Abstract

A 91-year-old man had a node and erythema in the anal area resistant to treatment. A biopsy of the node in the anus showed atypical cells developing as Paget's disease, and staining revealed that the cells were CK7-positive, CK20-positive, and GCDFP15-negative. Therefore, tumor invasion with pagetoid spread (PS) from the anus to the skin was suspected, and the patient was referred to our department for a close examination and surgical treatment. Lower gastrointestinal endoscopy showed edematous, hemorrhagic mucosa in the anal canal, and he was diagnosed with adenocarcinoma via a biopsy. Additionally, redness and swelling with white moss were observed on the skin around the anus. Biopsy showed that Paget cells were diffusely present in the epithelium, and an image of squamous cell carcinoma directly under the epithelium was obtained. Taken together, the patient was diagnosed with the invasion of anal canal cancer with PS to the skin, and we performed laparoscopic abdominoperineal resection and skin carcinoma resection in the perineum. The histopathological analysis showed adenocarcinoma invading the external anal sphincter and subcutaneous adipose tissue in the vicinity of the pectinate line of the anal canal. Pagetoid spread of the adenocarcinoma was observed in the epidermis, and the open portion was slightly invaded up to the rectal mucosa. The anal skin region of the adenocarcinoma partially continued to the hair follicles, and it was complicated by squamous cell carcinoma invading the dermis. There are a few reports of anal canal cancer with PS, and the coexistence of adenocarcinoma and squamous cell carcinoma, as seen in the present case, is rare. We report our case together with relevant literature.

Highlights

  • Extramammary Paget’s disease (EMPD) is classified into primary and secondary forms

  • We identified four cases of Paget’s disease/EMPD complicated by invasive squamous cell carcinoma [3, 19, 20]

  • We performed immunostaining before surgery and diagnosed the patient with anal canal cancer with pagetoid spread (PS) complicated with squamous cell carcinoma

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Summary

Introduction

Extramammary Paget’s disease (EMPD) is classified into primary and secondary forms. Primary extramammary Paget’s disease is a skin disease in which carcinoma cells from skin appendages such as the perineum, perianal area, scrotum, penis, and axilla invade the epidermis. Extramammary Paget’s disease presents with erythematous plaques in elderly men and women [1]. Secondary EMPD with pagetoid spread (PS) is tumor invasion into neighboring organs around the epidermis due to intraepithelial progression [2]. Extramammary Paget’s disease is a rare condition, and a populationbased study reported an incidence rate of 0.11 per 100,000 person-years [1]. We report a rare case of anal canal cancer with PS, accompanied by squamous cell carcinoma

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