Large gastrointestinal stromal tumor and advanced adenocarcinoma in the rectum coexistent with an incidental prostate carcinoma: A case report

INTRODUCTION

Gastrointestinal stromal tumors (GISTs) are the leading mesenchymal neoplasia in the gastrointestinal tract, but GIST arising from the rectum is rare. When a secondary neoplasia coexists in the vicinity of a rectal GIST, more aggressive treatment strategies may be needed to cure the diseases.

PRESENTATION OF CASE

We herein describe a 76-year-old man with a large gastrointestinal stromal tumor along with an advanced adenocarcinoma in the rectum that coexisted with prostate carcinoma. Preoperative examination revealed an advanced adenocarcinoma of the upper rectum and a large pelvic mass suggestive of a GIST or a neuroendocrine tumor arising from the anterior wall of the lower rectum. To eradicate the tumor, total pelvic exenteration with ureterocutaneous fistula was carried out after obtaining written informed consent. Immuhistochemical studies revealed the concurrence of an advanced rectal cancer (T3, N1, M0) and a malignant GIST (c-kit-positive, CD34-positive, vimentin-positive, and CAM5.2-negative), and an incidental prostatic acinar adenocarcinoma. The patient was given adjuvant chemotherapy with imatinib and remains disease-free as of 12 months after surgery.

DISCUSSION

A PubMed search for the case of coexistence of GIST with two other malignancies revealed only four cases, making this very rare condition.

CONCLUSION

Radical surgery with perioperative adjuvant chemotherapy using tyrosine kinase inhibitors is the choice for treatment of large GISTs with a malignant potential. Our report suggests that aggressive surgical approach would be feasible, when a secondary tumor is present near the GIST.     

Rectal gastrointestinal stromal tumor with metastasis to the penis: Case report and review of literature

We report the case of a 51-year-old gentleman with previously diagnosed gastrointestinal stromal tumor (GIST) of the rectum with metastasis to the penis. The patient underwent abdominoperineal resection of the primary tumor with negative margins and completed a three-year course of imatinib mesylate (Gleevec). Forty months after resection of his rectal tumor, the patient presented to his urologist with worsening testicular pain, mild lower urinary tract obstructive symptoms, and nocturia. A pelvic MRI revealed the presence of an ill-defined mass in the right perineum extending from the base of the penis to the penoscrotal junction. Biopsy of this mass was consistent with metastatic GIST. To our knowledge, this is the first report of metastatic GIST to the penis.     

胃肠道间质瘤46例临床诊治分析

目的探讨胃肠道间质瘤(G IST)的临床特点。方法对1998~2004年收治的46例G IST病人的临床表现、手术处理及病理结果进行回顾性分析。结果46例均行手术治疗。病理诊断良性13例(28.3%),潜在恶性10例(21.7%),恶性23例(50%)。随访时间6个月至6年,7例出现远处转移或局部复发,10例死亡(其中9例为恶性G IST)。结论G IST缺乏特征性临床表现,术前确诊率较低,其确诊依赖病理结果。目前G IST的治疗仍以手术切除为主,对复发或远处转移者积极再次手术可延长生存期。     

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目的　探讨胃肠道间质瘤的临床表现、诊断及治疗。方法　回顾性分析上海建工医院及复旦大学华山医院外科 2000年 9月至 2003年 12月间收治 35例胃肠道间质瘤病人的临床及病理资料。结果　35例均行手术治疗。肿瘤根治 4例,近端或远端胃大部切除、病变肠段切除、肿块切除或扩大切除 26例;因肿瘤广泛粘连,无法完整切除 5例。病理:CD117阳性 29例(82 .9% ),CD34阳性 31例 (88 .6% )。结论　胃肠道间质瘤在中老年人中好发,以便血、腹痛和腹部不适、腹部肿块表现为主。肿瘤的大小是胃肠道间质瘤良、恶性的重要临床指标。病理形态学检查是确诊间质瘤的唯一方法。完整的局部手术切除是最有效的治疗手段。对无法切除或有肿瘤残余及术后复发的病人可应用甲磺酸伊马替尼(格列卫)进行治疗。     

Acute diffuse peritonitis due to spontaneous rupture of a primary gastrointestinal stromal tumor of the jejunum: A case report

IntroductionGastrointestinal stromal tumors (GISTs) are the most common mesenchymal tumors of the gastrointestinal tract. Overt peritonitis caused by GIST rupture is very uncommon. Three types of GIST rupture have been described: closed perforation due to abscess (abscess type), hemoperitoneum leading to rupture of the hematoma capsule in the tumor (hemoperitoneum type), and perforation of the digestive tract via a fistula leading to central necrosis of the tumor (bowel perforation type). This report describes a patient with spontaneous tumor rupture and diffuse peritonitis, a variant of the bowel perforation type of GIST rupture.Presentation of caseA 74-year-old man presented with symptoms of vomiting and abdominal pain. Computed tomography (CT) scan revealed an approximately 10 × 7-cm mass in the pelvis with free air and fluid collection. Emergency laparotomy revealed a tumor in the jejunum, which was ruptured with a hole measuring 5 mm in diameter. The tumor and part of the jejunum were resected. Immunohistochemically, the mass was diagnosed as a GIST originating from the gastrointestinal tract. Despite chemotherapy with imatinib mesylate, the patient died 22 months after surgery.ConclusionsThis report describes a patient with acute diffuse peritonitis due to spontaneous rupture of a primary GIST of the jejunum.     

双镜联合技术在胃间质瘤治疗中的应用

胃肠道间质瘤(GIST)是胃肠道最常见的间叶源性肿瘤.对于未发生转移的GIST,手术切除是首选的治疗措施.近年来随着腹腔镜外科、内镜外科等微创外科技术的不断发展和成熟,双镜联合技术(LECS)已逐渐发展为胃间质瘤治疗中一种较为成熟的微创手术方式.本文将就近年来双镜联合技术在胃间质瘤治疗中的应用做一综述.     

46例胃肠道间质瘤的临床诊治分析

目的探讨胃肠道间质细胞瘤的诊断和治疗。方法回顾性分析1997年1月至2005年1月收治的46例胃肠道间质细胞瘤的临床资料。结果本组46例均行手术治疗。无手术中死亡病例。术后在切口感染及其它严重并发症。术后获得随访42例。其中良性及交界性GIST获得随访26例（26／28）,平均随访30个月,为无瘤生存。恶性GIST获得随访15例,平均随访21个月。其中7例为无瘤生存,2例分别于术后第10及21个月发生肝多发性转移。1例术后20个月发生全身骨转移,3例分别于术后第6、9及11个月死于多发性远处转移。结论GIST由于缺乏特异的临床表现,影像学及内镜检查往往在术前亦难以作出定性诊断,但B超内镜引导下细针穿刺活检及免疫组化检查对诊断比较可靠。手术治疗效果良好。手术治疗的原则是：良性及交界性GIST以局部切除为主,恶生GIST宜行较大范围的手术,必要时须行联合脏器切除术。     

胃肠道间质瘤伊马替尼术前治疗进展

目的 探讨伊马替尼术前治疗胃肠道间质瘤（gastrointestinal stromal tumor,GIST）的作用。 方法 采用文献复习的方法,对研究伊马替尼术前治疗GIST的文献加以综述。 结果 伊马替尼术前治疗是进展期GIST的有效治疗手段,能显著提高患者的手术切除率,延长总体生存时间。 结论 术前伊马替尼治疗转移性或局部进展期GIST疗效较好,应参考GIST基因分型结果个体化术前给药,值得进一步深入临床研究。     

影响胃肠道间质瘤预后因素的研究进展

胃肠道间质瘤(GIST)是起源于间叶组织的消化系统肿瘤。影响本病预后的因素有很多,随着病理学技术的发展和最新循证医学证据的提出,人们发现了更多影响GIST预后的因素。因此,本研究总结肿瘤部位、肿瘤大小、核分裂数、肿瘤是否破裂、手术方式、核抗原Ki67、琥珀酸脱氢酶复合物(SDH)、原癌基因C－KIT突变、血小板源性生长因子受体A(PDGFRA)突变表达等因素对GIST预后的影响。     

以便血为首发症状的胃肠道间质瘤20例诊治分析

目的探讨以便血为首发症状的胃肠道间质瘤诊治经验。方法对有完整资料、经术后病理和免疫组织化学证实的20例以便血为首发症状的胃肠道间质瘤临床资料进行回顾性分析。结果本组病人主要临床表现为黑便、腹痛、头晕、贫血。20例中首诊发现确切病灶11例,余9例为多次就诊发现。20例均接受手术治疗,术中发现伴腹腔播散1例。随访8～67个月,1例死亡,复发、转移5例,其余14例均健在,间质瘤无复发。结论对于以便血为首发症状的胃肠道间质瘤,应有序、及时、联合应用各种检查手段,以提高其首诊阳性率和早诊率。     

十二指肠胃肠道间质瘤外科治疗与预后因素分析

目的:探讨十二指肠胃肠道间质瘤的诊断、手术方式和预后因素。方法:回顾性分析18例经病理证实的十二指肠胃肠道间质瘤的临床资料,包括临床症状、诊断方法、病灶部位、手术方式、病理结果和预后等。结果:18例均行手术切除治疗,11例行十二指肠肿瘤局部切除术,3例行胰十二指肠切除术(Child术),2例行肠段切除术,2例行胃大部切除术;经24个月到7年随访,5例死于转移和复发,其余13例生存,生活质量良好。随访资料显示性别、年龄、肿瘤大小和手术方法与病人的复发和死亡率无关;导致病人复发和死亡唯一危险因素是肿瘤的危险度分级。结论:上消化道出血是十二指肠胃肠道间质瘤最为常见的症状。多因素分析显示,高危分级是十二指肠胃肠道间质瘤的独立预后因素;根据肿瘤部位和大小来决定手术方式。     

胃肠道间质瘤诊治新进展

胃肠道间质瘤是消化道最常见的间叶细胞肿瘤.近年来,随着组织学的进展,人们对于间质瘤的认识取得了新的进步,对其治疗也提出了新的观点,随着抗肿瘤药物伊马替尼的临床应用,胃肠道间质瘤的治疗方法起了重大的变化,由原先单一的手术治疗转为手术、药物的联合治疗.本文回顾近几年来国内外学者的研究及经验,对胃肠道间质瘤的诊断和治疗作一综述.     

Gastrointestinal stromal tumor of the lesser omentum: report of a case

We describe herein an extremely unusual case of a gastrointestinal stromal tumor (GIST) of the lesser omentum. A 45-year-old man was admitted to our hospital with an intra-abdominal mass that was subsequently misdiagnosed as a submucosal tumor of the stomach. The tumor arose from the lesser omentum and was removed without difficulty. Histologically, the tumor was composed of spindle–shaped cells with an interlacing bundle pattern, and immunohistochemical examination showed that it was positive for myeloid stem cell antigen (CD34), but negative for HHF35 and S-100 protein. These findings were consistent with a GIST lacking myogenic features and neural attributes. The patient had an uneventful postoperative course, and was free of recurrence when last seen 11 months after his operation. Received: April 3, 2000 / Accepted: March 6, 2001     

十二指肠胃肠道间质瘤的外科治疗及预后分析

目的:探讨十二指肠胃肠道间质瘤(gastrointesinal stromal tumor,GIST)的诊断、外科治疗和预后。方法:回顾性分析2006年3月至2012年10月收治11例十二指肠GIST病人的临床资料,并与同期治疗胃和小肠GIST病人的临床特征及预后进行比较。结果:11例病人CT的检出率为100%,内镜病理检出率为25%。均行手术切除治疗。2例行胰十二指肠切除术,7例行肿瘤局部切除术,2例行十二指肠节段切除术;围手术期无病人死亡,2例分别于术后10个月和2年出现复发、转移。十二指肠GIST病人的1年和2年无复发生存率为91%(10/11)和82%(9/11),预后略差于胃和小肠GIST。结论:十二指肠GIST的术前诊断应综合应用内镜和影像学技术,以提高其确诊率。治疗首选手术切除,根据肿瘤位置和大小合理选择手术方法。低危病人手术后基本可以治愈,中、高危病人术后应配合伊马替尼辅助治疗。该病的预后略逊于胃和小肠GIST。     

Neonatal gastrointestinal stromal tumor. Report of a case and review of literature

Gastrointestinal stromal tumor (GIST) is very rare in infancy. Most of the reported cases in the literature are in adults; some are in children but there are a few reported cases in the literature. The present case is a 6-day-old female neonate presenting with lethargy, poor feeding, constipation, abdominal distention, and rectal bleeding. She was operated on with the impression of intestinal obstruction, and right hemicolectomy was performed on her. Surgical specimen showed a well-defined and round 3-cm mass in the cecal area. Diagnosis was made by histologic and immunohistochemical studies which showed a GIST. The tumor showed positive vimentin and c-kit but negative for all other markers (desmin, actin, S100, NSE, and CD-34). So the case was an undifferentiated GIST. After 1 year of follow-up the patient was completely normal.     

胃肠道间质瘤的诊治现状与进展

目的总结胃肠道间质瘤（GIST）的发病机制及流行病学特点,探讨其诊断及治疗并分析其预后。方法复习与GIST的发病机制、流行病学、诊断、治疗及预后方面有关的文献并对其进行综述。结果 GIST为非上皮源性肿瘤,起源于Cajal间叶细胞,是消化道最常见的间叶性肿瘤,约占消化道肿瘤的1%～3%,中位发病年龄为40～60岁,胃为最好发部位。目前认为,GIST的发病机制与原癌基因c-kit或血小板源性生长因子受体α（PDGFRα）基因突变有密切关系,但PDGFRα和c-kit基因突变不会同时出现在同一患者中。GIST的临床表现缺乏特异性,临床诊断主要依靠内镜及影像学技术,最终确诊依靠病理学检查。目前对GIST的治疗以手术与分子靶向药物治疗为主,其预后与肿瘤危险度分级及治疗干预密切相关。结论 GIST是具有恶性潜能的肿瘤,其危险度分级是指导临床治疗及预后评估的重要指标,GIST的预防、诊断、治疗及复发的预防有待进一步研究。     

胃肠道间质瘤同时性肝转移的同期手术切除

目的 探讨同时性胃肠道间质瘤(gastrointestinal stromal tumor,GIST)肝转移行同期切除原发肿瘤和肝转移瘤的手术疗效.方法 回顾性分析7例GIST同时性肝转移同期手术病人的临床资料.结果 7例病人中男5例,女2例;中位年龄52(43～67)岁.原发肿瘤部位分布为:胃2例,小肠5例.全组接受手术切除,无明显并发症,1例病人术后2个月死于肝内复发,1例术后8个月肝内复发者服用伊马替尼,肿瘤进展得到控制.其余病人在随访1年期间未见肿瘤复发或转移.结论 同期手术切除对于合适的同时性GIST肝转移瘤病人安全、有效.     

胃肠道间质瘤的影像学表现及其病理分析

目的分析胃肠道间质瘤的影像学特点,探讨消化道造影及CT扫描对该肿瘤的诊断价值。方法回顾性分析经临床手术病理证实的57例胃肠道间质瘤的影像学资料,并参照其病理进行良恶性分析。结果 57例胃肠道间质瘤良性9例,占15.8%,恶性48例,占84.2%;贲门胃43例,占75.4%:小肠12例,占21.1%;肠系膜2例,占3.6%。直径3.0cm5例,占8.77%;直径3.0～5.0cm23例,占40.35%;直径5.0cm29例,占50.88%。术前转移2例,占3.5%。结论 (1)消化道造影与CT扫描影像学表现有助于胃肠道间质瘤的诊断;(2)肿瘤发生的部位、大小、生长方式、强化程度、邻近组织有无受浸及转移、风险等级是胃肠道间质瘤良恶性判断的重要标准;(3)影像学表现及病理为胃肠道间质瘤的临床治疗及预后提供重要参考价值。     

The GIST of targeted cancer therapy: a tumor (gastrointestinal stromal tumor), a mutated gene (c-kit), and a molecular inhibitor (STI571)

Although gastrointestinal stromal tumor (GIST) is the most frequent mesenchymal neoplasm of the gastrointestinal tract, until recently it has been an obscure disease. Now, there is widespread scientific and clinical interest in GIST because its principal pathogenetic defect has been identified and a specific molecular inhibitor of GIST has been developed. Most GISTs contain a gain-of-function mutation in thec-kit proto-oncogene. Mutation results in constitutive activation of the Kit receptor tyrosine kinase, which induces cellular proliferation. STI571 is an oral agent that selectively inhibits Kit. It is a landmark development in cancer treatment and marks a new era of targeted molecular therapy. Its efficacy proves that a specific inhibitor can counteract the effects of a genetic defect responsible for neoplasia. Althought ST1571 was first applied to GIST only 2 years ago, it has already revolutionized the treatment of patients with metastatic disease and is also currently being tested as an adjuvant therapy after the resection of primary GIST.     

胃肠道间质瘤临床诊治分析(附48例报告)

目的探讨胃肠道间质瘤（GIST）的诊断与治疗。方法对我院1999年1月至2004年12月收治的48例GIST患者的病理特点及外科治疗进行回顾性分析。结果本组GIST发生部位：胃29例（60．4％），小肠11例（22．9％），结肠3例（6．3％），直肠4例（8．3％），肛管1例（2．1％）。主要临床表现为消化道出血（52．1％）和腹部肿块（35．4％），免疫组化表型CD117阳性率为83．3％（40／48），CD34阳性率为77．1％（37／48），SMA阳性率为27．1％（13／48），S-100阳性率为22．9％（11／48）。48例均行手术切除。全组术后均未行放、化疗，随访12-60个月，平均35个月，随访率为100％。良性及交界性患者均无复发；13例恶性者中6例复发，其中5例死亡。结论GIST在中老年人中好发，部位以胃及小肠最多见，以消化道出血、腹部肿块表现为主。肿瘤的大小是GIST良、恶性的重要临床指标。GIST的诊断有赖于病理形态学检查与免疫组化的结合。完整的局部手术切除是最有效的治疗手段。