ORIGINAL ARTICLE   

Gazzetta Medica Italiana - Archivio per le Scienze Mediche 2022 January-February;181(1-2):26-33

DOI: 10.23736/S0393-3660.19.04222-0

Copyright © 2019 EDIZIONI MINERVA MEDICA

language: English

Extramammary Paget’s disease: a clinical features analysis of 31 patients in China

Dong ZENG ¹, Haixia LONG ¹, Dan MA ², Xianghua ZENG ¹, Xingxing SU ¹, Yake HUANG ³, Shicong ZHU ¹, Bo ZHU ¹ ✉

¹ Institute of Cancer, Xinqiao Hospital, Third Military Medical University, Chongqing, China; ² Department of General Surgery, Xinqiao Hospital, Third Military Medical University, Chongqing, China; ³ Department of Obstetrics and Gynecology, Xinqiao Hospital, Third Military Medical University, Chongqing, China

BACKGROUND: Extramammary Paget’s disease (EMPD) is a rare skin malignancy and the incidence of EMPD is relatively low in China, so many young doctors know little about it. Therefore, it is essential to investigate the clinical features, diagnosis and treatment of EMPD and to improve the understanding of the disease.
METHODS: The clinical data of 31 cases of EMPD admitted to the Second Affiliated Hospital of the Army Medical University, Chongqing, China, from 2004 to 2018 were retrospectively reviewed and analyzed.
RESULTS: The age of the patients ranged from 28 to 75 years, and the mean age was 60.6 years old. EMPD was mainly seen in males (77.4%) and occurred in the external genitalia. The main clinical symptoms were red plaque-like (58.1%), eczema-like changes (41.9%) with itching (77.4%), often misdiagnosed as dermatitis (41.9%) and eczema (41.9%). The time from onset to pathological diagnosis was between 1 month and ~30 years. In the patients who were examined by immunohistochemistry, all Paget cells expressed EMA (6/6), CEA (5/5) and 35ßH11 (5/5). The vast majority of patients were treated surgically (87.1%). A total of 24 (77.4%) patients were followed up for a period of 1 month ~9 years, and a total of 8 patients relapsed.
CONCLUSIONS: EMPD is a rare disease in China, and its diagnosis relies on histopathological biopsy. A variety of comprehensive treatments, such as topical drugs, radiotherapy, chemotherapy, photodynamic therapy, and surgery can be used, but surgical excision is the standard treatment. EMPD is prone to recurrence and should be followed-up for long-term monitoring.

KEY WORDS: Paget disease, extramammary; Diagnosis; Therapeutics; Surgical procedures, operative