肾上皮样血管平滑肌脂肪瘤1例报告及文献复习

目的：探讨肾上皮样血管平滑肌脂肪瘤（EAML）的临床病理特征、诊断、鉴别诊断、治疗方法及预后。方法：通过观察1例肾EAML患者的临床病理学表现与免疫组化标记并复习有关文献,讨论其组织学特征及临床生物学行为。结果：CT平扫呈略高密度实性肿块,边界清楚,病灶内无明显脂肪密度;增强扫描密度相对均匀,实质期强化程度最高,排泄期密度下降较少。镜检示肿瘤细胞弥漫分布,瘤体主要成分为上皮样细胞,细胞异型性明显,大小形状不等,核大且染色明显加深,偶有多核巨细胞,核分裂像明显。免疫组化染色显示瘤细胞HMB45＋,SMA＋,CK-。结论：病史、体检结合影像学检查能够给我们提供重要的诊断线索,组织病理学特点结合免疫组化标记有助于该疾病的确诊,治疗方法以手术切除为主,对于预后较差的患者应按照肾细胞癌标准长期随访。     

Isotretinoin and ulcerative colitis: A case report and review of the literature

This case report describes a case of ulcerative colitisthe onset of which occurred after the use of isotreti-noin for acne treatment. Our patient, a healthy maleyoung adult, after several months of isotretinoin use,developed gastrointestinal disorders and after thoroughmedical workup was diagnosed with ulcerative colitis.The literature regarding a possible correlation betweenisotretinoin use and ulcerative colitis is scarce. Never-theless, recent epidemiological studies have shed morelight on this possible association.     

肾上皮样血管平滑肌脂肪瘤一例报告并文献复习

目的 探讨肾上皮样血管平滑肌脂肪瘤(EAML)的病理及影像学特点.方法 患者,男,47岁.左上中腹疼痛不适1年入院.查体左上腹部可扪及巨大质韧肿块,叩痛(+).CT检查左肾见一最大径约28 cm肿块伴腹主动脉旁多枚肿大淋巴结,增强后呈不均性中等度强化.平扫相对高密度;乏脂肪;强化方式呈"快进慢出".全麻下行根治性左肾切除及周围淋巴组织清扫术.检索Ovid和CBM数据库相关文献复习讨论. 结果病理检查:肿瘤包膜完整,来源于左肾中上部,不能与左肾完全分离;切面为鱼肉样组织,伴灶性坏死组织;镜下:密集分布的上皮样细胞,无脂肪组织;免疫组化染色黑色素瘤相关抗原(HMB-45)强阳性、上皮膜抗原(EMA)阴性.病理报告:左肾EAML伴腹腔淋巴结转移,输尿管残端、血管残端及肾周组织未见肿瘤浸润.术后随访1年肿瘤未见复发转移.结论 EAML是一种罕见的、有恶性潜能的问叶肿瘤,有一定的病理及影像学特点,预后相对较好.     

The effect of appendectomy on the course of ulcerative colitis: a systematic review

Aim Previous studies have shown significantly lower appendectomy rates in ulcerative colitis (UC) patients compared with healthy controls. Evidence indicating that the appendix has an immunomodulatory role in UC has been accumulating. To examine the latest evidence on the effect of appendectomy on the disease course of UC. Method PubMed, The Cochrane Library and EMBASE were searched. Primary end‐points were number of relapses, use of steroids, number of hospital admissions and number of colectomies. Results The search resulted in six observational studies (five case–control studies and one cohort study) totalling 2532 patients. Owing to clinical heterogeneity, no meta‐analysis could be conducted. One study found lower relapse rates in patients appendectomized before the onset of UC [absolute risk reduction (ARR) = 21.5%; 95% CI: 1.71–45.92%]. Another two studies found a reduced requirement for immunosuppression in appendectomized patients (ARR = 20.2%; 95% CI: 9.67–30.46% in the first study and ARR = 21.4%; 95% CI: 10.32–32.97% in the second study). In addition, one study found lower colectomy rates in nonappendectomized patients (ARR = 8.7%; 95% CI: 1.29–18.66%) and two studies found lower colectomy rates in appendectomized patients (ARR = 21.4%; 95% CI: 13.17–28.79% in the first study and ARR = 18.7%; 95% CI: 7.50–29.97% in the second study). Conclusion There are limited and conflicting data available regarding the effect of appendectomy on the disease course of UC. Most studies suggest a beneficial effect and the minority find no, or a negative, effect.     

Long term follow-up of surgical treatment for ulcerative colitis—With special reference to recurrence and the quality of life—

From 1961 to 1984, fifty-one patients with ulcerative colitis were surgically treated at Tohoku University Hospital. The results of various types of surgical treatments for ulcerative colitis were analysed and discussed with special reference to recurrence and the quality of life at the time of follow-up. Total colitis and left-sided colitis were observed in 42 and 9 patients, respectively. There were five operative deaths. Thirteen patients received total proctocolectomy and 38 patients received various types of conservative operations at thefirst operation. In seven patients there was a recurrence of the disease in the retained colon or rectum and proctocolectomy with ileostomy was carried out. Forty-six patients were followed. Four died of other diseases and two were lost to follow-up. The periods of follow-up after the final operation ranged from 3–29 years. The types of operative procedures were ileostomy in 19, ascendicostomy in 14, ileoproctostomy in 6 and ascendicoproctostomy in 1 patient. Following treatment of recurrence of the disease, in most patients who had undergone various surgical treatments, favorable results of quality of life were attained, even in those with ileostomy. These results indicate that it is important to select the most proper types of operative procedures for surgical treatment of ulcerative colitis by individualizing each case.     

Delivery of a normal newborn after intensive medical treatment for an acute exacerbation of ulcerative colitis during pregnancy: A case report

(Received for publication on Sept. 21, 1998; accepted on May 27, 1999)     

Giant angiomyolipoma in a tuberous sclerosis patient and review of the literature

Introduction

About 20% of renal angiomyolipomas (RAML) are associated with tuberous sclerosis complex (TS). About 34–80% of patients with TS present with RAML. RAMLs associated with TS are at higher risk of potentially life-threatening hemorrhage and hypovolemic shock. Only a few case reports of giant RAML, defined as larger than 10?cm in diameter, and its management, have been reported.

肝上皮样血管内皮瘤1例并文献复习

目的提高对肝上皮样血管内皮瘤的认识。方法结合我院诊治的一例肝上皮样血管内皮瘤的患者资料和文献复习,探讨该病的临床特点、诊断、治疗及预后等。结果肝上皮样血管内皮瘤病因尚不明确,最常见的临床表现是右上腹疼痛、肝肿大和体重减轻,MRI或CT特点为:病灶对比增强扫描大于3 cm的病灶增强后有向心性强化倾向,而小于3 cm的病灶呈环形强化,门脉期及延迟期持续环形强化,肝内静脉主干及分支终止于肿瘤的边缘,形成"棒棒糖征"。病理学检查主要特征是具有细胞内血管腔的上皮样瘤细胞并呈血管内皮标志物染色阳性。肝上皮样血管内皮瘤的治疗以手术切除和肝移植为主,预后介于良、恶性肿瘤之间。结论肝上皮样血管内皮瘤发病率低、临床表现复杂,易误诊,临床诊治过程中应予以重视。     

溃疡性结肠炎32例手术治疗分析

目的探讨溃疡性结肠炎(UC)手术治疗的指征、时机、方式及转归。方法回顾性分析32例接受手术治疗的UC患者的住院病历资料,记录患者的临床表现、诊疗过程、手术方式及转归,并对患者术后情况进行随访。结果 71.9%(23/32)手术病例为重型UC;初发型占21.9%(7/32),慢性复发型78.1%(25/32);广泛结肠病变占93.8%(30/32),其中全结肠受累占81.3%(26/32)。手术原因:药物治疗不能达到或维持缓解26例(81.3%);UC相关结直肠癌(UC-CRC)/上皮内瘤变(IEN)5例(15.6%);并发肠穿孔1例(3.1%)。手术方式:全结直肠切除、回肠储袋肛管吻合术(IPAA)19例(59.4%),回肠永久造瘘术或长期保留造瘘口7例(21.9%),全结肠或次全结肠切除、肠吻合术6例(18.8%)。术后并发症发生率69.6%(16/23),分别为肠梗阻9例、吻合口狭窄2例、盆腔感染2例、直肠阴道瘘1例、切口疝1例、储袋炎2例、残余直肠UC复发2例。25例(78.1%)患者接受随访2个月~22年,21例UC治愈,2例因残余直肠UC复发继续药物治疗,2例因UC-CRC广泛转移死亡。结论手术是药物难治性及出现并发症UC的治疗选择,多数患者手术效果满意,术后并发症发生率较高,手术时机、术式及术后并发症的预防及随访有待进一步规范。     

Three cases of adenocarcinoma following restorative proctocolectomy with hand-sewn anastomosis for ulcerative colitis: a review of reported cases in the literature.

Restorative proctocolectomy (RPC) has been accepted as optimal surgical therapy for most patients with ulcerative colitis. The occurrence of adenocarcinoma adjacent to the ileoanal anastomotic site for ulcerative colitis is a serious but rare outcome. There are 16 reported cases. We report three additional cases and review previous cases in the literature.     

Surgical management of colonic perforation due to ulcerative colitis during pregnancy: Report of a case

This report describes a young female in her secondtrimester of pregnancy with known ulcerative colitis onmaintenance medical therapy. She was admitted forabdominal pain, and workup revealed a colonic stric-ture and ulceration with contained perforation. Aftermultidisciplinary discussion she was managed withcolectomy and end ileostomy. She delivered a healthynewborn 18 wk after surgery. Only a few prior reportsdescribed surgical management of inflammatory boweldisease during pregnancy, with recent results indicatinglow risk of adverse outcomes.     

Ileostomy carcinomas a review: the latent risk after colectomy for ulcerative colitis and familial adenomatous polyposis

BACKGROUND: Ileostomy carcinoma after colectomy for ulcerative colitis and familial adenomatous polyposis is rare. METHODS: Forty-three case reports from the literature and a case of ours are reviewed. RESULTS: The risk of malignancy following ileostomy formation appears to be increased compared to the very low incidence of primary small bowel carcinoma. Chronic physical or chemical irritation of the stoma may predispose the ileal mucosa to colonic metaplasia with subsequent adenoma formation, dysplasia and invasive malignant change. This is particularly so where ileostomies are fashioned for familial adenomatous polyposis and ulcerative colitis. CONCLUSION: Routine surveillance of patients who have had an ileostomy for 15 years or longer may lead to earlier detection of this complication.     

Discontinuous appendiceal involvement in ulcerative colitis: Pathology and clinical correlation

Continuous mucosal involvement from the rectum proximally is one of the hallmarks of ulcerative colitis. However, recent pathologic series report appendiceal ulcerative colitis in the presence of a histologically normal cecum, representing a "skip" lesion. The clinical significance of this rinding has not been established. Eighty patients, 54 males and 26 females, average age 37.9 years (range 14 to 82 years) who underwent proctocolectomy for ulcerative colitis from January 1990 to September 1995 were examined to determine the rate of discontinuous appendiceal involvement. Excluded were 12 patients with prior appendectomy and 11 with fibrotic obliteration of the appendiceal lumen. Of the remaining 57 patients, seven (12.3%) had clear appendiceal involvement in the presence of a histologically normal cecum. These seven patients clinically were indistinguishable from the 50 patients without skip involvement of the appendix in terms of age at surgery, pretreatment medications, type of surgery, interval from diagnosis to definitive procedure, complications, functional results, and clinical course. Discontinuous appendiceal involvement was found in 12.3% of patients undergoing proctocolectomy for ulcerative colitis, and clinically these patients behave as those without this feature.     

Giant abdominopelvic epithelioid angiomyolipoma associated with tuberous sclerosis: Report of a case

(Received for publication on July 16, 1998; accepted on Mar. 11, 1999)     

Restorative proctectomy after emergency laparoscopic colectomy for ulcerative colitis: a case-matched study

OBJECTIVE: The aim of the study was to evaluate feasibility and safety of restorative proctectomy with ileal pouch anal anastomosis (IPAA) through a Pfannenstiel incision after prior laparoscopic colectomy. METHODS: Seventeen patients who underwent restorative proctectomy after laparoscopic emergency colectomy for ulcerative colitis (UC) were prospectively evaluated. Results were compared with results of a group of 21 case matched patients that had restorative proctectomy and IPAA via a midline incision in the same period. RESULTS: Median operation time was longer, although not significantly, in patients who had a restorative proctectomy through a pfannenstiel (186 min) compared to a restorative proctectomy through a midline incision (158 min). Procedure related complications were comparable between the groups, respectively, 1 of 17 patients in the pfannenstiel group and 3 of 21 patients in the median laparotomy group. Median hospital stay in the pfannenstiel group was 10 days and in the midline group 12 days. CONCLUSIONS: After laparoscopic assisted emergency colectomy for ulcerative colitis, restorative proctectomy is feasible and can be performed safely through a Pfannenstiel incision.     

溃疡性结肠炎的治疗（附193例分析）

为了解炎性肠病的治疗变迁，本文随访分析了该院１９５８～１９９４年间收治的溃疡性结肠炎１９３例，对比了前２０年和近１７年的发病情况和治疗效果。发现近１０余年诊治该病由年平均４例．上升达６．６例；但重症或转外科治疗的病例明显减少，未再出现合并肠穿孔或中毒性巨结肠病例。近９０％病人经内科治疗痊愈或好转，外科手术死亡率前２０年为４３％（９／２１），１９７８年后无１例死亡（０／６）。作者认为，近年治疗效果的提高，除一般病人体质改善外，与抗菌药物趋向完善，充分的静脉营养支持和选择恰当的手术方式有关。     

Laparoscopic surgery for ulcerative colitis – a meta-analysis

OBJECTIVE: To assess the safety and feasibility of laparoscopic surgery for patients with ulcerative colitis. METHODS: A search of published studies in English between January 1992 and September 2005 was obtained, using the MEDLINE and PubMed databases and the Cochrane Central Register of Controlled Trials. Two independent assessors reviewed the studies using a standardized protocol. Where raw data, means and standard deviations were available, meta-analysis was performed using the Forest plot review. Studies where medians and ranges were presented were separately analysed. RESULTS: The duration of surgery for laparoscopic and open procedures were similar (weighted mean difference 62.92 min, P = 0.19). Patients were able to tolerate oral intake significantly earlier, with a weighted mean difference of 1.39 days (P = 0.002), but recovery of bowel function was similar (weighted mean difference 0.73 days, P = 0.36). The length of hospital stay was shorter for patients who had undergone laparoscopic surgery, with a weighted mean difference of 2.64 days (P = 0.003). The complication rate was higher in open colectomy, compared to laparoscopic colectomy (67.6%vs 39.7%, P = 0.005). For restorative proctocolectomy, complication rates were comparable between the laparoscopic and open groups (P = 0.25). CONCLUSIONS: The time taken to perform laparoscopic surgery is similar to open surgery. Patients are able to tolerate oral intake earlier, and have a shorter hospitalization. Laparoscopic colectomy was safer compared to the open procedure, but both were equally safe for patients who had restorative proctocolectomy. Thus, laparoscopic surgery for ulcerative colitis is both safe and feasible.