Armitage N C, Jass J R, Richman P I, Thomson J P, Phillips R K
St. Mark's Hospital, London, UK.
Br J Surg. 1989 Jan;76(1):60-3. doi: 10.1002/bjs.1800760119.
Paget's disease of the anus is a rare disorder of controversial origin and is frequently associated with malignancy. We studied eight patients and carried out immunohistochemical studies to determine whether particular functional profiles might be indicators of a malignant association. One patient presented with synchronous carcinoma and two developed carcinomas 3 and 10 years after excision of Paget's disease. Five patients underwent wide local excision and have not developed cancer (median follow-up 6 years, range 5-13 years). However, four patients developed recurrent Paget's disease. Immunohistochemical studies showed that in general Paget cells stained positively with CAM 5.2 (a cytokeratin marker), gross cystic disease fluid protein (a marker for apocrine cells), human milk fat globule glycoprotein (HMFG 1 and 2) and carcinoembryonic antigen but negatively for PR3A5 (a marker for colonic goblet cells). Three cases had a staining profile which was quite different from that usually observed and these were associated with malignancy. One showed an antigenic profile more typical of a large bowel carcinoma. Paget's disease of the anus appears to run one of two clinical courses: to develop malignancy; or to recur locally, often on repeated occasions. Wide local excision is the treatment of choice but long-term follow-up is necessary because of the cancer risk. An immunohistochemical staining pattern which is different from usual may indicate a higher malignant risk and/or identify some cases of Paget's disease as representing a downward 'pagetoid' extension from a anorectal adenocarcinoma rather than a true epidermotropic apocrine neoplasm of the perianal skin.
肛门佩吉特病是一种起源存在争议的罕见疾病，常与恶性肿瘤相关。我们研究了8例患者，并进行免疫组化研究以确定特定的功能特征是否可能是恶性关联的指标。1例患者同时患有癌，2例在佩吉特病切除后3年和10年发生了癌。5例患者接受了广泛局部切除，未发生癌症（中位随访6年，范围5 - 13年）。然而，4例患者出现了佩吉特病复发。免疫组化研究表明，一般来说，佩吉特细胞对CAM 5.2（一种细胞角蛋白标志物）、大汗腺囊性病液蛋白（一种顶泌汗腺细胞标志物）、人乳脂肪球糖蛋白（HMFG 1和2）和癌胚抗原呈阳性染色，但对PR3A5（一种结肠杯状细胞标志物）呈阴性染色。3例患者的染色特征与通常观察到的不同，且这些与恶性肿瘤相关。1例显示出更典型的大肠癌抗原特征。肛门佩吉特病似乎有两种临床病程：发生恶性肿瘤；或局部复发，常反复发生。广泛局部切除是首选治疗方法，但由于存在癌症风险，需要长期随访。与通常情况不同的免疫组化染色模式可能表明恶性风险较高和/或确定某些佩吉特病病例代表来自肛管直肠癌的向下“佩吉特样”延伸，而不是肛周皮肤真正的亲表皮性顶泌汗腺肿瘤。
