Department of Urologic Oncology, University of Texas M. D. Anderson Cancer Center, Houston, Texas, USA.
J Urol. 2011 Jul;186(1):97-102. doi: 10.1016/j.juro.2011.02.2685. Epub 2011 May 14.
Extramammary Paget's disease is a rare intra-epithelial malignancy that is occasionally associated with an invasive adenocarcinoma component as well as other secondary cancers. We investigated a consecutive series of patients referred for extramammary Paget's disease of the penis or scrotum at a single center in the contemporary era to determine the presence of secondary cancers and treatment outcomes.
Between December 1990 and February 2009, 20 patients with extramammary Paget's disease of the scrotum and/or penis were seen. Patients were investigated with computerized tomography of the abdomen/pelvis, chest x-ray, cystoscopy, colonoscopy and serum prostate specific antigen measurement to rule out obvious secondary cancer. Clinical charts were reviewed with institutional review board approval. Mean followup of treated cases was 48 months (range 7 to 208).
Patients were 50 to 86 years old with a history of symptoms of between 6 months and 10 years. Eight patients had invasive adenocarcinoma, of whom 2 died of the disease and 3 who had disease at 5, 7 and 40 months, respectively, were undergoing multimodal therapy. No patient with confirmed intra-epidermal disease only died of the disease. Invasive disease predicted nodal and metastatic progression.
Surgical resection to achieve tumor-free margins resulted in durable relapse-free survival of patients with intra-epidermal extramammary Paget's disease alone. Extramammary Paget's disease with invasion was associated with regional metastatic progression. The latter scenario as well as failure to treat localized extramammary Paget's disease alone was associated with a fatal outcome. Systemic chemotherapy should be further explored in patients with invasive adenocarcinoma or lymph node positive disease.
派杰氏病（Extramammary Paget's disease）是一种罕见的上皮内恶性肿瘤，偶尔与侵袭性腺癌成分以及其他继发性癌症相关。我们调查了在一个单一中心的当代连续系列患者，这些患者因阴茎或阴囊的派杰氏病而被转介，以确定是否存在继发性癌症和治疗结果。
1990 年 12 月至 2009 年 2 月期间，共有 20 名患有阴囊和/或阴茎派杰氏病的患者就诊。对患者进行了腹部/骨盆计算机断层扫描、胸部 X 光、膀胱镜检查、结肠镜检查和血清前列腺特异性抗原测量，以排除明显的继发性癌症。经机构审查委员会批准，对临床病历进行了回顾。经治疗的病例的平均随访时间为 48 个月（7 至 208 个月）。
患者年龄为 50 至 86 岁，症状持续时间为 6 个月至 10 年。8 名患者患有侵袭性腺癌，其中 2 人死于该疾病，另外 3 人分别在 5、7 和 40 个月时患有疾病，他们正在接受多模式治疗。没有仅患有表皮内疾病的患者死亡。侵袭性疾病预测了淋巴结和转移性进展。
对于仅患有表皮内派杰氏病的患者，通过手术切除以实现无肿瘤边缘，可实现持久的无复发生存。伴侵袭的派杰氏病与区域性转移进展相关。在后一种情况下，以及未能治疗单纯的派杰氏病，与致命的结局相关。对于侵袭性腺癌或淋巴结阳性疾病患者，应进一步探索全身化疗。
