Quinn Adam M, Sienko Anna, Basrawala Zane, Campbell Steve C
Department of Pathology, Loyola University Medical Center, Maywood, IL 60153, USA.
Arch Pathol Lab Med. 2004 Jan;128(1):84-6. doi: 10.5858/2004-128-84-EPDOTS.
The scrotum is an uncommon site for the presentation of extramammary Paget disease (EMPD). We describe a case of EMPD that was discovered in a patient who had been previously diagnosed and treated for squamous cell carcinoma in situ of the scrotum 3 years earlier. Pathologic examination of the current scrotectomy specimen revealed an erythematous patch with areas of pale induration. Microscopic examination revealed areas with the characteristic histology of Paget disease adjacent to areas characteristic of Bowen disease. Immunohistochemical findings demonstrated a strong expression of carcinoembryonic antigen, cytokeratin 7, and low-molecular-weight cytokeratins (CAM 5.2) in both of these areas, giving support to the overall diagnosis of EMPD. High-molecular-weight cytokeratins (34betaE12) were uncharacteristically expressed in the cytoplasm of the Paget cells with equal or greater strength than in the surrounding keratinocytes, suggesting some degree of squamous differentiation. Very few publications have reported the coexistence of EMPD with squamous cell carcinoma in situ, occurring mostly in the vulva. To our knowledge, our case is the first report of scrotal EMPD with features of Bowen disease. Our findings support the theory that primary EMPD arises multifocally from multipotential epidermal cells.
阴囊是乳腺外佩吉特病（EMPD）罕见的发病部位。我们描述了一例EMPD病例，该患者3年前曾被诊断为阴囊原位鳞状细胞癌并接受治疗。对此次阴囊切除标本的病理检查显示有一个红斑区域伴有苍白硬结区。显微镜检查发现，佩吉特病特征性组织学区域与鲍温病特征性区域相邻。免疫组化结果显示，这两个区域癌胚抗原、细胞角蛋白7和低分子量细胞角蛋白（CAM 5.2）均呈强表达，支持EMPD的总体诊断。高分子量细胞角蛋白（34βE12）在佩吉特细胞的细胞质中呈异常表达，强度与周围角质形成细胞相当或更强，提示有一定程度的鳞状分化。很少有出版物报道EMPD与原位鳞状细胞癌共存，这种情况大多发生在外阴。据我们所知，我们的病例是首例具有鲍温病特征的阴囊EMPD报告。我们的发现支持原发性EMPD由多能表皮细胞多灶性起源的理论。
