Invasive Extramammary Paget's disease of the vulva = Invasive Extramammary Paget's disease of the vulva

다국어 초록 (Multilingual Abstract)

Extramammary Pagets disease is a rare, slow-growing skin neoplasm. Extramammary Pagets disease of the vulva (EPDV) counts less than 1% of all vulvar malignancies. Because of its rarity and unpredictable disease course, diagnosis is delayed. The deep invasion (≥1mm depth) has been reported as poor prognosis because spread through the epidermal basal membrane results in a high percentage of nodal metastasis and a low survival rate. Thus, patients with invasive EMPD can be treated less successful than in situ tumor. Although reports of EPDV have been reported often in dermatology, they have rarely been reported as gynecologic papers in Korea. In order to identify in the clinical course and treatment of EMPD in Korean patients, we present here a case of a 69-year-old woman with DM and HTN, admitted to the gynecological department because of right side of vulvar itching sensation. Prior to our hospital visit, the patient underwent right vulvar lesion biopsy on August 19, 2017 at local gynecology hospital. Histopathologic result of her skin biopsy was reported as compound nevus and there was no evidence of malignancy. At the time of our hospital on June 26, 2018, her Right vulvar lesion was entirely involved and had erythematous patches and nodules with erosion and discolored lesion. Right vulvar lesion excision under general anesthesia was performed again on July 06, 2018. Unlike the previous biopsy, histologic result was reported extramammary Paget disease with 5mm invasion depth. Surgical treatment was performed on July 23, 2018: bilateral vulvectomy without pelvic lymphadenectomy using frozen section analysis of excision margins. The final histopathologic examination revealed an Extra-mammary Paget's disease with focal stromal invasion at right vulva. We are currently undergoing outpatient follow-up due to improved symptoms.