光动力疗法治疗乳腺外Paget's病28例

Photodynamic Therapy of 28 Cases of Extramammary Paget's Disease

目的总结乳腺外Paget's病(Extramammary Paget's disease,EMPD)的临床病理特征,比较光动力疗法与手术治疗的治疗效果、复发情况、复发时间及生存情况。方法分析总结2002年至2013年EMPD患者28例,17例选择手术作为初始治疗,其中3例术后接受了光动力治疗(photodynamic therapy,PDT),1例接受了术后辅助放疗;9例进行PDT治疗;2例进行了化疗。结果 (1)手术治疗后1个月,17例患者临床症状均消失,病灶100.0%完全缓解(complete response,CR);美容效果2例非常满意,11例满意,2例一般,2例不满意。3例接受皮瓣转移的患者出现了皮瓣成活不良,皮缘坏死,需再行清创术及植皮术。随访期间2例复发。(2)9例PDT治疗患者,1例确诊后1个月出现消化道症状,经直肠镜检查证实结直肠腺癌转外科手术治疗。余8例在治疗后1个月:3例患者症状消失,病灶CR。4例患者症状明显改善,其中3例病灶部分缓解(partial response,PR);1例好转(mild relief,MR)。1例症状改善,病灶PR。美容效果:5例非常满意,3例满意。对未达到CR的患者进行再PDT治疗局部病灶均能得到较好的控制,6例达到CR,2例PR,所有患者均未出现肛门挛缩引起的排便困难及肛门括约肌、感觉神经受损引起的大便失禁,均对术后生活质量满意。但所有CR患者在随访过程中均复发,治疗到复发的时间分别是6、14、18、32、33和88个月。随访过程中2例肛周EMPD合并结直肠腺癌,1例外生殖器EMPD合并膀胱癌。经综合治疗后1例肛周、1例外生殖器EMPD分别于起病后32和38个月死亡,另1例并发内脏肿瘤的患者目前随访33个月存活。(3)2例选择了化疗作为初始治疗,症状均改善。其中,病灶1例MR、1例稳定(stable disease,SD),美容效果均为满意。结论 PDT治疗肛周EMPD能够缩小手术范围,为患者手术打下良好的基础,可作为一种有效的治疗方法。

Objective To summarize the clinical and pathological features of EMPD and introduce the therapeutic effect, recurrence, complications and survival time of several feasible methods. Methods By retrieving electronic medical records and follow-up data of patients whose first diagnosis was Extramammary Paget＇s disease, we summarized the clinical and pathological features of EMPD and introduced the therapies, therapeutic effect and complications for EMPD. Results Totally 28 （25 men and 3 women） patients with EMPD were analyzed. The average age of the patients was 66. 5 （range 32- 80） years old. Most lesions were located in apocrine glands intensive regions, like the external genitalia, anus and perineum. Clinical- ly, the lesions usually present as well-defined, moist and erythematous plaques accompanied by pruritus. Paget＇s ceils were found in all the histopathological examinations of the tissue specimens. As the primary treatment, 17 patients received surgical treatment, 3 patients photodynamic therapy and 1 patient radiotherapy after surgery. All these 17 patients got CR and their clinical symptom disappeared three months after the treatment. 2 patients were very satisfied with the cosmetic results, 11 patients satisfied, 2 patients ambiguous, and 2 patients dissatisfied. 3 patients who accepted flap plasty had flap necrosis and needed further debridement and skin grafting. In 2 cases, EMPD recurred during the follow-up. 9 patients received PDT treatment as a primary treatment, but one was found with a con- comitant signet-ring cell carcinoma of the colon in routine colonoscopy one month after the first PDT treatment and altered to receive ab- dominoperineal resection and had an artificial anus. As to the remaining 8 patients, 3 patients got CR with symptom gone. Symptoms of 4 patients were improved significantly, 3 of them being PR and 1 MR. One patient got s symptomatic improvement. 5 patients are very satisfied with the cosmetic results, and 3 satisfied. The remaining lesions were given PDT for the second time even the third time. At last, 6 patients got CR, 2 PR. However, patients got CR relapsed at Month 6, 14, 18, 32, 33 and 88 respectively. During the follow-up, 2 patients of perianal EMPD were found with metachronous colorectal adenocarcinoma, and a scrotum EMPD patient with metachronous bladder cancer. One patient of perianal EMPD and one patient of scrotum EMPD died in Month 32 and 38 respectively after the comprehensive treatment. Two patients received chemotherapy as a primary treatment： one got MR, and the other SD. Both of them were satisfied with the cosmetic results. Conclusions Wide surgical excision in combination with intraoperative margin control is a recommended treatment for non-invasive and non-perianal EMPD. Boasting reasonable response rates and minimal morbidity, PDT is a feasible treatment option for PPD （ Perianal Paget＇s disease）.