Extramammary Paget's Disease: An Annotated Review

Abstract

The incidence, clinical features, histogenesis, and treatment of extramammary Paget's disease (EMPD) are reviewed. This unusual skin lesion is associated with an underlying adnexal neoplasm in about 50% of cases. Also, the incidence of distant organ malignancies of EMPD is higher than expected by chance. Even in the absence of a recognizable underlying cancer, EMPD may occasionally produce distant metastases, indicating the malignant potential of this condition. Histochemical, immunohistological, and lectin binding studies demonstrate that the cell of origin of EMPD is the exocrine cell of sweat glands. Although EMPD may arise from eccrine cells, derivation from apocrine cells appears more common. The treatment of the primary lesion, by wide margin excision, is fraught by a high recurrence rate. Chemosurgery may reduce local relapse of EMPD. The value of adjuvant radiation therapy is unestablished. Chemotherapy has induced remission in 2 cases of advanced EMPD and needs testing in clinical trials.

Vulvar Paget Disease: One Century After First Reported

“…The surgical resection of visibly involved skin and mucosa, with visibly normal skin margins of resection, without frozen section assessment, is a reasonable and effective approach to vulvar cutaneous Paget disease [93]. As the frequency of local recurrence is strongly correlated with the presence of invasive disease [15] we reported in separate tables intraepithelial (Table 3) Because most recurrences of intraepithelial vulvar Paget disease consist entirely of intraepidermal disease, which does not affect survival [7], and the extent of the initial operation does not correlate with disease recurrence, conservative local excision for intraepithelial lesions is a reasonable consideration in young and old patients.…”

Dermatoses of the Male Genital Area

“…Three patterns of EPD have been accepted: (1) an epithelial in situ form without associated cancer; (2) an epithelial form with associated adnexal carcinoma; (3) EPD associated with visceral malignancy, which may be in an underlying organ of the genitourinary or gastrointestinal tract or at a distant site [42]. An intensive clinical examination with respect to visceral and anogenital malignancies is essential once the diagnosis of EPD has been made.…”

Extramammärer Morbus Paget – Klinik, Histogenese, Therapiemöglichkeiten

“…Interessanterweise kam es unter einer Monotherapie mit Docetaxel zu einer Remission vorhandener Lymphknotenmetastasen, nachdem diese zuvor auf Polychemotherapieschemata nicht angesprochen hatten [66]. Die insgesamt erheblichen Nebenwirkungen dieser Therapieschemata sollten berücksichtigt werden [68]. Mit dem Nachweis von HER2/neu in einer Subgruppe von Patienten mit EMP könnten sich, ähnlich einer Therapie von HER2/neu-positiven Mammakarzinomen, neue Ansatzpunkte für eine zielgerichtete Antikörpertherapie ergeben (42).…”

“…Die Prognose des sekundären EMP mit einem assoziierten Karzinom benachbarter Organe ist von der Prognose des Primärtumors abhängig. Für die gesamte Gruppe der Patienten mit einem sekundären EMP wird die durchschnittliche Überlebens-zeit mit nur 3 Jahren angegeben [68]. …”