BACKGROUND: Extramammary Paget's disease (EMPD) is an uncommon skin tumor that usually occurs in the genital area. Few studies on EMPD have been conducted. OBJECTIVE: To evaluate the clinical and pathologic features, treatments, recurrence, survival rates, and prognostic factors of EMPD. METHODS: A total of 35 (27 men and 8 women) patients with EMPD was analyzed. The average age of the patients was 73.4 years. RESULT: Twenty nine Of the 35 patients had lesions in the genital area, one in the genital and perianal area, three in the axillary area and two in the perianal area. Eighteen patients had in situ lesions, five had inguinal lymph node metastases and two had distant metastases at the time of diagnosis. Surgical resection was performed in 30 cases and radiation therapy, was administered in three cases. Six patients died of EMPD, and the overall 5 year survival rate was 75%. CONCLUSION: The presence of a nodule on the primary lesion, clinically palpable lymph nodes, the level of tumor invasion, and lymph node metastases were found to be significant prognostic factors in the 35 EMPD cases at our institution.
BACKGROUND: Extramammary Paget's disease (EMPD) is an uncommon skin tumor that usually occurs in the genital area. Few studies on EMPD have been conducted. OBJECTIVE: To evaluate the clinical and pathologic features, treatments, recurrence, survival rates, and prognostic factors of EMPD. METHODS: A total of 35 (27 men and 8 women) patients with EMPD was analyzed. The average age of the patients was 73.4 years. RESULT: Twenty nine Of the 35 patients had lesions in the genital area, one in the genital and perianal area, three in the axillary area and two in the perianal area. Eighteen patients had in situ lesions, five had inguinal lymph node metastases and two had distant metastases at the time of diagnosis. Surgical resection was performed in 30 cases and radiation therapy, was administered in three cases. Six patients died of EMPD, and the overall 5 year survival rate was 75%. CONCLUSION: The presence of a nodule on the primary lesion, clinically palpable lymph nodes, the level of tumor invasion, and lymph node metastases were found to be significant prognostic factors in the 35 EMPD cases at our institution.
Authors: Maryam Pourmaleki; Jonathan H Young; Nicholas D Socci; Sarah Chiang; Marcia Edelweiss; Yanyun Li; Mianlei Zhang; Lev Roshal; Dennis S Chi; Klaus J Busam; Ingo K Mellinghoff; Travis J Hollmann Journal: Oncotarget Date: 2019-10-22