Peritonitis secondary to spontaneous perforation of a primary gastrointestinal stromal tumour of the small intestine: A case report and a literature review

IntroductionA few cases of acute abdomen caused by perforation of small-intestinal gastrointestinal stromal tumours (GISTs) have been reported in the literature.Presentation of caseTogether with a review of the published cases, here we report a case of an elderly patient with peritonitis due to spontaneous perforation of a GIST of the jejunum. An 82-year-old man was admitted to the emergency unit of our hospital with fever and severe abdominal pain. An abdominal enhanced computed tomography scan detected a 6 cm solid mass in the left upper quadrant adherent to a jejunal loop and surrounded by free fluid and free air. Due to the radiological features of the mass, the diagnosis of a perforation of a GIST arising from the jejunum wall was suspected. The patient underwent emergency laparotomy. Intraoperative findings confirmed diffuse peritonitis secondary to jejunal tumour perforation. A segmental resection of the jejunum containing the mass was performed followed by a mechanical end-to-side anastomosis. The histopathologic examination of the mass confirmed the diagnosis of a perforated GIST of the small intestine (high-risk category). The post-operative course was uneventful and the patient was treated with adjuvant imatinib therapy.DiscussionTwenty-one other cases of spontaneous perforation of small intestine GISTs are reported in the literature and are summarized in the present review.ConclusionThe described case is the tip of the iceberg and spontaneous rupture or perforation of GISTs are a far more frequent first presentation of this rare tumour.     

Malignant rhabdoid tumours of the small intestine with multiple organ involvement: Case report

Introduction and importanceMalignant rhabdoid tumours (MRTs) were first described as an infrequent variant of Wilms’ tumour and have been reported in several organs. The small intestine is a rare site for MRTs.Case presentationA 70-year-old man presented with appetite loss and melena. Haemorrhagic small intestinal tumours, swollen mesenteric and paraaortic lymph nodes, a tumour in the left kidney, and multiple tumours in the lung were found. He underwent partial resection of two haemorrhagic small intestinal tumours classified as MRTs based on the results of a pathological examination. However, melena appeared again on postoperative day 6. We performed another operation and resected approximately 180 cm of the small intestine that contained multiple tumours. All lesions were classified as MRTs. Unfortunately, melena appeared again 4 days after the second operation. He did not want invasive therapy and died from massive melena 2 months after the initial surgery.Clinical discussionMRTs of the small intestine are uncommon and have an extremely poor prognosis. Although curative resection is an important treatment, cases of metastasis at diagnosis and postoperative early recurrence have been observed, as was the case for the patient described herein. In these cases, effective systemic therapy is necessary. Recently, tumour suppressor genes were shown to be involved in the occurrence of MRT, and new therapies for MRT have been studied.ConclusionWe herein conclude effective systemic therapy is necessary for MRTs with multiple organ involvement. The development of new drugs for this disease is ongoing.     

Cold preservation of the small intestine with the new Celsior-solution

The aim of the present study was to evaluate the potential of Celsior, a recently developed cardioplegic and heart storage solution, to protect the small bowel during ischemic storage. Small bowel segments were isolated from rats, flushed with either UW or Celsior solution, and cold-stored for 18 h at 4 °C in the respective solution. After ischemic storage, some preparations were freeze-clamped for analysis of tissue metabolites while other preparations were tested for structural and functional integrity by isolated perfusion in vitro using a previously validated model. After 18 h of ischemic storage no significant differences were seen between Celsior and UW with regard to the development of edema, energy charge, or creatine phosphate, but lactate accumulation was significantly reduced in the Celsior group, although glucose catabolism was not inhibited. Histological evaluation of the cold-stored organs showed no differences with regard to structural integrity between the two groups. Total vascular resistance upon reperfusion was significantly lower in the Celsior group (666 ± 126 vs 827 ± 88 MPa s m^{–3 *}), as was the intestinal release of LDH (9.7 ± 4.4 vs 18.2 ± 4.6 U/l *). Carbohydrate absorption from the intestinal lumen amounted to venous effluent concentrations of 0.58 ± 0.24 vs 0.18 ± 0.15 mg% ^* of galactose in the Celsior and UW groups, respectively. Within the limits of this in vitro pilot study, Celsior provided better postischemic recovery of the small bowel than UW in terms of vascular perfusion characteristics, enzyme release, and carbohydrate absorption and may, thus, be considered a suitable alternative for intestinal organ preservation. Received: 13 June 1997 Received after revision: 19 September 1997 Accepted: 8 October 1997     

Laparoscopic Management of Gastrointestinal Stromal Tumours

Gastrointestinal stromal tumours (GISTs) represent the most common non-epithelial tumour of the digestive tract. Laparoscopic approach may be considered ideal for the resection of these tumours. Their particular biological behaviour allows for a curative resection even without large resection margins and extensive lymphadenectomies. Method: Over a period of five years, eight patients benefited from laparoscopic resections (six patients with primary gastric GIST and two patients with small bowel GIST). Pathological features were analyzed including tumour size, surgical margin status, immunohistochemical staining profile, and tumour mitotic index. All cases were confirmed GISTs by immunohistochemical staining.

Results: The mean age of the patients was 61.6 years (range 48–71years). Of these patients, five initially presented to hospital with acute gastrointestinal haemorrhage (four cases) or occult bleeding (one case). Gastric or small bowel tumours were identified during subsequent investigation. A presumptive diagnosis of gastric GIST was made in gastric lesions based on endoscopic, ultrasonic, and CT scan characteristics. Small bowel tumours were identified and presumed to be GIST based on information gathered from video capsule endoscopy, enteroscopy and CT scan. Complete resection was obtained with laparoscopic approach in six cases, while in two cases resection was laparoscopically assisted, with added oncology safety margins as preoperative definitive diagnosis was not available. Tumours varied in dimension from 2.5cm to 9 cm and their malignant risk score using Fletcher criteria was low in three cases, intermediate in three cases and high in two cases. Mean postoperative stay was 3.8 days and there were no complications, nor postoperative mortality. After a mean follow-up of 26 months (range 1 to 60 months) all patients are symptom free and free of recurrent disease.

Conclusions: A selective approach to laparoscopic resection of gastro-intestinal GISTs allows safe resection and very good results, on the condition that a clear diagnosis of GIST has been established on preoperative assessment. The laparoscopic feasible and safe considering the biological particularities of GIST, and it carries no additional risks.     

Small bowel tumours in emergency surgery: specificity of clinical presentation

BACKGROUND: Despite advances in diagnostic modalities, small bowel tumours are notoriously difficult to diagnose and are often advanced at the time of definitive treatment. These malignancies can cause insidious abdominal pain and weight loss, or create surgical emergencies including haemorrhage, obstruction or perforation. The aim of the present study was to describe the clinical presentation, diagnostic work-up, surgical therapy and short-term outcome of 34 patients with primary and secondary small bowel tumours submitted for surgical procedures in an emergency setting and to look for a correlation between clinical presentation and the type of tumours. METHODS: From 1995 to 2005, 34 consecutive surgical cases of small bowel tumours were treated at the Department of Emergency Surgery of St Orsola-Malpighi University Hospital, Bologna, Italy. Clinical and radiological charts of these patients were reviewed retrospectively from the department database. RESULTS: All patients presented as surgical emergencies: intestinal obstruction was the most common clinical presentation (15 cases), followed by perforation (11 cases) and gastrointestinal bleeding (eight cases). Lymphoma was the most frequent histologic type (nine patients), followed by stromal tumours (eight patients), carcinoids (seven patients), adenocarcinoma (seven patients) and metastasis (three patients). Of the nine patients with lymphoma, eight were perforated, all patients with stromal tumours had bleeding, and all carcinoids patients had bowel obstruction. There were two patients with melanoma metastasis, both had bowel intussusception. Resection of the neoplasm was carried out in 32 patients and two patients were deemed unresectable and received a palliative procedure. CONCLUSIONS: The present study shows that there is a correlation between small bowel tumours and clinical emergency presentation: gastrointestinal stromal tumours (GIST) mostly bleed; carcinoids make an obstruction; lymphomas cause a perforation; and melanoma metastasis causes intussusception.     

Adenocarcinoma of the small intestine: 21-Year review of diagnosis,treatment, and prognosis

Background: The purpose of this study was to evaluate the tumor characteristics and treatment associated with an improved overall survival in patients with adenocarcinoma of the small intestine. Methods: The records of all patients with primary adenocarcinoma of the small bowel seen between January 1971 and December 1991 were reviewed retrospectively. The study comprised 38 patients, 22 (58%) with duodenal tumors, 11 (29%) with jejunal tumors, and five (13%) with ileal tumors. Results: Although not statistically significant, the patients with duodenal adenocarcinoma lived longer than the patients with jejunal or ileal lesions (p=0.77). The overall survival was 23% and seemed to correlate best with absence of lymph node metastases (p=0.04) and pancreaticoduodenectomy for localized duodenal tumors (p=0.04). The patient's age, duration of symptoms, disease-free interval, tumor location, type of recurrence, and histologic grade did not significantly influence survival. Conclusions: The lethality of small-intestinal adenocarcinoma appears to be related to a delay in diagnosis and treatment. When a definitive surgical procedure is performed before lymph node metastases appear, the patient's chance for long-term survival is greatly improved.Presented at the 46th Annual Cancer Symposium of The Society of Surgical Oncology, Los Angeles, California, March 18–21, 1993.     

胃肠道间质瘤的规范化治疗

Gastrointestinal stromal tumors (GISTs) is a subset of mesenchymal tumors and represents the most common mesenchymal neoplasm of gastrointestinal tract.In the last decade,GISTs has become well known because of the effectiveness of imatinib mesylate.The molecular targets for tyrosine kinase receptor inhibitor are not only of importance for the treatment of patients but also useful for the development of a novel drug modalities and new strategies in basic cancer therapy.The improved understanding of the molecular mechanism of GISTs has made its diagnosis standardized.Many multicenter phase Ⅱor phase Ⅲ clinical trials have been completed to establish the role of adjuvant and neoadjuvant therapy.It is important to select the correct management strategy,which is multidisciplinary,integrated,and individualized.This paper provides an overview of the rational treatment of GISTs at present.     

The use of small intestine in bladder reconstruction

Reconstruction of the bladder is a treatment available to patients who have a diseased or damaged bladder, and small bowel is the most commonly used tissue. Augmentation cystoplasty increases the total bladder capacity, whereas substitution cystoplasty replaces the whole organ. This is either drained through a continent cutaneous stoma or is reanastomosed to the urethra as an orthotopic reconstruction. Although the treatment for invasive bladder cancer has not changed greatly in the last few decades, the use of orthotopic bladder reconstruction allows for a great improvement in the quality of life for patients who undergo cystectomy. These reconstructive techniques can also be offered to patients with other forms of pelvic malignancy that involve the bladder.     

小肠胃肠间质瘤64例治疗与预后分析

目的探讨小肠胃肠间质瘤（GIST）的治疗及预后影响因素。方法回顾性分析天津医科大学附属肿瘤医院2002年4月至2010年11月收治的经手术治疗且有完整随访资料的64例小肠GIST患者的临床和随访资料。结果全组患者术后均未接受化、放疗．有14例患者术后予以伊马替尼靶向治疗。64例小肠GIST患者5年生存率为51．2％;61例R0切除患者术后复发转移27例（44．3％）。单因素预后分析显示,手术方式（P=0．001）、肿瘤大小（P=0．018）、周围组织粘连侵犯（P=0．015）、伴发远处转移（p=0．000）、肿瘤坏死出血（p=0．032）、F1etcher分级（P=0．027）及就诊时症状（p=0．012）与小肠GIST患者预后有关。多因素预后分析显示,周围组织粘连侵犯（P=0．026）、伴发远处转移（P=0．000）和就诊时症状（P=0．019）是小肠GIST患者预后的独立影响因素。结论评估小肠GIST患者的预后,应结合手术方式、肿瘤大小、与周围组织脏器有无粘连侵犯、有无伴发远处转移、有无肿瘤坏死出血、就诊时症状、Fletcher分级以及靶向治疗情况进行多方面考虑。     

Small bowel cancer: A 30-year review

Background: Small bowel cancer is a relatively rare tumor with an incidence of 2,700 new cases and 900 deaths per year. The influence of stage on survival has been reported only once previously. Patterns of recurrence are unreported. Methods: All cases of small bowel cancer treated at our hospital over a 30-year period (1960–1989) were reviewed. Results: The site of most cancers was the duodenum (46%), followed in frequency by the jejunum (33%) and the ileum (21%). Adenocarcinoma was the most common histology (63%), followed in frequency by lymphoma (15%), leiomyosarcoma (13%), carcinoid tumors (6%), and miscellaneous (3%). Analysis of stage distribution by site showed a decrease in stages I and II with more distal locations. Associated cancers occurred in 11%, but none were seen in the group with carcinoid tumors. Actuarial 10-year survival rates were 24% for those with adenocarcinoma (all stages) 75% for stage I, 25% for those with stage II, and 0% for stage III. A subgroup of 10 patients who underwent a pancreaticoduodenectomy (one stage I, seven stage II, two stage III) had a 30% 10-year survival rate. Those patients with lymphoma had a 12% 10-year survival rate, and those with leiomyosarcoma had a 20% 10-year survival rate. A 100% 10-year survival rate was observed in those with carcinoid tumors. Peritoneal carcinomatosis was the most common failure pattern (33%), followed in frequency by local recurrence in 23% and abdominal wall recurrence in 15%. Conclusions: A correlation exists between the pathologic stage and the survival rate for adenocarcinoma. The most common recurrence pattern for adenocarcinoma was carcinomatosis, followed in frequency by abdominal wall recurrence. Leiomyosarcoma preferentially metastasizes to the liver. The results of this study were presented at the 46th Annual Cancer Symposium of The Society of Surgical Oncology, Los Angeles, California, March 18–21, 1993.     

小胃肠道间质瘤的诊断和治疗策略

长期以来胃肠道间质瘤(GIST)的实际发病率常被低估.随着对这一疾病的认识和检查技术的进步,小GIST患者(直径＜5 cm)的检出率逐年升高.该类患者多无明显临床症状,常在体格检查被意外发现.对于这类患者的诊断需要联合内镜和CT检查.在明确诊断的基础上,对直径t≥2 cm的小GIST患者应积极行肿瘤的完整切除术,而目前腹腔镜手术逐渐成为治疗的标准术式.对于直径＜2 cm的微小GIST,特别是微小胃间质瘤,目前的指南推荐可以行密切随访,有学者提倡内镜切除肿瘤.笔者认为应当谨慎鉴别小GIST恶性潜能而后选择个体化治疗策略.     

腹腔镜小肠胃肠道间质瘤切除术临床分析(附65例报告)

目的:探讨腹腔镜手术治疗小肠胃肠道间质瘤(gastrointestinal stromal tumor,GIST)的安全性、可行性和临床疗效。方法:收集我院2005年1月至2013年1月腹腔镜手术治疗的65例小肠GIST病人的临床资料,分析术后恢复及疗效。结果:65例病人均顺利完成腹腔镜手术,无中转开腹。平均手术时间(85±38)min,平均手术出血量(20±12)mL,平均切口长度(4±1)cm。术后中位排气时间为2(1~4)d,中位住院时间为7(5~20)d。4例(6.1%)发生术后并发症,经非手术治疗后痊愈。中位随访时间50(6~102)个月,术后死亡1例、复发2例。结论:腹腔镜手术治疗小肠GIST是安全、可行的,具有创伤小和恢复快等优势,并能弥补内镜和影像学诊断的不足。     

Small,spontaneously ruptured gastrointestinal stromal tumor in the small intestine causing hemoperitoneum: A case report

IntroductionGastrointestinal stromal tumors (GISTs) are clinically asymptomatic until they reach a significant size; therefore, GISTs that are 2 cm or less are typically asymptomatic. Patients with symptomatic GISTs typically present with abdominal pain, gastrointestinal bleeding, or a palpable mass but rarely present with hemoperitoneum.Presentation of caseA 72-year-old Japanese man presented to us with acute onset abdominal pain. Physical examination showed peritoneal irritation in the lower abdomen. Findings of abdominal computed tomography were suggestive of hemoperitoneum; therefore, urgent surgery was performed. Approximately 1500 ml of blood in the abdominal cavity was removed. A small, ruptured mass was found in the middle of the small intestine, and partial resection of the small intestine, including the mass, was performed. The resected tumor was 2 cm in size and exhibited an exophytic growth pattern. Immunohistochemical staining revealed that the tumor was positive for KIT and CD34; therefore, a final diagnosis of GIST was made. Treatment with imatinib at 400 mg per day was started from postoperative month 1. The patient is doing well without recurrence 5 months after surgery.DiscussionEven small GISTs in the small intestine can spontaneously rupture and cause hemoperitoneum. Moreover, when a patient presents with sudden abdominal pain and hemoperitoneum without an evident mass on imaging, clinicians should be aware of the possibility of bleeding from a small GIST in the small intestine.ConclusionWe present an extremely rare case of a patient with a small, spontaneously ruptured GIST in the small intestine, resulting in hemoperitoneum.     

胃和小肠间质瘤的预后因素分析

目的分析胃和小肠间质瘤的预后因素。方法回顾性分析1995年1月至2005年1月北京大学临床肿瘤学院收治的105例胃肠道间质瘤病人的临床资料。结果小肠间质瘤初诊即为晚期者较多，因而根治性切除比例低。结论多因素分析发现，肿瘤部位和病期过晚是胃和小肠间质瘤的独立预后因素。     

胶囊内镜在小肠疾病诊断中的价值

目的探讨胶囊内镜对小肠疾病的诊断价值。方法2002年9月至2007年3月间对155例患者所进行的159例次胶囊内镜检查进行回顾性研究。记录胶囊内镜在胃和小肠的平均运行时间,评价患者的耐受性、胶囊内镜完成检查情况及胶囊内镜下病变的检出情况等。结果155例患者中,不明原因的消化道出血97例,腹痛42例,腹部不适6例,腹泻4例,体检6例。93．1％的患者（148／159）完成了全小肠摄影。所有患者在检查过程中未诉特殊不适。胶囊内镜胃内平均停留时间为65．5（1～335）min,小肠平均停留时间为282．2（45～524）min。胶囊内镜病变检出率为78．6％（125／159）。胶囊内镜发现血管病变43．4％（69／159）,小肠炎性病变28．3％（45／159）,黏膜下结节10．1％（16／159）,小肠憩室8．2％（13／159）,小肠肿物5．7％（9／159）以及小肠息肉、小肠异物、小肠寄生虫等。不明原因消化道出血病变检出率为89．7％（87／97）,腹痛查因病变检出率为73．8％（31／42）。结论胶囊内镜检查安全性较高,患者容易接受。胶囊内镜对小肠疾病、尤其消化道不明原因出血者具有重要的诊断价值。     

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.     

Management of complications in surgery of the small intestine

Summary BACKGROUND: Complications in surgery of the small intestine develop after operations of the small intestine itself, or more often, after other abdominal surgery. METHODS: Review. RESULTS: Procedures involving a risk of small intestinal complications include formation and closure of ileostomies, surgery for Crohn's disease and, alternatively, surgical therapy of acute occlusive mesenteric ischemia, adhesions, intestinal obstruction and peritonitis. Enterocutaneous fistula due to iatrogenic lesions or in the course of peritonitis therapy as well as short bowel syndrome resulting from extended resections cause significant morbidity and mortality. CONCLUSIONS: This review summarizes the variety of diseases and surgical procedures leading to complications and discusses the appropriate therapy.      

小肠间质瘤41例的预后分析

目的 探讨影响小肠间质瘤预后的因素.方法 回顾性分析2005年1月至2010年12月河南省肿瘤医院普外科手术治疗的41例小肠间质瘤患者的临床及随访资料,进行NIH恶性危险度分级,采用Kaplan-Meier法比较不同因素对生存率的影响,并用COX多因素回归分析对该组病例进行预后分析.结果 本组41例小肠间质瘤患者位于十二指肠12例,空回肠29例,其中有消化道出血16例.有消化道出血史患者术后3年生存率为22％,而无出血史者为65％,两者相比差异有统计学意义(x2=6.613,P=0.012).COX多因素回归分析表明肿瘤原发部位、复发转移及消化道出血是小肠间质瘤预后的有效预测指标.结论 消化道出血是小肠间质瘤最常见的临床症状,肿瘤原发部位、复发转移及消化道出血是影响小肠间质瘤预后的独立影响因素.     

外伤性小肠破裂的诊断和治疗

目的总结外伤性小肠破裂的诊断和治疗经验，以期提高诊治水平。方法回顾性分析我院2003年1月-2004年12月60例外伤性小肠破裂的诊断和治疗的临床资料。结果本组60例均行手术治疗．其中治愈58例，死亡2例。1例死于MODS，另1例死于术后腹腔内大出血。术后并发症是MODS1例，出血性休克1例，伤口感染4例和不完全粘连性肠梗阻1例。结论早期诊断，正确的术前、术中处理，术后严密观察病情，及时发现和积极处理术后并发症是提高外伤性小肠破裂治愈率和降低死亡率的关键。