Authors: Gen Suzuki, Kazutaka Machida, Akito Asato, Sho Seri, Chika Ikemoto, Hideya Yamazaki, Kei Yamada, Tetsuya Katsumori

Abstract

Penoscrotal extramammary Paget’s disease (EMPD) is a rare intraepithelial adenocarcinoma in older male patients, and no effective nonsurgical treatment strategies are currently available. The aim of this study was to report the usefulness of external radiotherapy (RT) for penoscrotal EMPD in an inoperable elderly patient. This report presents the treatment of an 89-year-old man with widespread penoscrotal EMPD. A multidisciplinary treatment team decided on radical RT. The patient received a radiation dose of 61.8 Gy in 30 fractions through electron and photon beams. His treatment tolerance was good, and no severe toxicity had been observed up the last follow-up. At 6.5 years after the RT, the patient showed no evidence of recurrence. Definitive RT resulted in excellent disease control and minimal toxicity; thus, it could be a promising nonsurgical therapeutic option for penoscrotal EMPD, even in extremely elderly individuals.

Introduction

Extramammary Paget’s disease (EMPD) is a rare neoplasm of the skin that generally affects the anogenital area. Complete surgical removal of the localized disease is the chosen treatment for EMPD.1 Surgical excision carries the risk of anesthesia-related complications and functional decline, making it difficult to indicate, especially in the elderly. Nonsurgical interventions have also been investigated currently, with varied results.

Radiotherapy (RT) has been suggested as a less invasive and potentially curative treatment, with a lower risk of adverse effects, but the existing published evidence has several limitations. Most articles report small-scale retrospective studies, referring to patients treated with large heterogeneities in terms of RT dose and technique. Moreover, many reports lack long-term observations after treatment and therefore lack information about the true effects of definitive RT.

Herein, we present the case of an elderly patient with penoscrotal EMPD treated with definitive RT who attained long-term complete response without serious toxicity.

Case Report

An 89-year-old man presented with an erythematous patch on his penoscrotal skin. He first noticed the skin lesion 5 years before the consultation, expanding gradually. Topical moisturizing creams did not improve the lesion. On local examination, an erythematous patch of 8 cm in the penoscrotal region was observed. He did not report any itching or tenderness. A skin biopsy revealed the invasion of Paget’s cells into the dermis. Neither regional lymphadenopathy nor distant metastases were detected on computed tomography. A discussion with the patient and his family revealed their preference for a nonsurgical treatment strategy because of the patient’s age. We decided to administer electron beam therapy, which could eradicate a tumor with minimal adverse reactions in normal tissue such as the rectum and bladder. Informed consent was obtained from the patient and his family.