乳腺外Paget''s病六例的手术治疗

乳腺外Paget’s病（extramammary Paget’s disease．EMPD）在临床上较少见，多发生在男性或女性外生殖器、肛门或腹股沟等部位。从1997-2003年，我科共收治EMPD患者6例，均施行病灶扩大切除＋皮瓣转位或游离植皮修复术，现报告如下。     

阴囊Paget病2例报告

目的提高临床对阴囊Paget病的认识。方法报告2例阴囊Paget病的患者的临床资料。患者临床初期可表现为红色皮疹,以后表现为乳头状增生与溃烂交替出现,表面附有恶臭分泌物。均经病理检查确诊;手术切除病灶。结果2例患者术后痊愈出院。结论阴囊Paget病极易误诊为阴囊皮肤慢性湿疹或炎症,须依靠病理检查确诊;手术切除病灶有一定疗效。     

阴茎阴囊Paget病18例诊治分析

目的：探讨阴茎阴囊Paget病的临床特点和诊治方法,提高对阴茎阴囊Paget病的诊治水平。方法：对18例阴茎阴囊Paget病患者的临床资料进行回顾性分析,全部患者先行患处活检,后行术中快速冷冻切片辅助检查切缘的病灶根治性切除治疗,18例均未行预防性腹股沟浅组淋巴结清扫术。术后未行化疗或放疗。结果：术后18例患者均I期愈合。随访6个月～7年,平均32个月。2例复发,均为真皮层受累患者。其中1例局部复发,接受再次手术治疗,至今存活;1例术后2年发现腹股沟区淋巴结转移,再次手术1年后由于肺转移死亡。1例因其他疾病死亡。结论：阴茎阴囊Paget病应尽早行皮肤活检确诊,该病的首选治疗方法为术中快速冷冻切片辅助检查切缘的病灶根治性手术切除,手术效果好,预后满意,对复发者可行二次手术。     

阴囊Paget病的诊断与治疗(附64例分析)

目的：探讨有关阴囊Paget病的临床特点、预后因素和手术处理原则，提高外科医生对阴囊Paget病的认识。方法：回顾性分析我院1993～2003年收治的64例阴囊Paget病患者的临床资料。结果：104例患者平均年龄68岁，从初发症状到明确诊断的间隔时间平均为2年9个月。除1例采用激光治疗外，其余均采用手术病灶切除术，术后皮肤结构重建，效果满意。术后局部复发8例，其中与该病相关死亡3例。结论：阴囊Paget病容易漏诊，发现阴囊、会阴附近部位皮肤出现可疑的湿疹样病变，应尽早行皮肤活检。早期彻底切除病灶能减少局部复发。     

乳腺外佩吉特病七例报告

目的 对乳腺外佩吉特（Paget)病的临床表现、治疗方法及其预后进行分析。方法 对收治的7例乳腺外Paget病例资料进行回顾性分析。结果 3例有腹股沟淋巴结转移的患者均在5年内死亡,无腹股沟淋巴结转移者均存活。结论 乳腺外Paget病首选手术切除。一旦有淋巴结转移,其5年生存率极低。     

Mohs手术联合光动力治疗乳房外Paget病的临床观察

目的:观察Mohs手术联合光动力治疗乳房外Paget病的临床疗效。方法:8例确诊为乳房外Paget病患者纳入治疗观察,术前皮疹部位用ALA封包,Wood灯下标记手术边界,运用Mohs手术切除病灶,伤口拆线后每隔10d光动力治疗1次,总计治疗4次,随访半年观察其疗效。结果:纳入治疗的患者伤口均甲级愈合,未出现感染等并发症,随访半年无复发,外阴部形态结构功能无明显影响,患者就医满意度高。结论:Mohs手术联合光动力治疗外阴部位乳房外Paget病效果较好,病灶切除干净,患者术后恢复快,肿瘤复发率低,可作为外阴部乳房外Paget病的首选治疗方案。     

阴囊Paget病19例临床分析

目的：探讨阴囊paget病的临床特点、诊治方法及预后,提高对阴囊Paget病的诊治水平。方法回顾性分析了本院1993年2月至2013年2月间收治的19例阴囊paget病患者的病例资料并随访。除1例因腹股沟淋巴结及远处广泛转移未行手术治疗外,其余18例均行根治性病灶切除术,术前均已行患处活检证实。结果19例患者住院8~42天,平均住院18天。18例术后均行病理学检查证实为 Paget病。术后16例获随访,随访时间0.5~11年。1例在术后1年出现复发,再次行手术治疗。1例远处淋巴结转移者未行手术治疗,随访8月后死亡,死因为Paget病全身广泛转移。余14例无瘤生存。结论对怀疑为阴囊Paget病的老年男性应尽早行皮肤活检确诊。局部病灶根治性切除治疗无淋巴结Paget病患者复发较少,预后良好。     

乳腺外Paget′s病的诊治附——21例报告及文献复习

目的　探讨乳腺外Paget′s病的诊治方案及其注意事项。方法　分析 2 1例乳腺外Paget′s病的临床资料、诊治经过及随访结果。结果　 2 1例均经病理检查证实。 18例获随访 ,其中 3例手术后 1年复发 ;2例术后 2～ 3年死于广泛转移 ;2例术后 5年死于其他疾病 ;11例 5年无复发。结论　本病易诊为湿疹等 ,对难治性湿疹样改变应早期活检 ,局部广泛切除是本病首选治疗方法     

股前皮瓣治疗外阴部乳腺外Paget’S病一例

乳腺外Paget’s病（Extramammary Paget’s Disease,EMPD）是一种较少见的表皮内腺癌。我科于2009年3月收治一名EMPD患者,现报道如下。     

肛周Paget病伴腺癌1例并文献复习

目的：复习肛周Paget病伴腺癌1例并探讨肛周Paget病的诊疗进展。 方法：回顾2013年3月收治1例81岁女性治疗过程,并复习相关文献。 结果：患者行一期经腹会阴联合切除,术后随访6个月未见复发。肛周Paget病是乳腺外Paget病的一种罕见表型,其诊断主要依靠病理及免疫组化检查。最常用于诊断的免疫标记物是CK7、CK20、 GCDFP-15、HMB45、CAM 5.2、c-erb、S100 protein和EMA。肛周Paget病的治疗取决于局部侵袭的广度和深度,区域淋巴结受侵程度及全身播散情况,分为局部切除、局部扩大切除、腹会阴联合切除和/或腹股沟淋巴结清扫、放疗、化疗、局部姑息治疗等。 结论：肛周Paget病临床极为罕见,诊断及治疗有待制定统一标准。     

A case of coronary artery bypass grafting for effort angina in a patient with post skin grafting for an extramammary Paget’s desease

The extramammary Paget’s disease of anogenital area is rare, and is seen as a precancerous lesion or carcinoma in situ. In most cases, surgical treatment including dissection of regional lymph nodes, skin resection and grafting should be considered first. A 74-year-old male suffered from effort angina and underwent triple coronary artery bypass grafting (CABG) including saphenous vein graft (SVG) harvested from right lower leg. Five years previously he underwent skin grafting for extramammary Paget’s disease of both inguinal areas and the perineal area. The postoperative course was uneventful and he was discharged 23 days after the CABG operation. Extremammary Paget’s disease is localized in epidermis for years and there has been no report of direct invasion into vessels. Therefore, there appears to be no reason not to use an SVG if there is no luminal thrombus or occlusion.     

阴茎Paget病的手术治疗及随访(附10例报告)

目的:研究阴茎Paget病的临床特点并探讨其外科治疗的适宜方法。方法:回顾性分析江苏省人民医院及江苏省省级机关医院2008年至2012年外科治疗10例阴茎Paget病患者的治疗情况及随访资料。结果:所有10例患者都接受了局部扩大切除术,并用阴囊皮瓣或游离皮瓣对缺损处进行修补,手术顺利,术后阴囊皮瓣及游离皮瓣均存活,10例患者均无淋巴结转移。术前与术后1~2个月时的IIEF评分显示患者的勃起功能、性高潮及性生活满意度无显着性差异(P值分别为0.229、0.761和0.801)。结论:当阴茎皮损怀疑Paget病时,应尽早行活检,确诊后尽早行外科治疗。并根据患者病情,选择阴囊皮瓣或游离皮瓣进行修补,能够最大程度地保留阴茎术后形态和功能,是一种理想的首选治疗方法。     

Clinical research of holmium laser therapy in extramammary Paget’s disease

This study aims to investigate the safety and efficiency of the holmium laser therapy in extramammary Paget’s disease. The clinical data of 61 patients was collected since 2002 to 2012, confirmed as non-subcutaneous invasive extramammary Paget’s disease by biopsy and underwent surgery. All patients were divided into two groups. Group A included 30 patients who underwent the holmium laser therapy. Group B included 31 patients who underwent the traditional surgical therapy. The clinical data of all patients included preoperative, intraoperative, and postoperative management and follow-up records. Compared with the traditional operation group, the holmium laser group had a shorter operation time and was easier to perform. There were no significant differences between the two groups in cases of intraoperative and postoperative complications, the recurrence-free survival, and the disease-specific survival. But the holmium laser group had a longer recovery time than the traditional operation group in large and deep nidus. Multiple-factor analysis of prognostic parameters of 61 patients confirmed that any of these two methods chosen was not a prognostic parameter for recurrence-free survival. The holmium laser therapy might prove to be a preferable alternative to the traditional operative therapy of extramammary Paget’s disease. However, the holmium laser therapy did not demonstrate to have an obvious advantage over traditional operative therapy in the recurrence-free survival and the disease-specific survival.     

PSA筛查在会阴Paget病中的价值

目的:探讨前列腺特异性抗原(PSA)筛查在会阴Paget病中的价值。方法:回顾性分析2003年1月~2012年12月在我院治疗的63例会阴Paget病患者临床资料。本组患者术前均行PSA检测,对于血清PSA4μg/L者行经直肠超声引导下前列腺穿刺活检术以明确病理诊断。将本组患者中PSA4μg/L者所占比例和前列腺癌(PCa)的发生率分别与文献报道的一般人群中相应比例进行比较。结果:本组63例患者均经病理证实为Paget病。19例(30.2%)患者肿瘤浸润至真皮层,2例(3.2%)累及提睾肌,4例切缘阳性。术前血清PSA4μg/L者14例,在全组患者中所占比例(22.2%),明显高于一般人群中PSA筛查时血清PSA4μg/L的比例(6.7%)。14例血清PSA4μg/L的Paget病患者中6例(42.9%)经前列腺穿刺活检证实合并有PCa,其发生率远高于PSA4μg/L的一般人群中PCa的发生率(25%~30%)。结论:与一般人群相比,会阴Paget患者更容易发生PCa,其具体机制有待进一步研究。     

阴囊、阴茎Paget病临床病理分析

目的:探讨阴囊、阴茎Paget病的临床病理特点、诊断及鉴别诊断依据。方法:复习13例阴囊、阴茎Paget病的临床病理资料,采用HE染色、特殊染色(AB/PAS)及免疫组化技术进行观察分析。结果:阴囊、阴茎Paget病以老年男性多见,发病年龄55～84岁,中位年龄为71岁。肉眼皮损呈湿疹样改变。光镜下表皮内可见数目不等的Paget细胞呈单个散在、巢状或条索状分布。所有病例AB/PAS染色均阳性;13例均表达CK7、CEA、EMA抗原,不表达CK5/6、S-100及P63,GCDFP-15与CK20阳性表达率分别为76.92%(10/13)和53.85%(7/13)。结论:阴囊、阴茎Paget病为低度恶性的皮肤上皮性肿瘤,有特殊的临床及组织病理学特点,免疫组化标记有助于该肿瘤的诊断。     

Oncoplastic breast surgery combining partial mastectomy with immediate breast reshaping using a keyhole-shaped skin glandular flap for Paget’s disease

Oncoplastic breast surgery (OBS), which combines the concepts of oncologic and plastic surgery, is becoming more common worldwide. We herein report the results of OBS in Japanese patients with Paget’s disease. We performed OBS combining partial mastectomy with immediate breast reshaping using a keyhole-shaped skin glandular flap in two patients. In these two patients, who were diagnosed as having Paget’s disease with a restricted intraductal component in the central area of their non-ptotic breast, we performed oncoplastic surgery combining partial mastectomy with immediate breast reshaping using a keyhole-shaped skin glandular flap. Neither of the two patients received contralateral surgery to produce symmetrical breasts. The observation period ranged from 6 to 12 months, and the bilateral breast volumes and inframammary lines were symmetric. OBS combining partial mastectomy with immediate breast reshaping using a keyhole-shaped skin glandular flap was successfully performed in two patients with Paget’s disease.     

Perianal Paget’s disease: case report and review of the literature

A peri-anal skin lesion, often eczema-like and with symptoms of pruritus, that does not resolve after classical local therapy should be biopsied. We present a case of peri-anal extramammary Paget’s disease (EMDP) and associated anal adenocarcinoma. Reviewing the literature, more than 30% of patients with EMDP present a second primary tumour in their past, present or future history. In Europe, the risk of developing a new primary tumour in patients with this condition is increased compared with the standard population. In cases of peri-anal Paget’s disease (PPD), specific histochemical markers allow us to differentiate between a primary and a secondary form, the secondary one is strongly associated with colorectal and anal tumours. We provide information about the most commonly suggested therapy for PPD with or without associated malignancy and about the recommended follow-up.     

"Pagetoid" eccrine carcinoma of the vulva: report of an unusual case with review of the literature

Sweat gland carcinoma of the vulva is rare and may be classified as being of eccrine, apocrine, or mixed origin. Most reported cases of vulvar sweat gland carcinomas associated with extramammary Paget disease describe a tumor of apocrine origin. We report a case of a vulvar sweat gland carcinoma of eccrine origin associated with Pagetoid extension. A review of the literature and the differential diagnosis are also presented. To our knowledge, this is the second case of vulvar sweat gland carcinoma of eccrine origin associated with extramammary Paget disease.     

Penile and scrotal Paget's disease: 130 Chinese patients with long-term follow-up

OBJECTIVES

To report a retrospective series of 130 Chinese patients with penoscrotal extramammary Paget’s diseases (EMPD), with a long‐term follow‐up, and thus improve the diagnosis and therapy of this disease.

PATIENTS AND METHODS

The history, clinical presentation, pathology, treatment, and prognosis of 130 patients were analysed. All cases were confirmed by skin biopsy, and then the patients had local wide resection to remove the involved skin and subcutaneous tissue. The large defective wound was reconstructed using a split‐thickness skin graft or local flap.

RESULTS

Forty‐five patients were evaluated by frozen‐section biopsy of the margins during surgery, five of whom had positive margins and then had an extended resection immediately. Most of these patients had local skin or adjacent scrotal flaps to cover their skin defects. Of the 130 patients, 81 had a mean (range) follow‐up of 3.2 (0.5–10) years after surgery. Five of nine patients with positive margins and three (4%) of 72 with negative margins had tumour recurrence. Five patients died from metastatic disease.

CONCLUSIONS

Penoscrotal EMPD needs be differentiated from other chronic dermatitis. A 3 cm surgical margin should be sufficient and frozen‐section pathological examinations are necessary for some complicated conditions. Skin grafts or local flaps are good for large skin defects.