Department of Dermatology, Asahikawa Medical University, Midorigaoka-Higashi 2-1-1-1, Asahikawa, Japan.
Dermatol Surg. 2012 Dec;38(12):1938-44. doi: 10.1111/j.1524-4725.2012.02584.x. Epub 2012 Sep 18.
Extramammary Paget's disease (EMPD) is an uncommon skin tumor that usually occurs in the genital area. Few studies on EMPD have been conducted.
To evaluate the clinical and pathologic features, treatments, recurrence, survival rates, and prognostic factors of EMPD.
A total of 35 (27 men and 8 women) patients with EMPD was analyzed. The average age of the patients was 73.4 years.
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%.
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.
派杰氏病（EMPD）是一种罕见的皮肤肿瘤，通常发生在生殖器区域。目前针对 EMPD 的研究较少。
评估 EMPD 的临床和病理特征、治疗方法、复发率、生存率和预后因素。
分析了 35 例（27 名男性和 8 名女性）EMPD 患者。患者的平均年龄为 73.4 岁。
35 例患者中有 29 例病变位于生殖器区域，1 例位于生殖器和肛周区域，3 例位于腋窝区域，2 例位于肛周区域。18 例患者为原位病变，5 例患者在诊断时出现腹股沟淋巴结转移，2 例患者出现远处转移。30 例患者行手术切除，3 例患者行放射治疗。6 例患者死于 EMPD，总 5 年生存率为 75%。
本研究中，原发灶出现结节、临床可触及淋巴结、肿瘤侵袭程度和淋巴结转移是影响 EMPD 患者预后的重要因素。
