Penoscrotal Extramammary Paget's Disease with Multiple Lymph Node Metastasis

describe a case of penoscrotal extramammary Paget’s disease with multiple lymph node metastasis. A 69-year-old man was referred to us with a oneyear history of a painless erythematous skin rash on the penis and scrotum (Fig. 1). He first visited a local clinic and was treated with topical agents. Despite conventional treatment, the lesion extended to the scrotum and penis. The patient initially underwent a punch biopsy and the pathology report came back as EMPD. On physical examination, there was a 2-cm round, firm mass in the left inguinal area. A computed tomography (CT) scan of the abdomen revealed a 2.1-cm round peripheral rim-enhancing lymph node (LN) in the left inguinal area and a 2.8-cm enlarged ovoid lymph node in the right inguinal area (Fig. 2). Ultrasound-guided biopsy of the localized mass in the left inguinal area confirmed the diagnosis of metastatic carcinoma. Before wide excision of the scrotal and penile lesion, mapping biopsies were performed to determine the exact margins of the lesion. However, during the preoperative period, the mass of the right inguinal area increased from 2.8 to 3.58 cm. Therefore, the patient was admitted to the department of oncology and had an extensive cancer work-up. The tumor markers were within normal range: alpha-fetoprotein 1.5 ng/mL (normal range, 0-20 ng/mL), carcinoembryonic antigen 2.6 ng/mL (normal range, 0-5 ng/mL), and prostate-specific antigen 0.712 ng/mL (normal range, 0-3 ng/mL). Routine colonoscopy and esophagogastroduodenoscopy were performed to check for internal malignancy of the lower gastrointestinal track. However, there was no evidence of an underlying internal malignancy. A whole-body positron emission tomography (PET)/CT scan revealed metastatic lymphadenopathy on both inguinal areas and the left external iliac space. No other abnormal hypermetabolic lesion was found (Fig. 3). After the full cancer work-up, the patient underwent wide local excision with both inguinal and left external iliac lymphadenectomy. We performed surgical wide excision of a 16.5 cm × 8.5 cm lesion of the scrotum and penis. The skin defect was covered with a scrotal flap and split-thickness skin graft for the penile shaft. The pathologic findings with immunohistochemical staining revealed extramammary Paget’s disease (Fig. 4), measuring 7 cm × 4.2 cm. Moreover, a 1.5 cm × 1 cm dermal invasion lesion of the penis and scrotum was seen (Fig. 5). All of the surgical safety margins were clear. In this Fig. 1. Preoperative view: a 69-yearold male patient with penoscrotal extramammary Paget’s disease. Penoscrotal Extramammary Paget’s Disease with Multiple Lymph Node Metastasis