Extramammary paget's disease of the perineal skin: Role of radiotherapy

Besa, Pablo; Rich, Tyvin A.; Delclos, Luis; Edwards, Creighton L.; Ota, David M.; Wharton, J. Taylor

doi:10.1016/0360-3016(92)91024-h

Cited by 123 publications

(123 citation statements)

References 13 publications

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“…Dentro de las alternativas terapéuticas se han mencionado el uso de radioterapia [16][17][18] , quimioterapia, aplicación tópica de agentes citotóxicos e incluso el uso de láser de dióxido de carbono guiado por diagnóstico fotodinámico. La combinación de quimioterapia y radioterapia puede estar indicada en determinados casos, sin embargo, son necesarios estudios clínicos para valorar su efi cacia e importancia real.…”

Section: Discussionunclassified

Enfermedad de Paget perianal

et al. 2010

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Extra mammary Paget disease. Report of one caseExtra 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.Key words: Extra mammary Paget disease, vulva, perianal region. ResumenLa 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.Palabras clave: Enfermedad de Paget perianal, colgajos regionales, colgajos locales. CASOS CLÍNICOS IntroducciónLa enfermedad de Paget debe su nombre a James Paget 1 quien publicó una serie de casos de localización mamaria. La primera ubicación extramamaria conocida fue en el escroto. En general la ubicación extramamaria más frecuente es la vulva, siendo la perianal más rara.A diferencia de la enfermedad de Paget de la mama, que siempre está asociada a carcinoma intraductal, en la región perianal puede o no estar asociada a carcinoma subyacente. Debemos considerar además que en un alto porcentaje de casos puede haber asociación con otros tumores de localización tubo ováricos y colorrectales.Aunque las alternativas terapéuticas publicadas pueden ser variadas, la extirpación amplia suele ser lo más recomendado, generando defectos que deben ser reconstruidos mediante colgajos locales o regionales principalmente.

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Section: Discussionunclassified

Enfermedad de Paget perianal

et al. 2010

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“…The role of radiotherapy is not well defined in literature. Besa et al [11] evaluated the influence of radiotherapy in a series of 65 patients with EMPD arising in the vulva, perianal area or scrotum. They recommended radiation therapy as primary treatment in inoperable patients, and as alternative to surgery for recurrence disease previously excised or in case of mutilating surgery.…”

Section: Figure 1a: Pre Treatment Evaluationmentioning

confidence: 99%

Perianal Extramammary Paget’s Disease: Diagnosis, Treatment and Prognosis

Felice¹

2014

J Cytol Histol

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“…However, more detailed cases of successful treatment with radiotherapy have recently been published 4,5,[11][12][13][14][15][16][17][18] . Several investigators have made a strong case for the use of primary radiotherapy for curative intent in patients not considered suitable for surgery, and for the use of postoperative radiotherapy after resection 4,5,11 . Besa et al 11 observed a 36%-66% rate of positive margins after surgical excision alone in their series of 65 patients with empd of the perineal skin, and the actuarial local recurrence rate for patients treated with surgery was 40% within 5 years.…”

Section: Case Descriptionmentioning

confidence: 99%

“…Several investigators have made a strong case for the use of primary radiotherapy for curative intent in patients not considered suitable for surgery, and for the use of postoperative radiotherapy after resection 4,5,11 . Besa et al 11 observed a 36%-66% rate of positive margins after surgical excision alone in their series of 65 patients with empd of the perineal skin, and the actuarial local recurrence rate for patients treated with surgery was 40% within 5 years. They also noted the limited ability of surgery to adequately control a multicentric widespread process and the likelihood of surgery to be associated with considerable morbidity or functional impairment.…”

Section: Case Descriptionmentioning

confidence: 99%

“…We chose to use electron beam for the purpose of sparing the deeper tissues, given that only superficial tissues were at risk. Based on their experience of 12 cases, Besa et al 11 recommended a dose of more than 50 Gy using a single appositional field for the treatment of empd. Based on experience with other types of adenocarcinoma (such as postoperative breast radiotherapy after complete tumour excision), Brown et al 4 also suggested that 50 Gy is likely the minimum dose required to eradicate microscopic disease.…”

Section: Case Descriptionmentioning

confidence: 99%

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Perianal Paget disease treated definitively with radiotherapy

et al. 2012

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Extramammary Paget disease (EMPD) is a relatively rare cutaneous disorder described as an apocrine gland tumour occurring in both a benign and a malignant form with metastatic potential. The areas of the body affected are the vulva, perianal region, penis, scrotum, perineum, and axilla, all of which contain apocrine glands. When EMPD affects the perianal region, it is called perianal Paget disease (PPD). All forms of EMPD, including PPD, are typically treated by wide surgical excision. Perianal Paget disease usually occurs later in life in patients who are often poor surgical candidates, but the available literature is scarce regarding other treatment modalities, including definitive radiotherapy. We contend that PPD can be safely and effectively treated with radiotherapy, and here, we present the case of a 75-year-old woman with PPD who was successfully so treated. A brief review of the literature concerning the diagnosis, natural history, and treatment of PPD is also included.

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Extramammary paget's disease of the perineal skin: Role of radiotherapy

Cited by 123 publications

References 13 publications

Enfermedad de Paget perianal

Enfermedad de Paget perianal

Perianal Extramammary Paget’s Disease: Diagnosis, Treatment and Prognosis

Perianal Paget disease treated definitively with radiotherapy

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