Clinical Features And Follow Up Analysis Of 19 Patients With Extramammary Paget Disease

Extramammary Paget's disease (EMPD) is an uncommon neoplasm of apocrine gland-bearing skin. The most common site of involvement is the vulva, perineal, perianal, scrotal and penile skin. Clinically, the lesions present as well defined, moist, erythematous or white plaques and are usually accompanied by pruritus. EMPD is difficult to be diagnosed at the initial time and the recurrence rate is high. The local recurrence rate is high (18-37%) due to several characteristics of EMPD, such as multicentricity and ill-defined margins. Generally, patients with EMPD have a good prognosis because the tumor cells spread only in situ within the epidermis. Wide local excision is the choice of treatment for patients with in situ tumor. On the other hand, invasive EMPD is frequently associated with regional lymph node metastasis and has a poor prognosis. Given the rarity of this disease, little is known about its prognostic factors. A few studies have suggested that the invasion level of the primary tumour may be the most important prognostic factor. ObjectiveTo evaluate the clinical and pathological features of extramammary Paget disease (EMPD) and investigate prognostic factors for survival.MethodsThe medical records of 19 patients with EMPD from Sir Run Run Shaw Hospital were retrospectively reviewed. Collect the following research contents of EMPD patients:age, gender, time from onset to diagnosis, clinical manifestation, secondary malignance, pathology and lymph node condition.ResultsThe mean age at diagnosis was 70.26±8.9 years. The average time between onset and diagnosis was 43 month (ranged:1-6.5 years). There were 4 females and 15 males. The most common symptom was multifocal erythema, erosion, and eczematoid dermatitis on external genitals and anus.13 patients were treated with surgical excion, of which 2 recurred.5 patients were treated with photodynamic therapy.1 patient had no therapy. Of the patients who were followed up, seven died. The 5-year overall survival rate for all patients was 61.20%. On univariate analysis, lymph node metastasis, tumor invasion level, secondary malignant or distant metastasis, and elevated serum carcinoembryonic antigen (CEA) levels were significant prognostic factors.ConclusionsEMPD is difficult to be diagnosed at the initial time and the recurrence rate is high. Invasion level, secondary malignancy or distant metastasis, and lymph node metastasis are important prognostic factors in EMPD. It is suggested that suspect patient should carry out skin and lymph node biopsy. Immunohistochemistry could be helpful to diagnose and treatment. Long time follow-up is necessary.