阴茎阴囊Paget氏病9例报告

目的 提高对阴茎阴囊Paget氏病的认识,做到早期诊断,减少误诊率,提高治疗效果.方法 本组阴茎阴囊Paget氏病患者共9例,年龄55～83岁,平均69岁.病程6个月～11年.主要临床表现为阴茎阴囊皮肤瘙痒、红斑、糜烂、渗液,可见局部皮肤表面粗糙、皮损逐渐扩大,稍隆起.Ray分期标准,A1期5例,A2期3例,B期1例.9例均行手术治疗,距病灶边缘2.0 cm切除病变区域,深度达深筋膜,术中快速冷冻切片检查,肿瘤细胞累及切缘者1例,即予以扩大切除范围.1例术后病理发现肿瘤累及切缘,随即再次手术治疗.结果 9例均经病理学检查确诊.切口Ⅰ期愈合7例,Ⅱ期愈合1例,切口感染1例,经换药后治愈.术后6例获随访,随访时间1～10年.1例术后1年出现局部复发,复发灶位于原手术切口,再次手术治疗后痊愈.死亡2例,死亡原因均与本病无关.结论 对阴茎阴囊皮损的可疑Paget氏病患者应尽早行组织学活检,确诊后行病灶根治性切除术.本病预后良好.     

Extramammary Paget's disease of the perianal region

Objective Perianal Paget's disease (PPD) is a rare entity. The standard treatment for either in situ or invasive extra mammary Paget's disease (EMPD) is surgical excision. Local recurrence and morbidity from surgery, especially in the elderly, can, however, be high. The aim of this article is to review our experience with PPD and question the currently preferred treatment approaches in light of its histopathology and therapeutic outcome. Patients and methods A chart review of our patients with PPD from 1996 to 2002 was carried out to determine their outcome after treatment. Data from review of the literature are presented. Results Five patients with in situ disease (four females, median age 68 years) were diagnosed as having PPD. A complete surgical excision was attempted in 4 patients and the fifth was treated by photodynamic therapy. At present, all patients are alive, two are free of disease, one has persistent disease and two have local recurrence. Conclusion Considering the significant rate of recurrence even after wide local excision, the extent of surgery needed and the good prognosis with long‐term survival, we question whether nonsurgical modalities should be considered in place of surgery as primary treatment for noninvasive PPD, with radical surgery being reserved for failures or invasive disease.     

Extramammary Paget's disease with invasive adenocarcinoma of the penoscrotum: Case report and systematic review

Extramammary Paget's disease (EMPD) with an invasive adenocarcinoma of the penoscrotum is rare. In addition, high misdiagnosis and delayed diagnosis rates were also noted. In this article, we present a new case and a literature review to analyze the relationship between an internal malignancy and this disease. A 58-year-old man presented with a nonhealing pruritic and erythematous lesion on the right side of penile shaft and scrotum for 1 year. A provisional diagnosis of dermatitis was made by a dermatologist. A biopsy with immunohistochemical staining revealed Paget's disease with an invasive adenocarcinoma. Thorough preoperative systematic surveys showed no abnormality. The patient subsequently underwent wide local excision with negative margins and reconstruction with a split-thickness skin graft on the right side of the penile shaft and scrotum. Postoperatively, the wound healed well, and there was no obvious sign of local recurrence 5 months after the surgery. There is a high rate of misdiagnosis of EMPD. An internal malignancy related to a gastrointestinal cancer is rare in EMPD. Early diagnosis with a specific biopsy and adequate frozen section-guided wide excision contribute to a good prognosis for EMPD.     

Extramammary Paget's disease of the axilla: an unusual case

Extramammary Paget's disease is a rare lesion, often involving the skin of the genital or perianal regions. Less commonly, it has been reported to affect the skin of the axilla. There are very few other cases of extramammary Paget's disease reported in the literature, and the appropriate use of newer techniques such as magnetic resonance imaging and sentinel lymph node biopsy in this setting is not well-studied. We present a case of extramammary Paget's disease of the axilla, and discuss the known literature regarding this rare disease.     

Extramammary Paget's disease arising in mature cystic teratoma of the ovary.

We report a case of extramammary Paget's disease in ovarian mature cystic teratoma. The patient was a 70-year-old Japanese woman who complained of lower abdominal pain. Examination showed elevation of carcinoembryonic antigen and CA 19-9. Ultrasonography and computer tomography revealed a cystic tumor of the left ovary. The gross appearance of the resected ovary was typical for mature cystic teratoma. Microscopic observation revealed a lesion of Paget's disease within the squamous epithelium. The tumor cells had intracytoplasmic mucin and positive immunoreactivity for carcinoembryonic antigen, epithelial membrane antigen, and cytokeratin; but they were negative for S-100 protein and vimentin. On multiple and serial sections, underlying adenocarcinomas were not found either in the ovary or other primary sites. From these pathological findings, we concluded that the disease was an intraepithelial adenocarcinoma, possibly derived from multipotential cells in squamous epithelium of ovarian mature cystic teratoma. This is the first reported case, to our knowledge, of extramammary Paget's disease arising in mature cystic teratoma of the ovary.     

Paget病19例

目的 提高对Ｐａｇｅｔ病的诊治水平。方法 回顾性分析１９８７年至１９９７年收治的１９例Ｐａｇｅｔ病的临床表现及诊治方法。结果 本组乳腺Ｐａｇｅｔ病６例,乳腺外１３例,其中阴茎,阴囊１１例,腹股沟处１例,肛周肛管１例。随访时间０．５－１１年,平均５年４个月,随访中３例复发者,其截端切片病理检查均可见病变组织,另１例死于其他疾病。     

Extramammary Paget's disease of the breast: an unusual location with prognostic implications

Extramammary Paget's disease is a neoplastic process of intraepidermal origin; its management and prognosis are very different from those of mammary Paget's disease. We present a case of extramammary Paget's disease located in the breast. This is an exceptional location, whose differential diagnosis is important because of its therapeutic and prognostic implications.     

Total arthroplasty in Paget's disease of the hip. A clinical review and review of the literature

The results in 30 Paget's disease patients with 37 affected hips treated with total hip arthroplasty (THA) for symptomatic coxarthrosis were compared with the results of previously reported series. Metabolic activity of the disease subsequent to surgery seemed to have no effect on the clinical outcome during 7.8 years of follow-up study; location of the disease (acetabulum only, femur only, or both) or the presence of protrusio alone also had no effect. For the femur, failure rates were similar for prostheses implanted in Pagetic or non-Pagetic bone. Good results can be anticipated for patients with Paget's disease treated with cemented THA.     

Paget's disease of the vulva. A histogenetic study of five cases including ultrastructural observations and review of the literature.

Five cases of extramammary Paget's disease of the vulva were studied by histologic, ultrastructural, and immunologic methods. In one of these cases, the associated invasive adnexal carcinoma was studied in a similar fashion. In all five cases, carcinoma-in situ of the sweat glands was observed. In two cases, the glands were identified as aporcrine. In the other three it could not be determined whether they were apocrine or eccrine. In one case tumor cells were seen extending from a focus of underlying carcinoma-in-situ of sweat glands through the excretory duct into the surface epithelium. The ultrastructural findings indicated that the Paget's cells as well as the cells of the invasive carcinoma were adenocarcinoma cells, but the findings were not conclusive as to whether the cells were apocrine or eccrine. There was no evidence of origin of the Paget's cells from keratinocytes. We believe that the bulk of evidence favors an extraepidermal origin for extramammary Paget's disease. The tumor is most commonly derived from the secretory portion of sweat glands, probably either of apocrine or eccrine types, or the dermal or poral portion of the sweat duct. Some cases of Paget's disease are derived from other types of glands.