Concurrent extramammary Paget's disease involving the genitalia and axilla: Case report and literature review

Abstract

Concurrent multiple primary extramammary Paget's disease (EMPD) is rare.Herein, we present two Chinese cases of concurrent primary EMPD involving both the genitalia and the axilla, and they also had a history of other malignancy.We also summarise the cases of multiple primary EMPD previously described in literature. Careful examination of all apocrine sweat gland-bearing sites and additional internal malignancies is recommended for patients with EMPD.

Extramammary Paget's disease involving the axilla: case series and literature review

“…A total of 303 cases of EMPD were identified, of which 16 cases (5.3%) with axillary involvement were recorded and summarized in Table Regarding the clinical manifestations of the disease, 11 patients (68.8%) presented with unilateral axillary lesions, two patients with bilateral axillary lesions, and three patients with both axillary and genital involvement, which included the two cases we reported previously. 2 Well-demarcated erythematous to brownish patches or plaques were typical clinical manifestations in most patients, erosions, verrucous hyperplasia, and variable pigmentation including hyperpigmentation and hypopigmentation were also observed in some cases (Figure 1).…”

“…Gene mutations in RAS , RAF , PIK3CA , AKT1 , ERBB2 , ERBB3 , TP53 , KMT2C , ARID2 , and FSIP2 have been observed in EMPD 9–12 . Genetic background and oncogenes activation leading to multicentric malignant transformation may underlie the possible pathogenesis of multiple EMPD and EMPD with secondary malignancies 2,6 . We suspected that excessive activation of the androgen receptor‐related pathway 7 may be associated with male predominance; however, further research is needed.…”

“…[9][10][11][12] Genetic background and oncogenes activation leading to multicentric malignant transformation may underlie the possible pathogenesis of multiple EMPD and EMPD with secondary malignancies. 2,6 We suspected that excessive activation of the androgen receptor-related pathway 7 may be associated with male predominance; however, further research is needed. Asymptomatic lesions are more prevalent in axillary EMPD than in anogenital EMPD, which may lead to the ignorance and progression of the disease.…”

Protruding mass in the pubis

“…6 Multiple EMPD involving separate sites have also been reported. 7 The mechanism of multiple EMPD and the association with other malignancies remain unclear. Genetic background and oncogene activation leading to multicentric malignant transformation may be the possible pathogenetic explanation.…”

Exophytischer Tumor am Mons pubis

“…6 Es wurde auch über multiple EMPD berichtet, die an verschiedenen Stellen auftreten. 7 Der Mechanismus der multiplen EMPD und der Zusammenhang mit anderen bösartigen Erkrankungen sind noch unklar. Der genetische Hintergrund und die Aktivierung von Onkogenen, die zu multizentrischen malignen Transformationen führen, könnten eine mögliche pathogenetische Erklärung sein.…”