棘层松解性乳腺外Paget病临床病理观察

Clinicopathological features of acantholytic extramammary Paget disease

目的 提高对棘层松解性乳腺外Paget病(AEMPD)的临床特征和组织病理学的认识。方法 对我院诊治的1例AEMPD患者的临床和病理资料进行回顾性分析,并结合文献进行复习。结果 患者男性,42岁,右侧阴囊、阴茎大片红斑伴瘙痒,界限尚清,表面破溃、糜烂。镜下示:表皮过度角化及角化不全,颗粒层增厚,棘层松解;可见表皮内裂隙形成及绒毛状基底细胞,棘层见散在或呈巢状的肿瘤细胞(Paget细胞)浸润;细胞排列紊乱,异型明显,细胞呈圆形或卵圆形,胞质丰富且嗜酸或透明,核大深染;真皮浅层见较多慢性炎症细胞浸润,未见肿瘤细胞。免疫组化CK7、CK8、CEA和EMA弥漫阳性,CK20、GCDFP-15、HMB-45、MelanA阴性,P63基底细胞阳性,Ki-67增殖指数约70%。结论 AEMPD是少见,出现棘层松解是术后复发的危险因素。对该病的认识有助于避免漏诊和误诊。

Objective To investigate the clinicopathological features of acantholytic extramammary Paget disease(AEMPD). Methods The clinical and pathological data of a patient with AEMPD were analyzed retrospectively with review of literature. Results A 42-year-old male patient presented with a large infiltrating erythematous lesion on the right scrotum and penis with a clear boundary. The surface was ruptured, wet, erosive, dark red, prone to bleeding, and a small amount of exudate and white discharge. Microscopically, the epidermis showed mild parakeratosis, hyperplasia of the spinous layer, scattered or nested large transparent cells(Paget cells) were visible in the epidermis, the cells were atypia, disordered arrangement, and the spinous layer showed loosely-like changes. There were many chronic inflammatory cells in the dermis, and no tumor cells were observed. Immunohistochemically, Paget cells were positive for CK7, CK8, CEA and EMA, but CK20, GCDFP-15, HMB-45 and MelanA were negative;P63 was positive only for basic cell. The MIB-1(Ki-67) labeling index was about 70%. Conclusion AEMPD is a rare pathological type of extramammary Paget disease(EMPD), and the presence of sphinolysis in EMPD is a risk factor for postoperative recurrence, deepening the understanding of the disease will help to avoid missed diagnosis and misdiagnosis.