Extramammary Paget Disease of the Vulva - Case Report / Relato de Caso: Doença de Paget Extramamária da Vulva

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.

Extramammary Paget's disease: Analysis of 17 Chinese cases.

Background/Objective: Extramammary Paget’s Disease (EMPD) seems to be more common in Caucasians than Chinese. We report the clinical manifestations, management, and prognostic characteristics in 17 Chinese patients. Methods: Medical records and biopsies of 17 patients who had been treated at a large university hospital in China between March 2005 and January 2012 were reviewed. Results: Of the 17 patients, 14 were men. They had lesions on the scrotum and the penis. Of the three women, two had vulvar and one had inguinal lesions. All patients underwent Mohs micrographic surgery (MMS). Three men had metastasis to the inguinal lymph nodes and underwent an extensive local excision with inguinal lymphadenectomy. Eight patients who had positive excision margins received additional radiation therapy. The mean follow-up duration was 54 months (4-85 months). One patient had two recurrences. Three had metastasis to the inguinal lymph node. One had metastasis to the bone and concomitant prostate cancer. Two patients died of the disease. Conclusion: A striking difference in presentation of EMPD in Chinese compared with Caucasians is the male predominance and location on the penis and scrotum. Mohs micrographic surgery followed by radiotherapy is an effective treatment. Long-term follow-up suggests that the disease has a good prognosis when it does not metastasise.

Case for diagnosis / Caso para diagnostico

An 83 year-old man was observed for an 8 year-long pruritic, erythematous plaque of the left axilla. He had been continuously medicated with topical antifungals and steroids with occasional symptomatic relief, but with disease progression. After a clinical and histological diagnosis of Extramammary Paget's Disease, a total excision with wide margins was performed. He remains disease-free 12 months after surgery. We report this rare presentation of Extramammary Paget's Disease on a male patient.

Um homem de 83 anos foi observado por placa eritematosa pruriginosa da axila esquerda com 8 anos de evolução. Estivera continuamente medicado com antifúngicos e corticóides tópicos com alívio sintomático ocasional, contudo com progressão da doença. Após diagnóstico clínico e histológico de Doença de Paget Extramamária, foi feita excisão total com margens largas. Mantém-se sem evidência de doença aos 12 meses após cirurgia. Salientamos esta apresentação rara de Doença de Paget Extramamária num paciente do sexo masculino.

Enfermedad de paget perianal / Extra mammary paget disease: report of one case

Extra mammary Paget disease is rare and mostly affects perianal and vulvar zones, in patients of both sexes and from the sixth decade of life. We report a 66 years old woman with a vulvar Paget disease, subjected to a vulvectomy. Two years later, a pruriginous and erythematous plaque appeared in the perianal region. The pathological study informed an extra mammary Paget disease. The patient was subjected to a perianal excision and the correction of the surgical defect in a second intervention. After one year of follow up, there is no evidence of disease relapse.

La enfermedad de Paget de localización extramamaria (EPEM) es una patología infrecuente que afecta preferentemente a zonas perianal y vulvar en pacientes de ambos sexos y desde la sexta década de vida. Presentamos un caso clínico tratado en conjunto por los Servicios de Cirugía General, Cirugía Plástica, Ginecología y Anatomía Patológica del Hospital Universitario de Getafe.

Perianal paget's disease

We report a rare case of Perianal Paget's disease. It presents with non-specific anal symptoms. The treatment of choice of Perianal Paget's disease is surgical excision. This patients due to refusal of any surgical intervention, managed successfully with radiotherapy. The patient had an eventful recovery after this conservative method

Extramammary Paget's disease with aggressive behavior: a report of two cases

Extramammary Paget's disease (EMPD) is an intraepithelial neoplastic disorder which is included as a rare malignant condition. However, it sometimes shows aggressive behavior of local recurrence and coexisting malignancy. We had experienced nine cases of EMPD involving the scrotum for seven years. Two cases of them presented metastasis. The first case presented extensive inguinal lymph node metastasis with underlying adnexal adenocarcinoma one year after wide local excision. The second case initially presented multiple metastasis to the liver and in the lymph node. The latter, showing fulminant progression with liver metastasis, may be only the second case reported in English literature. EMPD is considered as a malignant neoplasm with aggressive behavior from initial presentation. Because wide local excision of the lesion alone may be occasionally insufficient, a careful follow-up must be done to detect recurrence or internal malignancy.

Doença de Paget da vulva: relato de três casos / Vulvar Paget's disease: report of 3 cases

Entre 1983-90 foram diagnosticados e tratados três casos de doença de Paget da vulva no Departamento de Ginecologia do Hospital A. C. Camardo da Fundaçäo Antonio Prudente. Uma das pacientes (caso 2) apresentava um arcinoma de mama concomitante. Uma outra paciente (caso 1) apresentou também um câncer de mama após a doença Paget vulvar. A vulvectomia simples foi o tratamento de escolha em dois casos e na 3ª paciente foi feita vulvectomia total com linfadenectomias inguinal e pélvica. Um caso acha-se vivo (caso 3) quatro anos após a cirurgia. Os outros dois casos sobreviveram seis e sete anos, vindo a falecer devido a acidente vascular cerebral e a infarto do miocárdio repentino, livres de neoplasia. Nos casos de doença de Paget da vulva há possibilidade de existirem outros tumores primários. O clínico deve estar sempre alerta quanto a esta possibilidade.

Enfermedad de Paget perianal / Perianal Paget disease

Presentamos un caso de Paget perianal como contribución a la casuística, actualización de la patología y discusión de los controvertidos tratamientos. Se trató un paciente de sexo masculino y de 60 años, con lesión de 10 años de evolución, eritematosa, con erosiones, muy pruriginosa, de 12 cm de diámetro, que involucra circunferencialmente la piel perianal. No se halló tumor maligno asociado, cuya frecuencia oscila entre el 50 y el 86 por ciento. Se realizó, por negativa del paciente a la cirugía, terapia radiante con Co 60,3500 rads. Se obtuvo mejoría sintomática y disminución del tamaño lesional en un 60 por ciento. Se comprobó por biopsia la persistencia de enfermedad microscópica en la piel aparentemente recuperada. Se enfatiza que el tratamiento de la lesión es el quirúrgico, con adecuados márgenes de resección en superficie y profundidad, utilizando biopsias pre e intraoperatorias, para evaluar correctamente la verdadera extensión lesional y evitar las recidivas que se calculan en más de 35 por ciento cuando no se han tomado estos recaudos.

Doença de Paget Vulvar: relato de um caso / Vulvar Paget's Disease: report of a case

A doença de Paget Vulvar é um carcinoma de pele caracterizado pela presença das células de Paget na epiderme. Manifesta-se geralmente através de uma placa eritematosa de crescimento lento que evolui para uma lesäo descamativa, exsudativa ou verrucosa. Por isso, muitas vezes é confundida com um eczema e o diagnóstico correto passa a ser tardio. Os autores relatam um caso diagnosticado no Hospital Santa Rita e a seguir revisam a literatura sobre essa entidade