Extramammary Paget’s disease of the perianal region treated successfully with radiotherapy

Dear Editor: Extramammary Paget’s disease (EMPD) is a relatively rare cutaneous neoplastic disorder that mainly affects apocrine sweat gland-rich areas such as the vulva, perianal region, scrotum, penis, and axilla [1]. When EMPD affects the perianal region, it is called perianal Paget’s disease (PPD). The disease generally presents between the 5th and 8th decades, and is more common in females than males. PPD can be categorized as primary (the majority of cases), occurring as a primary adenocarcinoma in the epidermis, or secondary, coming from epidermotropic spread or metastasis of an underlying neoplasm with incidence ranging from 33 to 86 % [2]. Thus, penile-scrotal EMPD can be associated with genitourinary cancers, whereas PPD can be associated with gastrointestinal malignancies, mostly rectal adenocarcinoma. Therefore, therapeutic management is highly related to the presence of an underlying malignancy and possible regional or systemic spread. Wide excision of the lesion with a margin of at least 3 cm beyond the affected area with reconstruction of the tissue defect or abdominoperineal excision is the standard modality of the treatment. Despite the aggressive nature of the procedures, PPD has recurrence rates of 44–60 % [3]. The reason behind these high recurrence rates is the multicentric nature of PPD, which can be misleading during the intrasurgical frozen section. Mohs micrographic surgery which represents an alternative surgical method is allows the sparing of more normal tissue than conventional surgery. Moreover, it has shown promise in reducing recurrence rates, although they have still been reported at 27 %. However, many patients are not eligible for such aggressive surgical procedures because of older age and medical contraindications. Moreover, patients may refuse the surgery because of the risk of functional impairment and cosmetic disfigurements. In these cases, radiotherapy could be considered as a reliable alternative therapy. A 65-year old female was admitted to the gastroenterology outpatient clinic with a 1-year history of intermittent pruritus and redness in the perianal region. The patient had neither genitourinary nor gastrointestinal symptoms, such as rectal bleeding, change in bowel function, hematuria, dysuria, urinary frequency, or weight loss. She had a surgical history of gastric fundoplication and laparoscopic cholecystectomy 6 years previously. Her family history was negative for skin, colorectal, or genitourinary cancer. Clinical examination revealed well-circumscribed erythematous plaques with whitish superficial exudates, extending to the vulva. There was no evidence of an underlying rectal cancer with rectal examination. No enlarged inguinal lymph nodes were detected. The patient was referred to the dermatology clinic, where a perianal biopsy was made with differential diagnosis, as Bowen disease, vulvar intraepithelial neoplasia (VIN), lentigo maligna, malignant melanoma, EMPD, and epidermoid carcinoma. Histological examination revealed pagetoid cells that were positive for CEA (carcinoembryonic antigen), EMA (epithelial membrane antigen), and c-erbB2, compatible with EMPD. To exclude underlying malignancy, the patient was advised to undergo a screening colonoscopy, gynecological ultrasonography, mammography, and also whole-body CT, which revealed unremarkable results. All laboratory data including carcinoembrionic antigen (CEA) were normal. Although it was explained to the patient that the surgical B. Yasar (*) Department of Gastroenterohepatology, Haydarpasa Numune Training and Research Hospital, Istanbul, Turkey e-mail: drbyasar@yahoo.com