative resection margins within the anal canal were confirmed in all five patients. The remaining one patient with perianal EMPD did not undergo mapping biopsy of the anal canal because preoperative colonoscopy revealed that the Paget cells had spread into the lower rectum. Therefore, WLE with abdominoperineal resection was performed. During the median follow-up period of 37.3 months, no local recurrence was observed in all patients. Our small case series suggest the usefulness of mapping biopsy of the anal canal for the treatment of perianal and vulvar EMPD.
Subject(s)
Humans , Anal Canal , Biopsy , Colonoscopy , Consensus , Follow-Up Studies , Paget Disease, Extramammary , Rectum , Recurrence , SkinABSTRACT
BACKGROUND: Extramammary Paget's disease (EMPD) is a relatively rare cutaneous disorder. There are a few studies regarding the clinical features and prognostic factors of EMPD in Korean patients. OBJECTIVE: The aim of this study was to identify in detail the clinical findings and prognostic factors of EMPD in Korean patients. METHODS: A total of 19 patients with confirmed EMPD at our institution were included from January 2005 to December 2014. Clinical records and pathology slides of the patients were reviewed retrospectively. RESULTS: Patients' ages ranged from 50 to 87 years (median, 64.5 years), and incidence of EMPD was higher in men than in women (17:2, male:female). The most common predilection site was the penoscrotal area in men and the vulva in women. All patients with EMPD had no underlying malignancy. Four of 19 patients treated with wide excision had local recurrence during the follow-up period. Two patients with deep dermal invasion died due to multiple metastases. CONCLUSION: EMPD in Korea is characterized by its male predominance and penoscrotal location. Disease recurrence is common regardless of the surgical margin. Its prognosis could be associated with depth of invasion and lymph node metastasis.