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。     

中国31例肛周Paget病的临床特点

目的 总结肛周Paget病的临床特点.方法 回顾性分析国内发表的30例肛周Paget病和1例本院的临床资料.结果 发病男女之比为2.4∶1,平均年龄65岁;症状有肛周瘙痒(67.7%),湿疹样改变(54.8%),肛周赘生物(38.7%),糜烂、溃疡(36.1%),肛周分泌物(35.5%),大便习惯改变(22.6%),腹股沟肿块(12.9%),灰白苔样物(12.9%)和红肿(6.5%)等.误诊率高达67.7%,经常被误诊疾病为湿疹(48.4%)、痔(12.9%)和慢性炎症(6.4%).总手术率为90.3%,局部广泛切除占35.5%,Miles术占54.8%.结论 肛周Paget病在中国以老年男性居多,PPD的诊断依赖于典型的临床症状和病理检查;手术切除是最有效的治疗手段;对于远处转移者可辅以放疗和化疗等手段.     

阴囊Paget病18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.方法 回顾性分析我院18例阴囊Paget病的临床、病理资料和预后随访情况.结果 18例患者病理检查均为阴囊Paget病,17例获随访,随访时间术后5个月～8年,2例术后2年死于全身转移,2例术后3年死于心血管疾病,1例术后4年死于肺癌,2例分别在术后2年和3年局部复发,再次行手术治疗,其余术后无复发.结论 阴囊Paget病应及早行皮肤活检确诊,及时阴囊局部广泛切除为首选治疗方式,病变浸润真皮层及淋巴结转移者预后差,局部复发者可行二次手术.     

阴囊Paget病(附7例报告)

目的:提高对Paget病的诊治水平.方法:结合文献对收治的7例阴囊Paget病的病因病理、临床表现、治疗及预后进行讨论.结果:7例均采用阴囊部分切除术,术后2例局部复发,1例出现一侧睾丸、腹股沟淋巴结转移,经再次手术切除治愈;随访6例均健在,1例失访.结论:对于久治不愈的阴囊皮炎或湿疹应尽早行病灶活检,一经确诊应积极手术治疗.     

乳腺Paget病的临床诊治分析

目的探讨乳腺Paget病的诊断和治疗方法。方法回顾性分析7例乳腺Paget病患者临床资料。结果 7例患者乳头、乳晕区破溃、结痂、脱落等湿疹表现者有5例;其中有6例行乳腺改良根治术,1例行单纯乳房切除,随访6⁴8个月,2例淋巴结转移患者分别于术后12个月和34个月出现局部复发,其他患者未见转移复发。结论乳腺Paget病的临床表现较为明显,但在诊断中易误诊,对乳头乳晕区有湿疹样表现者均要考虑本病的可能,单纯的Paget病愈后良好,可行乳房单纯切除;Paget病合并其他类型的乳腺癌患者预后较差,应采用根治性切除术,同时给予相应的辅助治疗。     

阴囊Paget病15例临床资料分析

目的 :探讨阴囊Paget病的临床特点和诊治方法。　方法 :对 15例阴囊Paget病患者的临床、病理资料以及治疗和随访进行回顾性研究 ,全部患者先行患处活检 ,后行手术治疗。　结果 :15例患者病理检查均可见Paget病的典型特征 ;13例获随访 ,随访时间 0 .5～ 13年 ;2例术后 2年死于广泛转移 ,3例术后死于心脑血管病 ,1例术后 3年局部复发 ,再行手术切除 ;其余 7例无复发。　结论 :阴囊Paget病应尽早行皮肤活检确诊 ,局部广泛切除是本病首选治疗方法 ,手术效果好 ,预后满意 ,对局部复发可行二次手术。     

阴囊Paget病12例临床分析

目的探讨阴囊Paget病的临床特点和诊治方法,提高对阴囊Paget病的诊治水平。方法对1990年至2003年收治的12例阴囊Paget病的病理、临床表现、治疗及预后进行回顾性研究,12例患者组织活检确诊后行手术治疗,术中、术后均未行化疗或放疗。结果12例病理检查可见Paget病的典型特征;11例获随访,随访时间1~13年,平均5年3个月;1例淋巴结转移者,术后2年死于广泛转移;1例术后3年局部复发,再行广泛切除,至今17个月未见复发;1例术后5年出现腹股沟淋巴结转移,1年后死于肿瘤转移;余8例无复发。结论阴囊Paget病应尽早行皮肤活检确诊,局部广泛切除是首选治疗方法,切缘阴性是预防复发的关键。     

阴囊Paget病35例报告

目的 探讨阴囊Paget病的诊治. 方法 对35例Paget病患者的临床资料进行回顾性分析.35例患者均接受手术治疗,6例同时行单侧或双侧腹股沟浅组淋巴结清扫术. 结果 术后I期愈合27例.Ⅱ期愈合8例.随访2个月～3年,平均2年.1例因其他疾病死亡.8例出现局部复发.复发者均接受再次手术治疗. 结论 阴囊Paget病极易误诊为阴囊皮肤慢性湿疹等疾病,病理学检查可对该病作出正确诊断,对阴囊湿疹治疗6～8周无效时应作组织病理学检查.该病的首选治疗方法 为手术切除,行根治性切除术可获满意疗效.     

原发性甲状腺恶性淋巴瘤诊治分析

目的提高原发性甲状腺恶性淋巴瘤的诊断与治疗水平。方法回顾性分析1990—2005年收治的14例原发性甲状腺恶性淋巴瘤患者的临床资料。结果12例患者以颈部肿块就诊,2例以声音嘶哑就诊,病程2周至4年。9例行患侧甲状腺全切及对侧大部分切除术,3例行双侧甲状腺全切除术,2例行患侧甲状腺及峡部切除术。术后病理诊断为弥漫性大B细胞淋巴瘤9例;滤泡性淋巴瘤4例;淋巴母细胞性淋巴瘤1例。术后联合放、化疗7例,4例行单纯局部放疗,另外3例未行辅助治疗。随访13例(92.8%),1例淋巴母细胞性淋巴瘤术后10个月死亡,2例术后未行任何治疗的患者分别于术后18,31个月死于肿瘤转移,其余10例随访6-122个月,平均43.2个月,均健在。结论原发性甲状腺恶性淋巴瘤临床及病理易误诊。手术是首选治疗方法,其预后与病理类型、综合治疗方案密切相关,术后辅助化疗对本病的治疗甚为重要。     

负压封闭引流在肛周Paget病术后创面修复中的应用

目的观察负压封闭引流技术结合皮片移植修复肛周Paget病肿瘤扩大切除术后创面的疗效。方法自2009年10月至2013年12月,收治肛周非浸润性Paget病9例,d全身麻醉下行肿瘤扩大切除术;术后取自体一侧股后皮片移植修复创面,负压封闭引流固定皮片,持续负压吸引,定期更换敷料;观察有无感染、血肿等并发症发生,皮片存活情况,术后6个月随访肛门外观及功能重建情况。结果术后7 d,9例患者移植皮片均存活,1例患者术后3 d打开敷料后发现污染。6个月随访时未见肿瘤复发,移植皮片柔软,肛门周围可见自然褶皱,无一例排便功能障碍。结论负压封闭引流技术结合皮片移植用于肛周Paget病术后创面的修复可取得较满意的效果,值得在临床上推广。     

男性乳腺癌临床特征及诊治：附35例报告

背景与目的 男性乳腺癌是一种非常罕见的疾病，一般确诊时分期较晚，预后差。目前其治疗上多参照女性乳腺癌。笔者通过对男性乳腺癌的病例的回顾分析，探讨该病临床特点及诊治。方法 回顾分析大连医科大学附属第二医院于2003年3月—2019年6月收治的35例男性乳腺癌患者的临床特点、病理特征、治疗及随访情况。结果 全组患者就诊时均无远处转移，中位年龄65岁，主要临床表现为无痛性乳房肿块，所有患者均接受了手术治疗，仅2例（5.7%）患者接受了部分乳腺切除术，其余均接受全乳房切除术或更大范围的手术。18例（51.4%）接受了辅助化疗，4例（11.4%）接受了辅助放疗，17例（48.6%）接受了辅助内分泌治疗。30例（85.7%）术后病理诊断为浸润性导管癌。全组均获得随访，5年总生存率83.4%。结论 加强对男性乳腺癌的认识，做到早期诊断，并进行以手术为主的综合治疗非常重要。男性乳腺癌的诊治仍有待进一步研究和规范。     

罗哌卡因局部麻醉下手术治疗早期急性嵌顿痔124例疗效观察

为探讨罗哌卡因局部麻醉下手术治疗早期急性嵌顿痔的疗效,对124例早期急性嵌顿痔患者于罗哌卡因局部麻醉下行手术治疗,对外痔予剥扎切除,内痔予l：1消痔灵注射液黏膜及黏膜下层注射。结果显示,罗哌卡因局部麻醉后约5min肛门内外括约肌松弛,肛门坠胀疼痛缓解或消失,肛门水肿逐渐消退,痔核萎缩,向肛内退缩。发病12～48h的患者痔黏膜无糜烂、坏死,治疗效果更好。术后6h以后肛门疼痛：0级18例,I级61例,Ⅱ级45例。平均治愈时间8．6d。术后尿潴留3例,补充治疗8例。本组治愈121例,好转3例,有效率为100．0％。结果表明,罗哌卡因局部麻醉能够完全满足急性嵌顿痔手术麻醉要求,局部麻醉下手术治疗早期急性嵌顿痔安全有效,不良反应少,疗效肯定,值得临床推广应用.     

Paget's disease of the anus: a clinicopathological study

Paget's disease of the anus is a rare disorder of controversial origin and is frequently associated with malignancy. We studied eight patients and carried out immunohistochemical studies to determine whether particular functional profiles might be indicators of a malignant association. One patient presented with synchronous carcinoma and two developed carcinomas 3 and 10 years after excision of Paget's disease. Five patients underwent wide local excision and have not developed cancer (median follow-up 6 years, range 5-13 years). However, four patients developed recurrent Paget's disease. Immunohistochemical studies showed that in general Paget cells stained positively with CAM 5.2 (a cytokeratin marker), gross cystic disease fluid protein (a marker for apocrine cells), human milk fat globule glycoprotein (HMFG 1 and 2) and carcinoembryonic antigen but negatively for PR3A5 (a marker for colonic goblet cells). Three cases had a staining profile which was quite different from that usually observed and these were associated with malignancy. One showed an antigenic profile more typical of a large bowel carcinoma. Paget's disease of the anus appears to run one of two clinical courses: to develop malignancy; or to recur locally, often on repeated occasions. Wide local excision is the treatment of choice but long-term follow-up is necessary because of the cancer risk. An immunohistochemical staining pattern which is different from usual may indicate a higher malignant risk and/or identify some cases of Paget's disease as representing a downward 'pagetoid' extension from a anorectal adenocarcinoma rather than a true epidermotropic apocrine neoplasm of the perianal skin.     

侵袭性胸腺瘤手术治疗预后因素的随访

目的 观察侵袭性胸腺瘤的手术治疗效果,并分析影响患者预后的相关因素.方法 回顾性分析2000年1月至2009年12月接受手术治疗的59例侵袭性胸腺瘤患者的临床及随访资料.患者为连续病例,男性34例,女性25例;年龄18～72岁,平均49岁.根治性手术44例,姑息切除或活检15例.Masaoka分期:Ⅱ期18例,Ⅲ期30例,Ⅳ期11例;Ⅱ期患者术后未接受辅助放疗或化疗,Ⅲ、Ⅳ期患者术后接受辅助放疗和(或)化疗26例,未接受辅助放疗或化疗15例.分析手术方式、Masaoka分期、辅助放疗和(或)化疗与患者预后的关系.结果 本组59例患者随访时间1～111个月,平均54个月;失访3例,失访率6.1%.全组局部复发或全身转移19例,死亡14例,3年、5年生存率分别为86.8%、70.8%.单因素分析显示接受完全性切除手术、Masaoka分期较早及术后接受辅助放疗和(或)化疗的患者有较高的生存率(P＜0.05).多因素分析显示是否完全性切除、术后接受辅助放疗和(或)化疗是影响患者预后的独立因素(P＜0.05).结论 Masaoka分期与侵袭性胸腺瘤患者预后相关;完全性切除手术及术后辅助放化疗可显著提高患者生存率;部分复发患者再次手术后亦可获得长期生存.     

Functional reconstruction of large anogenital defects with bilateral V-Y advancement posterior thigh fasciocutaneous flaps.

BACKGROUND: In patients with perianal extramammary Paget's disease, wide local excision is recommended. In the reconstruction of the resulting large defects not only the closure of the defect but also the preservation of anogenital function need to be considered. METHODS: We used bilateral V-Y advancement posterior thigh fasciocutaneous flaps for reconstructing three large anogenital defects, including the total anal canal, resulting from wide local excisions attributed to extramammary Paget's disease. A temporary anus was constructed at the same time to allow the wound of the reconstructed anus to heal. After the surgery, the course of bowel function was followed up through interviews and manometric examinations. RESULTS: All defects were successfully reconstructed with the flaps, and bowel function recovered satisfactorily. Manometric examinations were carried out in two cases; anal pressures were recorded regularly and used as parameters of the recovery of bowel function before closing the artificial anus. Eventually, the patients recovered the function of defecation. CONCLUSIONS: We recommend the bilateral V-Y advancement posterior thigh fasciocutaneous flaps together with manometric studies as one of the most effective techniques for managing large anogenital defects. The use of sensate flaps, the restoration of original structures and manometric studies are conducive to successful outcomes.     

阴囊Paget病的治疗和预后(附23例报告)

目的:探讨阴囊Paget病的临床特点、治疗方式和预后。方法:回顾性分析23例阴囊Paget病患者的资料,分析其临床、病理、手术治疗和预后的特点。患者平均年龄62.4(49~72)岁,从出现症状到确诊的间隔时间平均为67.6(12~132)个月。A1期9例,A2期12例,B期1例,D期1例。首诊误诊率91.3%(21/23)。手术治疗20例,放疗2例,化疗1例。结果:失访1例,随访22例,平均随访时间119(7~233)个月。第1次治疗后,因肿瘤死亡1例,非肿瘤死亡3例。复发4例,复发伴腹股沟淋巴结转移1例,单独腹股沟淋巴结转移1例,12例无瘤生存。复发或转移患者均接受第2次手术治疗,非肿瘤死亡1例,5例无瘤生存。结论:阴囊Paget病在治疗上应以彻底切除病灶为首选治疗方式,术前或术中确定病灶的范围十分重要。肿瘤侵及真皮时易发生转移,切缘阳性是术后局部复发的关键因素。本病高龄患者居多,进展缓慢,早期治疗预后尚佳,一旦发生远处转移,预后差。     

Radiotherapy for adenoid cystic carcinoma of the palate

This is an analysis of 16 patients with adenoid cystic carcinoma of the palate who were treated with curative intent by radiotherapy alone (9) or surgery and radiotherapy (7). No patient received adjuvant chemotherapy. Follow-up ranged from 3 years to 19 years with 69% of the patients having follow-up of more than 5 years. Two patients in the radiotherapy alone group developed local recurrences, and both have had long-term surgical salvage. No local failures were seen in the combined therapy group. Twelve patients (75%) are alive and disease-free. Two patients died with distant disease, one within each treatment group. One patient in the combined therapy group is alive at 8 years with distant metastases, while one in the radiotherapy alone group died of intercurrent disease at 12 years. No significant complications were seen in either treatment group. The role of radiotherapy in the treatment of adenoid cystic carcinoma of the palate is reviewed.     

Timing of Surgery and the Role of Adjuvant Radiotherapy in Ewing Sarcoma of the Chest Wall: A Single-institution Experience

Background

Ewing sarcoma (ES) is the most common chest wall malignancy in adolescents. Current therapy incorporates chemotherapy to treat systemic disease and radiotherapy to assist with local control. We sought to evaluate the timing of surgery and role of adjuvant radiotherapy.

Methods

We reviewed the St. Jude Children??s Research Hospital chest wall ES experience from 1979 to 2009. Patient demographics, tumor characteristics, treatment variables, and outcomes were analyzed with respect to timing of surgery and use of adjuvant radiotherapy.

Results

Our cohort consisted of 36 patients with chest wall ES; median follow-up was 14.2?years, and 15-year estimate of overall survival was 66?%. In patients with localized disease, the timing of surgery (up-front vs. delayed) did not impact margin negativity or the use of adjuvant radiotherapy, but it did decrease the extent of chest wall resection. When considering radiotherapy in patients with localized disease, we found that patients who did not receive radiotherapy had smaller tumor size (median 6 vs. 10?cm) (p?=?0.04) and were more likely to have had negative margins (p?=?0.01) than patients who received adjuvant radiotherapy. One patient in each group developed a locoregional recurrence. The 15-year estimated of overall survival for patients who received adjuvant radiotherapy was 80 versus 100?% for those who did not.

Conclusions

Delayed surgery decreased the extent of chest wall resection and helped define a patient population with favorable tumor biology. Patients with complete pathologic responses to chemotherapy, and those with tumors <8?cm and negative surgical margins may be spared adjuvant radiotherapy without any decrement in overall survival.     

Crohn's disease: bowel resection to protect the proctium in severe perianal disease?

Patients with anal Crohn's disease generally have a bad prognosis. Up to 50% end up with an anus praeter or proctectomy. Many of these young people develop psychological and social problems causing them to become invalids. Local surgery and conservative therapy in such aggressive cases of Crohn's disease presenting with recurring perianal fistulae and abscesses are often unsuccessful; the destruction of the proctium continues. The purpose of our study was to look at the outcome of our patients with regard to these aspects. We included all 56 patients suffering from Crohn's disease treated at our clinic from 1984 until 1991 in a retrospective study and focused on the 13 patients with anal manifestation. The mean follow-up was 15.8 years (3-28 years). Often there was no improvement of perianal disease without resection of the involved bowel, especially in cases where both the colon and the rectum were affected. Anal destruction went on. Seven of the 13 patients suffering from anal complications finally received an anus praeter. An previous bowel-resection or the construction of a temporary anus praeter seem to be necessary to protect the proctium irrespective of abdominal symptoms in patients with recurrent severe perianal Crohn's disease.