Invasive extramammary Paget’s disease and the risk for secondary tumours in Europe

Zwan, Jan Maarten van der; Siesling, Sabine; Blokx, Willeke A.M.; Pierie, Jean-Pierre E. N.; Capocaccia, Riccardo

doi:10.1016/j.ejso.2011.12.008

Cited by 69 publications

(76 citation statements)

References 13 publications

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“…The neoplasm is found mainly at sites rich in apocrine glands, such as the external genitalia, the perianal region, the groin, and the axillas, 2,3 although it can also be found anywhere on the skin or mucosae. 7 The most common site is the vulva (65%), as in the cases we report, although this figure only represents 2% of all primary vulvar tumors. 4,9 The second most common site is the perianal region (20%), and the third is the male genitalia (14%).…”

Section: Discussionmentioning

confidence: 62%

“…4,9 Since this neoplasm is rare, there is no established protocol for its diagnosis, treatment, and follow-up, thus making it a challenge in clinical practice. 7 Diagnosis is based on symptoms and histopathology. Skin biopsy reveals cells with an abundant, pale cytoplasm, large pleomorphic nuclei, and, occasionally prominent nucleoli; the nuclei are sometimes eccentric and resemble signet rings (Fig.…”

Section: Discussionmentioning

confidence: 99%

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Extramammary Paget Disease: A Report of 10 Cases

Blasco‐Morente

Castro

Garrido‐Colmenero

et al. 2015

Actas Dermo-Sifiliográficas (English Edition)

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Extramammary Paget disease (EMPD) is a very rare form of intraepithelial adenocarcinoma. We report the cases of EMPD diagnosed in a referral hospital over a 5-year period from 2009 to 2013. Ten cases of EMPD were diagnosed in 4 men and 6 women aged between 50 and 79 years. Erroneous clinical diagnoses led to diagnostic delays of 1 to 5 years in all cases. Six patients had in situ lesions and dermal invasion was observed in the other 4 cases. Four patients had a second neoplasm and 2 of the patients with invasive EMPD died. Because EMPD is rare and its clinical presentation is nonspecific, a high degree of clinical suspicion is required to avoid delays in diagnosis and treatment.

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Section: Discussionmentioning

confidence: 62%

Section: Discussionmentioning

confidence: 99%

Extramammary Paget Disease: A Report of 10 Cases

Blasco‐Morente

Castro

Garrido‐Colmenero

et al. 2015

Actas Dermo-Sifiliográficas (English Edition)

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“…In a recent M.D. Anderson Cancer Centre report, the incidence of a second malignancy was 45% 31 , and in a European study, the overall standardized incidence ratio was 1.39 3 .…”

Section: Discussionmentioning

confidence: 99%

“…The true incidence of empd is not known, but in older reports, it represented 6.5% of all cases of Paget disease 2 . A recent European study revealed an age-standardized incidence of invasive empd of 6 per million person-years 3 .…”

Section: Introductionmentioning

confidence: 99%

Management of Penoscrotal Extramammary Paget Disease: Case Series and Review of the Literature

et al. 2013

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The medical literature available to guide the management of empd is limited, particularly for metastatic disease. Another challenge is to differentiate metastatic empd from a concurrent invasive cancer. Here, we present our case series of 6 patients with empd of the penoscrotal region, our experience with systemic therapy, and a literature review for this rare malignancy. METHODSWe identified 6 cases of penoscrotal empd treated at The Ottawa Hospital from 2007 to 2012. All cases underwent pathology review. After research ethics board approval, clinical and demographic data, including age at presentation, presence of a second malignancy, management, and follow-up were collected from patient records. The literature was reviewed for clinical and pathologic features, treatment options, and outcomes. RESULTS 3

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“…[11,17,[20][21][22][23][24][25] When local therapy of the primary PPD is successful, the prognosis is almost always favorable. [8,26] The treatment and prognosis of the secondary form of PPD depends mainly on the stage of the associated tumor and treatment should be more invasive. [14,19] Follow-up of PPD should be thorough because of the frequency of local recurrence and also because of the elevated risk of developing a second primary tumor.…”

Section: Introductionmentioning

confidence: 99%

Perianal Paget’s disease: case report and review of the literature

Bontinck

Rondou³

et al. 2016

Acta Chirurgica Belgica

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A peri-anal skin lesion, often eczema-like and with symptoms of pruritus, that does not resolve after classical local therapy should be biopsied. We present a case of peri-anal extramammary Paget's disease (EMDP) and associated anal adenocarcinoma. Reviewing the literature, more than 30% of patients with EMDP present a second primary tumour in their past, present or future history. In Europe, the risk of developing a new primary tumour in patients with this condition is increased compared with the standard population. In cases of peri-anal Paget's disease (PPD), specific histochemical markers allow us to differentiate between a primary and a secondary form, the secondary one is strongly associated with colorectal and anal tumours. We provide information about the most commonly suggested therapy for PPD with or without associated malignancy and about the recommended follow-up.

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Invasive extramammary Paget’s disease and the risk for secondary tumours in Europe

Cited by 69 publications

References 13 publications

Extramammary Paget Disease: A Report of 10 Cases

Extramammary Paget Disease: A Report of 10 Cases

Management of Penoscrotal Extramammary Paget Disease: Case Series and Review of the Literature

Perianal Paget’s disease: case report and review of the literature

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