Invasive adenocarcinoma arising in extramammary Paget's disease of the vulva

Evans, Andrew; Neven, Patrick

doi:10.1111/j.1365-2559.1991.tb00857.x

Cited by 24 publications

(17 citation statements)

References 26 publications

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“…EMPD does not frequently invade the underlying structures [2]. However, in some instances, EMPD tumor cells invade deep into the dermis and underlying tissues, forming a large mass of invasive carcinoma tissue [3]. EMPD cases with lymphatic invasion show a poor prognosis and generally higher mortality rates [4].…”

Section: Introductionmentioning

confidence: 99%

High expression of Ki-67 and cyclin D1 in invasive extramammary Paget's disease

Aoyagi

Akiyama

Shimizu

2008

Journal of Dermatological Science

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Section: Introductionmentioning

confidence: 99%

High expression of Ki-67 and cyclin D1 in invasive extramammary Paget's disease

Aoyagi

Akiyama

Shimizu

2008

Journal of Dermatological Science

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“…It is further postulated that, analogous to lentigo maligna, primary intraepidermal Paget disease (that is, without associated carcinoma) may eventually show dermal invasion after a significant period of intraepidermal growth and thus give rise to adenocarcinoma which may metastasise. 3 In our case it is most likely that the Paget disease arose secondary to the recurrent primary vulval adenocarcinoma (initially diagnosed eight years before this presentation). The symptoms of vulval irritation and discharge, which are those of Paget disease, were of relatively recent onset.…”

Section: Discussionmentioning

confidence: 73%

Extension of extramammary Paget disease of the vulva to the cervix.

1999

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Extramammary Paget disease of the vulva was found in association with vulval adenocarcinoma in an elderly woman who also had a uterine prolapse. The characteristic histological appearances of extramammary Paget disease were masked by striking reactive changes in the squamous epithelium. Primary excision of both the intraepithelial and invasive disease appeared complete. However, a subsequent hysterectomy with repair of the prolapse revealed extramammary Paget disease in the upper vaginal mucosa and cervix, a finding which is very rarely described. Pathogenesis and diagnosis of extramammary Paget disease is discussed, with diVerential diagnosis and reference to immunohistochemical methods. (J Clin Pathol 1999;52:538-540) Keywords: vulva; Paget disease; extramammary Paget disease; cervix Case report An 88 year old woman was referred to the gynaecology department with the diagnosis of a vulval tumour and uterine prolapse with profuse vulval discharge. The patient had received surgical treatment eight years earlier for invasive adenocarcinoma of the vulva, but the original histology was not available for assessment. Clinical examination revealed a large tumour arising on the left side of the vulva with associated thickening and scaling of the surrounding skin. Symptomatic prolapse of the uterus was also noted. Incisional biopsy of the tumour revealed the presence of invasive, poorly diVerentiated adenocarcinoma and the patient was admitted for radical vulvectomy, bilateral groin lymphadenectomy, and split skin grafting. Pathological findingsThe vulvectomy specimen was a crescent shaped piece of non-pigmented skin (17 cm lateral dimension, 8 cm anteroposterior) with a greater proportion of the skin lying to the left of the introitus. A firm, indurated, raised white tumour (2 × 1 × 1 cm) was present 2 cm to the left of the introitus. The tumour was at least 3 cm clear of the deep resection margin. Much of the skin around the tumour was rough and fissured and displayed multiple papillary projections. The skin at the introitus was glazed and shiny, suggesting atrophy. A 0.5 cm rim of macroscopically normal skin was present around the lateral resection margin.The histological diagnosis of an adenocarcinoma made on the original biopsy was confirmed. Some areas showed mucin production (fig 1), others had prominent signet ring cells, while other areas were less well diVerentiated. The abnormal skin adjacent to and overlying the tumour showed marked squamous hyperplasia with dramatic elongation of the rete ridges and striking keratinisation (fig 2). The epidermis had an atypical appearance and close inspection showed that it was infiltrated by a population of pale granular cells with large, pleomorphic nuclei and prominent nucleoli. The cells were concentrated in the basal epidermis but staining for mucin and epithelial membrane antigen (EMA) revealed that numbers of them were also were present in the upper strata. A diagnosis of extramammary Paget disease was made. The Paget disease was multifocal, with isolated ...

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“…So ist es umgekehrt auch durchaus vorstellbar, dass sich ein primär in den epidermalen Strukturen entstandener Morbus Paget in den kutanen Adnexen ausbreitet und von hier in ein invasives Wachstum übergeht. Beide Szenarien können im Einzelfall und besonders bei fortgeschrittenen Stadien histopathologisch nicht voneinander unterschieden werden [44,45]. Immunhistologische Untersuchungen können bei der Differenzierung der verschiedenen PagetFormen hilfreich sein.…”

Section: Pathogeneseunclassified

Extramammärer Morbus Paget – Klinik, Histogenese, Therapiemöglichkeiten

Wagner

Sachse

2011

J Deutsche Derma Gesell

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Invasive adenocarcinoma arising in extramammary Paget's disease of the vulva

Cited by 24 publications

References 26 publications

High expression of Ki-67 and cyclin D1 in invasive extramammary Paget's disease

High expression of Ki-67 and cyclin D1 in invasive extramammary Paget's disease

Extension of extramammary Paget disease of the vulva to the cervix.

Extramammärer Morbus Paget – Klinik, Histogenese, Therapiemöglichkeiten

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