原发性肛管直肠恶性黑色素瘤病例1例并文献复习

目的:探讨原发性肛管直肠恶性黑色素瘤(PAMM)的临床及病理特征、诊断和治疗方法。方法:回顾性分析1例原发性肛管直肠恶性黑色素瘤患者的病历资料,结合国内外文献,总结PAMM的临床和病理特点、诊断和治疗方法。结果:该患者术前肠镜提示距肛缘4cm处肿块,肠镜下活检病理提示恶性黑色素瘤,行腹部会阴切除术,术后病理再次证实肛管直肠恶性黑色素瘤。术后患者顺利出院。结论:PAMM是非常罕见的恶性肿瘤,临床表现缺乏特异性,确诊需要病理诊断。预后差,手术切术是治疗原发性肛管直肠黑色素瘤的最佳方案。     

诊断为结直肠高级别上皮内瘤变的临床意义及外科处理

目的 分析肠镜活检诊断为结直肠高级别上皮内瘤变的临床病理特征及外科治疗对策.方法 对2005年1月-2012年12月收治经内镜活检病理诊断为高级别上皮内瘤变的结直肠肿瘤患者82例的临床资料进行回顾性分析.71例行根治性结直肠癌手术,其中包括1例扩肛多次局切后术中病理补充行miles术,其他3例行单纯扩肛肿瘤切除术,8例行姑息性肿瘤切除术.将术后诊断与肠镜活检诊断进行比较分析.结果 术后病理3例(3.7％)仍为高级别上皮内瘤变,肿瘤直径1～3 cm,平均1.5 cm;其余79例(96.3％)证实为黏膜下层浸润性腺癌,肿瘤直径1 ～11 cm,平均4.7 cm,两组肿瘤最大直径差异有统计学意义(P＜0.01).Kappa一致值为0.104,术前术后差异较大,一致性较差.统计分析显示肿瘤癌变与大小和浸润深度相关.证实为腺癌的79例中已有8例出现肝转移,31例(39.2％)有局部区域性淋巴结转移;33例直肠肿瘤中(包括1例多原发性肿瘤)有30例(90.9％)术后病理证实为腺癌.结论 要重视结直肠上皮内瘤变的病理诊断,诊断为高级别上皮内瘤变的肿瘤患者绝大多数已存在浸润性癌,甚至有区域性淋巴结转移及远处转移.如肿瘤位置不涉及保肛的问题,结直肠肿瘤直径大于3 cm者,应首选作病变肠段的切除,术中病理诊断为浸润性腺癌,则应行根治性手术.对于低位直肠肿瘤应当多次内镜活检和或扩肛肿块切除后再决定治疗方案.     

睾丸网腺癌(1例报告并文献复习)

目的了解睾丸网腺癌的发病和临床特点，提高其早期诊断和治疗水平。方法分析1例睾丸网腺癌患者的临床资料，并结合文献对睾丸网腺癌的临床病理特点、早期诊断和治疗进行讨论。结果术前诊断为左睾丸鞘膜积液、左睾丸肿瘤待排，遂行左高位睾丸切除术，术后病理检查诊断为睾丸网腺癌。免疫组化：AFP（-），PLAP（-），CK（＋），CD30（-），CK20（＋）。术后CT示肝内多发转移瘤、左肾和左肾上腺转移改变。结论睾丸网腺癌是原发于睾丸网纵膈的恶性肿瘤，极为罕见，其临床表现独特，基本治疗是根治性睾丸切除术辅以根治性腹膜后淋巴结清扫术，放疗和化疗效果欠佳。该病预后差，文献报道5年生存率仅为13％。     

原发性结直肠淋巴瘤20例临床分析

目的:探讨原发性结直肠淋巴瘤的诊断及临床病理、治疗与生存预后的关系。方法：回顾性总结34年间因原发性结直肠淋巴瘤接受手术治疗者的临床资料;分析治疗和各临床病理因素对预后的影响。结果：肿瘤大小、淋巴结转移、有无邻近器官侵犯和远处转移、治疗方式对患者生存预后具有显著性意义（P<0.01）;年龄、性别、肿瘤单发或多发、淋巴瘤组织细胞恶性程度、肿瘤侵犯深度不是影响患者预后的独立因素（P>0.05）。结论：原发性结直肠淋巴瘤手术前误诊率高。治疗应以手术切除为主。根治手术辅以化疗可以获得较好的预后。     

放大肠镜在结直肠肿瘤样病变诊断治疗中的应用价值

目的 探讨应用放大肠镜诊断结直肠肿瘤样病变及指导治疗的价值。方法 用放大肠镜对61例结直肠肿瘤患者的78个病灶进行了染色后的放大观察，按工藤分型进行了腺管开口类型诊断；同步进行镜下摘除或手术切除后，将放大肠镜诊断结果与组织病理诊断结果相比较，分析其一致性。结果（1）依据放大肠镜所见，诊断腺瘤等肿瘤性病变，总体符合率为96.2％，敏感性98.4％，特异性85.7％；（2）依据放大肠镜的诊断，对70个（89.7％）良性病变进行了同步微创治疗；（3）结合放大观察的肠镜检查，为决定其他8个病灶的治疗方案提供了重要依据。结论 放大电子肠镜诊断结直肠肿瘤样病变及时、准确，利用它可以同步完成病变的微创治疗。     

结肠、上段直肠的鳞癌和腺鳞癌:临床和组织病理学研究

尽管结肠、直肠肿瘤是西方国家第 2位最常见的恶性肿瘤 ,但绝大多数是腺癌 ,结肠直肠的鳞癌和腺鳞癌很罕见。目前直肠腺癌和肛管鳞癌的治疗已经取得了很大进展 ,但结肠直肠鳞癌和腺鳞癌的临床病理特性和最恰当的治疗方式不明确。本研究目的是阐明这种罕见肿瘤的独特危险因素 (包括人乳头瘤病毒 )、临床病理特征、预后和预后相关因素 ,并讨论辅助治疗和作用。　　方法 :回顾分析单中心报告的病例最多的一组5 2例结肠和齿线 8cm以上的近侧直肠的鳞癌和腺鳞癌 ,其中 44例足够的组织材料可供研究。单纯性鳞癌 1 1例、混合性腺鳞癌 31例、腺癌伴…     

肛管直肠恶性黑色素瘤的诊治

目的 探讨肛管直肠恶性黑色素瘤的临床特点，诊断，治疗及预后。方法 对近21年来经手术及病理证实的11例肛管直肠恶性黑色素瘤临床特征，治疗方法及预后进行回顾性分析。结果 11例中7例有不同程度的便，肛周疼痛等肛门症状，术前误诊7例。全组均行腹会阴联合切除术，术后平均生存18个月，最长存活39个月。结论 肛管直肠恶性黑色素瘤恶性程度极高，死亡率高，较早发生淋巴和血行转移，宜 采用根治手术，辅以化疗及生物治疗的综合性治疗。     

直肠癌阴茎转移1例报告并文献复习

目的:提高对阴茎转移癌诊断和治疗的认识。方法:报道1例54岁直肠腺癌阴茎转移患者资料,结合文献讨论其好发部位、临床表现、发病机制和诊疗措施。结果:患者原发肿瘤与周围组织粘连严重,且有肝转移可能,行横结肠双腔造瘘术,术后给予奥沙利铂、替加氟及亚叶酸钙化疗,患者存活10个月,死于多器官功能衰竭。结论:阴茎转移癌预后差,但早期明确诊断和针对原发肿瘤合理的综合治疗和个体化治疗措施有助于提高患者生活质量、减少痛苦和延长生命。     

直肠癌根治术后复发29例临床分析

为探讨直肠癌根治术后复发的原因和对策 , 笔者回顾性分析218例直肠癌手术后29例复发患者的临床资料 。复发时间为3.6个月~7年，中位数为19个月。包括局部切除1例，术后复发时间4个月;Miles术11例，Dixon术17例，复发时间分别为21，19.5个月。复发病例中管状腺癌19例，黏液腺癌6例，乳头状癌2例，未分化癌1例，混合型1例;属DukesA期1例, B期15例, C期13例。复发部位主要在吻合口、盆腔会阴部及远处转移。 提示直肠癌根治术后复发与根治不彻底，以及肿瘤分期、病理类型、围手术期处理不当等多种因数有关。术后综合治疗能提高患者的生存率。     

肛管直肠恶性黑色素瘤诊治体会(附11例报告)

目的　探讨肛管直肠恶性黑色素瘤有效的治疗方法 ,以提高其生存率。方法　回顾性分析本院收治的 1 1例经病理证实的肛管直肠恶性黑色素瘤病人临床资料 ,结合有关文献 ,加以总结。结果　 1 1例病人中 ,7例行腹会阴联合切除术 (Mile’s)。其中 ,2例加行两侧腹股沟淋巴结清扫 ;3例仅行局部扩大切除 ;1例保留肛门经腹直肠切除吻合器吻合术 ,术后辅以化疗和生物治疗。 9例获得随访。根治性切除组的中位生存期为 2 7个月 ,而局部扩大切除组的中位生存期为 2 1个月。其中 1例因早期获得诊断 ,早期行根治性切除而存活近 4年。结论　早期诊断、早期发现是治疗的关键。早期行根治性切除 (Mile’s) ,术后辅以化疗及生物免疫治疗是处理肛管直肠恶性黑色素瘤的有效措施     

Primary squamous cell carcinoma of the prostate

BACKGROUND: Primary squamous cell carcinoma (SCC) is an uncommon tumor of the prostate gland. A 65-year old man complained of obstructive symptoms. METHODS/RESULTS: Transrectal palpation and diagnostic imaging indicated an ordinary adenocarcinoma, although serum prostate-specific antigen (PSA) was normal. Biopsy specimens revealed SCC with the serum SCC antigen elevated. The patient was treated with pelvic irradiation and systemic administration of cis-platinum and peplomycin, which resulted in shrinkage of the cancer. CONCLUSION: No evidence of recurrence has been seen for 18 months.     

胰腺肝样腺癌腹腔多发转移1例报告并文献复习

背景与目的 胰腺肝样腺癌（PHC）是一种罕见的易发生于胰腺体尾部的具有肝细胞癌（HCC）样分化特征的特殊类型腺癌,其发病率低,恶性程度高,侵袭性强,早期易发生淋巴转移和远处转移,疾病发展快,预后较差。PHC临床表现和影像学特征缺乏特异性,术前诊断困难,但多数患者可高表达甲胎蛋白（AFP）,为其诊断提供一定依据,而最终疾病的确诊需进行病理学检查。本文回顾性分析总结1例PHC并腹腔多发脏器转移的患者临床资料特点及诊治过程,并对国内外相关文献进行复习,旨在增加临床医生对PHC的认识,并不断完善疾病的治疗方案。方法 回顾性分析昆明医科大学第二附属医院肝胆胰外科收治的1例PHC并腹腔多发脏器转移患者的临床资料及诊治过程,并结合国内外相关文献对该病的发生机制、临床特点、疾病诊断及治疗方案等进行分析总结。结果 患者为63岁男性,因出现腹胀腹痛伴乏力、纳差3个月余入院,检查提示肝脏、胰腺、脾脏、胃等多脏器占位性病变,性质待排,后行姑息性减瘤手术治疗。术后病理结果提示为PHC,实性型,组织学分级3级。术后3个月出现肝内转移灶,行奥沙利铂130 mg+氟尿嘧啶200 mg肝动脉灌注化疗及栓塞治疗,后病灶转移至肺,于术后10个月因多器官功能衰竭死亡。结论 PHC是一种罕见的病因不明的具有HCC样分化特征的特殊类型腺癌,缺乏典型的临床及影像学表现,该病恶性程度高,早期易发生淋巴转移和远处转移,出现症状时多达中晚期,已丧失根治性手术切除机会;PHC发病机制不详,但病变表现为特征性的HCC样分化,可高度表达AFP及HCC免疫组织化学标志物,病理学检查是其诊断金标准;目前国内外尚无PHC的诊治共识,对于有手术切除机会的患者应积极手术治疗,切除病灶,改善预后;而对于无法根治性切除的患者可采取辅助治疗,目前认为放疗对其无效,而化疗是疾病的独立预后因素,但在化疗方案上仍存在争议;因此,有条件者可取材活检明确诊断后选择合适的化疗方案,提高患者治疗的客观缓解率,不能明确诊断者可参照消化道系统疾病治疗方案处理。     

Open-magnet MR defaecography compared with evacuation proctography in the diagnosis and management of patients with rectal intussusception

Objective The aim of this study was to determine whether open‐magnet magnetic resonance (MR) defaecography could provide more useful clinical information than evacuation proctography (EP) alone in the evaluation of a cohort of patients with full‐thickness rectal intussusception and could assist in decisions concerning management. Methods Ten patients (4 male; median age 43, range 30–65) with symptomatic circumferential rectal intussusception diagnosed on EP, underwent open‐magnet MR defaecography. Pathologies visible with each technique were recorded and 12 parameters of anorectal configuration and morphology measured and compared. Results There was discordance in the diagnosis of rectal intussusception in three cases. In another two patients, MR defaecography demonstrated mucosal descent only. Measurements of anorectal configuration and morphology were similar between techniques; only rectal size and lateral dimensions of the rectocoele were significantly different, being smaller on MR defaecography than EP. Two patients were shown on MR defaecography to have significant bladder descent and two female patients had significant vaginal descent. Conclusion EP remains the first line investigation for the diagnosis of rectal intussusception, but may not distinguish mucosal from full‐thickness descent. MR defaecography further complements EP by giving information on movements of the whole pelvic floor, 30% of the patients studied having associated abnormal anterior and/or middle pelvic organ descent. If surgery is planned for patients with rectal intussusception, MR defaecography provides useful information regarding the presence and degree of anterior pelvic compartment descent that may need to be addressed if a good functional outcome is to be achieved.     

尿路复合性恶性肿瘤（附21例报告）

报告２１例尿路复合性恶性肿瘤，位于肾盂２例，膀胱１９例。主要临床表现为血尿，绝大多数病人伴有尿路刺激症状。低分化的移行细胞癌（ＴＣＣ）与继发性复合肿瘤有密切关系。２１例中１例为ＴＣＣ复合肉瘤，１１例为ＴＣＣ复合鳞癌，７例为ＴＣＣ复合腺癌，２例为腺癌复合鳞癌。本组２例肾盂肿瘤分别行肾输尿管全长加膀胱袖口状切除术和肾部分切除术，术后存活６个月和１年；膀胱肿瘤１４例行膀胱部分切除术，已生存３年４例，１年２例，未满１年２例，３例１～２年内死亡，失访３例；２例根治性膀胱全切术已生存５年和３年以上；３例行ＴＵＲＢｔ，已生存３年１例，１５年１例，未满１年１例。对尿路复合性恶性肿瘤的组织学、临床和病理特征进行了讨论。     

Small cell carcinoma of the prostate: a report of three patients and a prognostic analysis of cases reported in Japan

Small cell carcinoma (SCC) originating from the prostate is rare. We report three cases of SCC of the prostate. Case 1: A 29-year-old man with large pelvic mass and pelvic lymph node metastases was diagnosed as having pure SCC of the prostate. Chemo-radiotherapy resulted in a great reduction of the tumor volume. However, the disease recurred immediately, and he died of disease 17 months after diagnosis. Case 2: A 65-year-old man presented with pure prostatic SCC with lung metastases. Although cystoprostatectomy combined with pre- and post-operative chemotherapy ended with no evidence of disease, he died after 16 months because of multiple metastases and local recurrence. Case 3: A 73-year-old man was diagnosed as having SCC and poorly differentiated adenocarcinoma of the prostate simultaneously. Chemo-endocrine therapy and pelvic irradiation were performed, achieving partial remission. However, he developed multiple distant metastases, and died of disease 15 months after diagnosis. We reviewed 82 cases previously reported in Japan. Patient's ages ranged from 24 to 86 years (mean 68.7 years). Many patients had lymph node or distant metastases (stage D, 73%). Thirty-seven (45%) were pure SCCs and 45 (55%) were associated with adenocarcinoma. The prognosis after the recognition of SCC is very poor, and the 1- and 2-year survival rates were 27% and 10%, respectively. Survival did not differ in patients with pure SCC or mixed glandular and small cell carcinoma. Higher elevation of pretreatment serum NSE value was associated with the poor prognosis.     

Giant T4 rectal carcinoma mimicking urinary bladder adenocarcinoma accurately diagnosed by immunohistochemistry and successfully treated with total pelvic exenteration: Report of a case

A 54-year-old man, with the chief complaints of hematuria, pollakisuria, and pneumaturia, was referred to our hospital, with a diagnosis of giant urinary bladder adenocarcinoma with massive invasion to the rectum. On the basis of the radiological diagnosis and findings of hematoxylin and eosin (H&E) of biopsy specimens, it was difficult to conclude whether the adenocarcinoma originated in the bladder or in the rectum. The immunohistochemical staining of the biopsy specimens showed cytokeratin 7 (CK7)(−) and CK20(+), which supported the notion that the adenocarcinoma possibly originated from the rectum. Although the prognosis of T4 bladder adenocarcinomas has been reported to be quite poor in comparison with that of transitional cell carcinomas, the postoperative prognosis of T4 rectal adenocarcinomas has been reported to be more favorable and such tumors are recommended to be surgically resected. Because no distant metastasis was detected, the patient underwent total pelvic exenteration with a reconstruction of the ileal conduit. Although the resected tumor measured 12 cm in diameter with n1 metastasis, the radial margin was cancer-negative, and the tumor was curatively resected. The immunohistochemical diagnosis of the resected tumor showed carcinoembryonic antigen(+), CK7(−), CK20(+), thrombomodulin(−), and uroplakin(−), which supported the rectal origin. At present the patient is undergoing postoperative adjuvant chemotherapy for rectal cancer.     

Primary squamous cell carcinoma of the rectum: An update and implications for treatment

AIM: To provide an update on the aetiology, pathogenesis, diagnosis, staging and management of rectal squamous cell carcinoma(SCC).METHODS: A systematic review was conducted according to the preferred reporting items for Systematic Reviews and Meta-Analyses guidelines. A comprehensive search of Ovid MEDLINE was performed with the reference list of selected articles reviewed to ensure all relevant publications were captured. The search strategy was limited to the English language, spanning from 1946 to 2015. A qualitative analysis was undertaken examining patient demographics, clinical presentation, diagnosis, staging, treatment and outcome. The quantitaive analysis was limited to data extracted on treatment and outcomes including radiological, clinical and pathological complete response where available. The narrative and quantitative review were synthesised in concert.RESULTS: The search identified 487 articles in total with 79 included in the qualitative review. The quantitative analysis involved 63 articles, consisting of 43 case reports and 20 case series with a total of 142 individual cases. The underlying pathogenesis of rectal SCC while unclear, continues to be defined, with increasing evidence of a metaplasia-dysplasia-carcinoma sequence and a possible role for human papilloma virus in this progression. The presentation is similar to rectal adenocarcinoma, with a diagnosis confirmed by endoscopic biopsy. Many presumed rectal SCC's are in fact an extension of an anal SCC, and cytokeratin markers are a useful adjunct in this distinction. Staging is most accurately reflected by the tumour-nodemetastasis classification for rectal adenocarcinoma. It involves examining locoregional disease by way of magnetic resonance imaging and/or endorectal ultrasound, with systemic spread excluded by way of computed tomography. Positron emission tomography is integral in the workup to exclude an external siteof primary SCC with metastasis to the rectum. While the optimal treatment remains as yet undefined, recent studies have demonstrated a global shift away from surgery towards definitive chemoradiotherapy as primary treatment. Pooled overall survival was calculated to be 86% in patients managed with chemoradiation compared with 48% for those treated traditionally with surgery. Furthermore, local recurrence and metastatic rates were 25% vs 10% and 30% vs 13% for the chemoradiation vs conventional treatment cohorts.CONCLUSION: The changing paradigm in the treatment of rectal SCC holds great promise for improved outcomes in this rare disease.     

Pelvic retroperitoneal leiomyoma

Primary retroperitoneal leiomyomas are a rare and unusual differential diagnosis in the acute abdomen. A 26-year-old woman presented with chronic constipation and acute abdominal pain. The diagnostic workup included contrast computer tomography, transabdominal ultrasonography, and colonoscopy that revealed an 8-cm homogeneous pelvic tumor with rectal compression. Retroperitoneal leiomyoma was suspected on imaging studies and finally confirmed after tumor excision and immunohistochemical staining.     

胰腺混合性腺泡- 神经内分泌癌1例报道及文献复习

目的:探讨胰腺混合性腺泡-神经内分泌癌(MANEC)的临床病理特征、鉴别诊断和治疗方法。方法:回顾性分析收治的1例胰腺MANEC患者的临床资料,结合国内外文献,总结该病的临床特点、病理特征、鉴别诊断和治疗方法。结果:该患者术前影像学检查提示十二指肠降部肿瘤,行手术治疗,术后病理提示胰腺MANEC。术后患者顺利出院,20个月后复查CT提示肿瘤无复发转移。结论:胰腺MANEC是非常罕见的肿瘤,常无典型的症状,最终需要病理确诊。预后至今不清楚,但是早期的手术治疗能显著提高总的生存期。     

Primary Enteric-Type Mucinous Adenocarcinoma of the Urethra in a Patient With Ulcerative Colitis

Primary carcinoma of the male urethra accounts for less than 1% of malignancies in men. Mucinous adenocarcinoma of the urethra is extremely rare, and its biologic behavior is poorly understood. We present herein a rare case of mucinous urethral adenocarcinoma in a male patient with longstanding ulcerative colitis and multiple sclerosis. The patient presented with a voluminous pelvic mass; core biopsy of the lesion demonstrated a mucus-producing adenocarcinoma. Given the patient''s history of subtotal colectomy, preoperative diagnosis was oriented towards a rectal stump adenocarcinoma. The patient underwent a pelvic exenteration: surprisingly, histology marked the prostatic urethra as the primary lesion site.Key words: Urethra, Adenocarcinoma, Mucinous, Ulcerative colitisPrimary enteric-type mucinous adenocarcinoma of the urethra is an extremely rare entity with aggressive clinical course regardless of treatment. Interestingly, it has been implied that inflammatory bowel disease and multiple sclerosis share common mechanisms of impaired histocompatibility. Moreover, patients with inflammatory bower disease who receive prolonged immunosuppression such as azathioprine tend to increasingly develop urinary tract malignancies. We report herein a complex case of enteric-type mucinous adenocarcinoma of urethral origin in a male with a longstanding history of ulcerative colitis and multiple sclerosis.