Shu Bo, Shen Xu-Xia, Chen Peng, Fang Xin-Zhi, Guo Yong-Lian, Kong Yun-Yi
Department of Urology, Central Hospital of Wuhan, Wuhan 430014, People's Republic of China.
Department of Pathology, Fudan University Shanghai Cancer Center, Shanghai 200032, People's Republic of China; Department of Oncology, Shanghai Medical College, Fudan University, Shanghai 200032, People's Republic of China.
Hum Pathol. 2016 Jan;47(1):70-7. doi: 10.1016/j.humpath.2015.09.005. Epub 2015 Sep 25.
To investigate the clinicopathological and immunohistochemical features and prognostic factors for invasive extramammary Paget disease (EMPD) on penoscrotum, we described the clinical presentations, histopathology, and follow-up courses of 41 cases. The age of the patients ranged from 42 to 84 years. All the patients were treated with wide surgical excision, and 14 were confirmed to have lymph node metastasis. During follow-up, 18 patients (43.9%) developed local or distant recurrence, and 13 patients (31.7%) died of the disease. Histologically, glandular formation with true lumina within the epidermis was found in 29 cases, and signet ring cells were seen in 11 cases. In invasive components, nodular/micronodular growth pattern, glandular formation, and strands/solid sheets existed in 95.1% (39/41), 43.9% (18/41), and 24.4% (10/41) of the cases, respectively. More than half of the cases had at least 2 different types of invasive growth pattern. CK7 was diffusely positive in all cases, whereas CK20 was focally positive in 8 cases. GCDFP-15 was expressed to a variable degree in 24 cases. Presence of strands/solid sheets, lymphovascular invasion, and perineural invasion in invasive EMPD were found to be correlated with higher lymph node metastatic rate. Univariate analysis revealed that patients with one of the following prognostic factors: delay in diagnosis more than 7.5 years, depth of invasion more than 1 mm, invasive pattern of strands/solid sheets, marked inflammation, lymphovascular invasion, and lymph node metastasis at diagnosis, had significantly shorter cancer-specific survival. We concluded that invasive EMPD is a rare malignant skin neoplasm with morphological diversity. Invasive pattern of strands/solid sheets is significantly associated with both lymph node metastasis and worse prognosis. Delay in diagnosis, depth of invasion, marked inflammation, lymphovascular invasion, and regional lymph node status are important prognostic factors.
为了研究阴茎阴囊浸润性乳腺外佩吉特病（EMPD）的临床病理、免疫组化特征及预后因素，我们描述了41例患者的临床表现、组织病理学及随访过程。患者年龄范围为42至84岁。所有患者均接受了广泛手术切除，其中14例证实有淋巴结转移。随访期间，18例患者（43.9%）出现局部或远处复发，13例患者（31.7%）死于该病。组织学上，29例在表皮内发现有真性管腔的腺管形成，11例可见印戒细胞。在浸润成分中，结节状/微结节状生长模式、腺管形成及条索状/实性片状结构分别存在于95.1%（39/41）、43.9%（18/41）及24.4%（10/41）的病例中。超过半数的病例至少有2种不同类型的浸润性生长模式。CK7在所有病例中均弥漫性阳性，而CK20在8例中局灶性阳性。GCDFP - 15在24例中呈不同程度表达。浸润性EMPD中条索状/实性片状结构的存在、淋巴管侵犯及神经周围侵犯与较高的淋巴结转移率相关。单因素分析显示，具有以下预后因素之一的患者：诊断延迟超过7.5年、浸润深度超过1mm、条索状/实性片状浸润模式、显著炎症、淋巴管侵犯及诊断时淋巴结转移，其癌症特异性生存期显著缩短。我们得出结论，浸润性EMPD是一种形态多样的罕见恶性皮肤肿瘤。条索状/实性片状浸润模式与淋巴结转移及较差预后均显著相关。诊断延迟、浸润深度、显著炎症、淋巴管侵犯及区域淋巴结状态是重要的预后因素。
