Paget's disease is an intra-epidermal adenocarcinoma seen over the nipple/areola (mammary Paget's disease) or in extramammary body zones, such as the anogenital and perineal skin and the axilla. Mammary and extramammary Paget's disease share many common clinicopathological features but also show several differences, namely, with regard to pathogenesis and association with underlying malignancies. Indeed, mammary Paget's disease is as a rule associated with an underlying breast carcinoma whereas association of extramammary Paget's disease with underlying (skin or visceral) malignancies occurs much less frequently. We review here the main clinicopathological and therapeutic features of mammary and extramammary Paget's disease.

Mammary and extramammary Paget's disease.

Summary

Background Extramammary Paget’s disease (EMPD) is a rare cutaneous carcinoma usually presenting as a genital erythematous lesion in the elderly. Although most EMPD tumours are in situ, invasive EMPD has a poor prognosis.



Objective  To evaluate the clinical and pathological features of EMPD and determine prognostic factors for survival.



Methods  The medical records of 76 patients with EMPD were retrospectively reviewed.



Results  Of the 66 patients who underwent curative surgical excision, five (8%) developed local recurrence, but surgical margin (≤ 2 cm or > 2 cm) was not correlated with local recurrence. Thirteen of the 76 patients (17%) developed systemic metastases and 10 of these died of disease. On univariate analysis, the presence of nodules in the primary tumour, clinical lymph node swelling, elevated serum carcinoembryonic antigen (CEA) levels, tumour invasion level and lymph node metastasis were significant prognostic factors. On multivariate analysis, invasion level and elevated serum CEA were the only factors that were significantly associated with reduced survival.



Conclusions  Invasion level and lymph node metastasis are important prognostic factors in EMPD. In patients with in situ tumour, local tumour control is the major aim of treatment; however, wide surgical margins are not associated with a lower risk of local recurrence.

Extramammary Paget’s disease: treatment, prognostic factors and outcome in 76 patients

Background Extramammary Paget's disease (EMPD) is a rare clinical entity and can be associated with other malignancies. We analyzed our experience for prognosis and impact of therapy on outcomes. Study design We conducted a retrospective review of all patients (n = 33) with EMPD treated at a tertiary care center from 1971 to 1998. Pathologic features of EMPD, concurrent secondary malignancies, and the effect of operations on recurrence were analyzed. Overall survival was compared with that of the general population. Results Male-to-female ratio was 4:29, and median age was 70 years. Median followup was 68 months, and no patient died from EMPD. The lesion was predominantly found on the vulva (76%). Patch-like, nonconfluent growth was present in 45% of patients, and no patient had pathologic lymph nodes. The most common signs and symptoms were irritation or pruritus (73%) and rash (61%). The presence of patches, invasive tumor growth, or a second malignancy were significantly associated with a higher recurrence rate. The type of operation, either local excision or hemivulvectomy, was not related to the time to recurrence. Complete gross resection was achieved in 94% of cases. Fifty-six percent of patients had microscopically positive margin and this correlated with a significantly higher recurrence rate (p = 0.002). The tumor recurred clinically in 14 of 33 patients (42%) after a median of 152 months (range 5 to 209 months). In those patients, between one and six reexcisions were performed. In 14 of 33 patients with EMPD (42%), 16 concurrent secondary malignancies were found. Overall survival rates for EMPD patients were similar to those of the general population. Conclusions EMPD is an infrequently diagnosed disease that is preferably managed with complete local excision and reexcisions if needed. A thorough search for frequently occurring secondary malignancies might be beneficial to provide the best outcomes for these patients.

Prognosis and management of extramammary Paget's disease and the association with secondary malignancies.

Treatment outcomes in a large cohort of patients with invasive Extramammary Paget's disease.

The outcomes of patients with positive margins after excision for intraepithelial Paget's disease of the vulva.

Extramammary Paget's disease.

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Extramammary Paget's disease.