Solitary Fibrous Tumor of the Greater Omentum,Mimicking Gastrointestinal Stromal Tumor of the Small Intestine: A Case Report

Solitary fibrous tumor (SFT) is one of the mesenchymal tumors, which rarely arises in the abdominal space. We report a very rare case of abdominal SFT, mimicking another mesenchymal tumor. A 52-year-old Japanese man was referred to our hospital for further evaluation and treatment of gallbladder polyp. Contrast-enhanced computed tomography (CT) showed an enhanced nodule within the gallbladder, and incidentally, also showed a well-circumscribed mass adjacent to the small intestine. The mass was depicted as slightly high density in plain CT, and with contrast-enhancement, the mass was partially stained in early phase and the stained area spread heterogeneously in delayed phase. Magnetic resonance imaging showed that the abdominal mass was depicted as slightly high intensity on T2-weighted imaging and low intensity on T1-weighted imaging. With double-balloon endoscopy and capsule endoscopy, we did not find any tumor inside the small intestine. These visual findings lead us to diagnose it as gastrointestinal stromal tumor of the small intestine with extraluminal growth. We planned to resect both the gallbladder polyp and the intraperitoneal tumor at the same time for pathologic diagnosis and treatment. When the operation was performed, we found a milk-white lobulated tumor on the greater omentum and the tumor was entirely resected. Microscopically, the gallbladder polyp was diagnosed as tubular adenoma, and the omental tumor was diagnosed as SFT. It is important to bear in mind that omental SFTs sometimes mimic other mesenchymal tumors and should be included in the differential diagnosis of abdominal tumor not revealed by endoscopy.Key words: Mesothelial tumor, Extrathoracic locationSolitary fibrous tumor (SFT) is one of the mesenchymal tumors, which was initially thought to usually arise in the pleura. In recent days, however, there have been several previously reported cases of extrapleural SFT; for example, orbit, thyroid, breast, and so on.¹ Nevertheless, SFTs arising in the abdominal space are still rare; in particular, there have been few reports of omental SFT in the past literature. We herein report a case of SFT of the greater omentum, which mimicked another mesenchymal tumor.     

胃肠道间质瘤92例

目的:探讨胃肠道间质瘤的特点及诊疗方法.方法:对92例患者分别行胃镜下肿瘤局部切除、胃大部分切除、胃楔形切除、腹腔镜下胃楔形切除、胰十二指肠切除、肠段切除、腹会阴联合切除及肿块局部切除等手术,其中4例术后服用甲磺酸伊马替尼巩固治疗.结果:术中及围手术期无死亡, 住院时间2～32 d,平均11.6 d.5例于术后21～73个月死亡,平均52.4个月.结论:缺乏特征性临床表现,目前治疗仍以手术切除为主,术中应行肿瘤局部切除或消化道部分切除.     

Malignant Gastrointestinal Stromal Tumors of the Small Intestine: A Review of 50 Cases From a Prospective Database

Background: Malignant gastrointestinal stromal tumors (M-GIST) are rare mesenchymal tumors originating in the wall of the gastrointestinal (GI) tract. Previous studies have included limited numbers of patients, and most included malignant and benign cases from throughout the GI tract. We reviewed the experience of a single tertiary cancer care center with M-GIST of the small intestine only.Methods: A prospective database identified all patients seen from 1989 to 1998. Clinical and pathological data, treatment, and outcome were analyzed. Overall median follow-up time was 24 months (range, 1-176 months).Results: Fifty patients (31 male, 19 female) were identified. Mean age at diagnosis was 55 years. Disease was localized in 11 patients, locally advanced (invasion into adjacent organs/peritoneum) in 24 patients, perforated in 4 patients, multiple primary lesions in 2 patients, and distant metastases in 9 patients. All patients underwent resection, which was complete in 70%. Locoregional recurrence (LR) developed in 43% (median, 25 months), and distant metastases in 59% (median, 21 months) of patients at risk. At last follow-up, 14 patients were alive (6 disease-free), 2 had died disease-free, and 34 died with recurrent disease. Overall survival (OS) was similar for localized and locally advanced disease; OS also was similar for patients with multiple primaries and distant metastases at diagnosis. Patients were grouped into three stages: (I) patients with localized and locally advanced disease; (II) patients with perforated; and (III) patients with multiple primaries and distant metastases. Actuarial OS at 5 years was 41% (n = 50)—42% for those with complete resection and 8% for incomplete resection. Univariable analysis showed that earlier stage at diagnosis (P = .001) and completeness of resection (P = .004) predicted for longer OS.Conclusions: Most patients with M-GIST of the small intestine relapse following resection, but survival may be prolonged. In univariable analysis, stage at presentation and complete resection were significant prognostic variables for OS; grade was not significant. Localized and locally advanced M-GIST of the small intestine have a mean OS > 5 years. Complete resection should be the goal of initial surgical treatment.Presented at the 53rd Annual Meeting of the Society of Surgical Oncology, New Orleans, Louisiana, March 16-19, 2000.     

胃肠道间质细胞肿瘤43例临床及病理分析

目的:探讨胃肠道间质瘤临床表现和良性、潜在恶性、恶性的病理诊断标准,及其对临床评估预后的价值.方法:选用43例临床资料完整的病例,经免疫组化S-P方法测定CD117、CD34、SMA、S-100、NSE及组织化学Masson染色.结果:43例免疫组化阳性率CD117 40/43例(93.0%);CD34 34/43例(79.1%);CD117及CD34皆阳性30/43(69.8%),SMA 16/43例(37.2%)向平滑肌分化;S-100阳性或兼有NSE阳性14/43例(32.6%)向神经鞘分化;6/43例(14.0%)双向分化.良性5例,潜在恶性10例,恶性28例,预后与肿瘤病理判定密切相关.结论:胃肠道间质瘤的病理诊断除结合临床各项检查外,仍需依靠免疫组化测定(CD117及/或CD34),其中必须一项呈阳性者方能确定诊断.     

Gastrointestinal Stromal Tumor of the Rectum: An Analysis of Seven Cases

Purpose Gastrointestinal stromal tumors (GISTs) rarely originate in the rectum. We investigated the clinicopathologic characteristics of rectal GISTs. Methods We analyzed the medical records of seven patients who underwent surgery for GIST of the rectum between 1998 and 2003. Results There were two men and five women with a median age of 55 years (range, 41–72 years) at the time of diagnosis. The median follow-up period was 23 months (range, 7–75 months). The chief symptoms were hematochezia, constipation, and anal pain. All patients underwent curative resection; in the form of abdominoperineal resection in five patients, transanal excision in one, and Hartmann's operation with prostatectomy in one. The median tumor size was 6.6 cm (range, 1–12 cm). Four patients received adjuvant radiation therapy. Local recurrence developed in two patients; 54 months and 23 months after surgery, respectively. Conclusion The common symptoms of rectal GIST were the same as those of other rectal tumors. Curative surgical resection should be done, but further studies are necessary to investigate better adjuvant treatment strategies for patients with rectal GISTs     

腹腔镜辅助小肠间质瘤切除术15例

目的探讨腹腔镜辅助小肠间质瘤切除术的疗效。方法2003年1月一2007年5月，10例小肠占位性病变、5例腹痛伴便血诊断不清共15例在全麻下行腹腔镜探查，发现小肠病变后在腹壁合适部位做小切口，将病变肠段提出腹壁外切除、吻合，回纳肠段关腹后重建气腹，观察肠段血运及腹腔内有无出血。结果15例手术均顺利完成。术后诊断为间质瘤，CD117阳性14例（93％），CD34阳性9例（60％）。极低危险性3例（20％），低危险性5例（33％），中危险性4例（27％），高危险性3例（20％）。肿瘤直径1．5—3．6cm，（2．44±0．63）cm。手术时间38—72min，（57．8±10．4）min。术中出血10—30ml，（20．1±6．5）ml。术后排气时间16—42h，（27．8±9．3）h。术后住院4—7d，（5．3±1．1）d。无并发症发生。15例随访5～36个月，（24．3±8．4）月，无复发。结论腹腔镜辅助小肠间质瘤切除术具有良好效果。     

胃肠道间质瘤20例诊治体会

目的探讨胃肠道间质瘤（gastrointestinal stromal tumor,GIST）的临床特点与诊治经验。方法GIST患者20例,病变位于胃14例,十二指肠2例,小肠3例,肠系膜1例;行内镜检查17例,CT检查20例,消化道造影检查3例,术前确诊3例。20例均行手术治疗：远端胃大部切除术8例,近端胃大部切除术4例（其中1例加行胰体、尾及脾切除术）,胃楔形切除术2例,胰十二指肠切除术2例,小肠部分切除吻合术3例,肠系膜间质瘤切除加行小肠部分切除吻合及结肠部分切除吻合术1例。结果免疫组织化学染色检查：CD117阳性19例（95%）,CD34阳性17例（85%）,S100阳性6例（30%）,SMA阳性6例（30%）。17例获随访,平均2年（2个月~5年）。9例死亡,8例已生存超过2年,其中5例无瘤生存,3例带瘤生存者予口服伊马替尼治疗,肿瘤未见明显增大。结论GIST术前确诊率低,内镜及影像学检查对诊断很有一定价值,手术是主要治疗方式,分子靶向治疗具有辅助意义。     

Transsacral Approach to Resect a Gastrointestinal Stromal Tumor in the Rectum: Report of Two Cases

Gastrointestinal stromal tumors (GISTs) rarely arise in the rectum. Whereas a local resection with negative margins is generally considered adequate for resectable GISTs, a wide resection is usually indicated for rectal lesions because of the technical impossibility of local resection. We report the cases of two patients who underwent resection of a rectal GIST using a transsacral approach. Both patients had an uneventful postoperative course, and no evidence of recurrence has been identified. The transsacral approach appears to be less invasive and should be considered as the treatment of choice for a rectal GIST.     

Gastrointestinal Stromal Tumor of the Rectum: Results of Surgical and Multimodality Therapy in the Era of Imatinib

Background

The rectum is a rare site of gastrointestinal stromal tumor (GIST), and factors determining long-term outcome remain unclear. In a population study, we assessed the outcome of rectal GIST patients treated at two referral centers.

Methods

A total of 39 patients diagnosed with rectal GIST between January 2002 and December 2010 were identified in prospective databases. Tumor and patient characteristics, treatment details, and outcome were evaluated. Median follow-up was 41 (3–110) months.

Results

A male predominance was noticed (M/F = 29/10). Median age was 53 years (range, 32–80 years). The cohort included, of 39 patients, 12 low-risk, 26 high-risk, and 1 with M1 disease. Of 38 patients with nonmetastatic disease, 36 underwent surgery as transabdominal (15 of 36) or local (21 of 36) resection. There were 21 patients who received preoperative and/or postoperative imatinib treatment. Patients with preoperative imatinib (16 of 36) had a significantly higher rate of R0 resections (p = .02). Five patients developed local recurrences. All of them had undergone local tumor excision with positive margins and without perioperative imatinib. Also, five patients suffered from distant metastases. All belonged to the high-risk group and underwent tumor surgery (3 R0, 2 R1) without receiving perioperative imatinib. A total of three patients died of disease. Perioperative imatinib was associated with improved local disease-free, disease-free, and overall survival (p < .01, p < .01, and p = .03, respectively). Local disease-free survival was significantly improved by negative resection margins (p < .01).

Conclusions

Complete resection is recommended to achieve local disease control. Preoperative imatinib was associated with improved surgical margins. Perioperative imatinib was associated with improved local disease-free, disease-free, and overall survival.     

胃肠道间质瘤的临床分析

目的探讨胃肠道间质瘤的临床诊断与治疗。方法回顾性分析95例胃肠道恶性间质瘤病人的临床资料。结果发生于胃、小肠、结直肠、肠系膜、大网膜的病例分别为65、25、2、2、1例；肿瘤直径1～10cm；其中恶性者47例，良性者48例；行根治性切除62例，行切除术31例，2例广泛转移者行肝结节活检。17例术后发生伤口感染，免疫组化指标：CD117、CD34、CK、EMA、S-100及Desmin阳性率分别为89．5％、86．3％、5．3％、0、9．5％及8．4％。随访80例，2例胃间质瘤广泛转移者分别于术后4月、6月死亡，17例术后5～25月死于其他疾病，余者均无瘤生存，5年生存率43．7％。结论提高对胃肠道间质瘤的认识，强化术中病理学检查及免疫组化检查，确定良恶性及恶性程度，采取合理规范的手术方式，对预后及防止复发有一定意义。     

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。     

Additional Primary Malignancies in Patients with Gastrointestinal Stromal Tumor (GIST): A Clinicopathologic Study of 260 Patients with Molecular Analysis and Review of the Literature

Annals of Surgical Oncology - The incidence of other primary neoplasms in gastrointestinal stromal tumor (GIST) patients is relatively high. Our aim was to better characterize the clinicopathologic...     

Gastrointestinal Stromal Tumor of the Stomach: Report of a Case

We report herein the case of a 70-year-old woman found to have a gastrointestinal stromal tumor (GIST) of the stomach. Preoperative X-ray and endoscopic examination revealed a hemispheric submucosal tumor with central depression in the anterior wall of the gastric fornix. The tumor, which was 3 cm in diameter, was resected by a laparoscopy-assisted procedure. Histologic examination revealed that it was composed of spindle-shaped cells with elongated nuclei, and few mitoses. Most of the tumor cells showed immunoreactivity for vimentin and CD34, but not for α-smooth muscle actin, desmin, or S-100 protein. The PCNA index was 40.5%. Thus, the GIST did not show differentiation toward smooth muscle or neural cells. A gastrectomy was not performed because the small size of the tumor, and the paucity of the mitoses indicated that it was benign. Nevertheless, careful and long-term follow-up is needed to monitor for signs of possible local recurrence or distant metastases. Received: February 9, 2000 / Accepted: September 26, 2000     

Blood Neutrophil-to-Lymphocyte Ratio is Prognostic in Gastrointestinal Stromal Tumor

Background

The immune system has been shown to play an important role in gastrointestinal stromal tumor (GIST). The neutrophil-to-lymphocyte ratio (NLR) in blood is an easily assessable parameter of systemic inflammatory response. The aim of this study was to determine whether the NLR is prognostic in GIST.

Methods

A total of 339 previously untreated patients with primary, localized GIST operated at our institution between 1995 and 2010 were identified from a prospectively collected sarcoma database. NLR was assessed preoperatively. Patients who received adjuvant imatinib treatment were excluded from the analysis (n = 64). Cox regression models were calculated and correlation analyses were performed.

Results

On univariate analysis, NLR was associated with recurrence-free survival (RFS) (P = 0.003, hazard ratio 3.3, 95 % confidence interval 1.5–7.4). Patients with a low NLR had a 1- and 5-year RFS of 98 and 91 %, compared with 89 and 76 % in those with a high NLR. The median RFS was not reached. Positive correlations were found between NLR and mitotic rate (Pearson correlation coefficient [r] = 0.15, P = 0.03), and NLR and tumor size (r = 0.36, P = 0.0001). RFS in patients with a GIST >5 cm with low NLR was significantly longer compared to patients with high NLR (P = 0.002). Flow cytometry analysis of freshly obtained GISTs revealed that neutrophils constituted a minimal percentage of intratumoral immune cells.

Conclusions

NLR is a surrogate for high-risk tumor features. Elevated blood NLR appears to represent systemic inflammation in patients with high-risk GIST.     

Laparoscopic Pancreas-Sparing Subtotal Duodenectomy in Gastrointestinal Stromal Tumor of the Duodenum

Background

The duodenum is a rare origin for gastrointestinal stromal tumors (GISTs).1 ^, 2 A decision of pancreatoduodenectomy or limited resection is a dilemma for surgeons. Recent reviews have suggested that types of surgery did not influence prognosis and limited resection was indicated for small GIST located some distance away from the ampulla of Vater (AOV).3 ^, 4 However, a laparoscopic, pancreas-preserving, subtotal duodenectomy was rarely performed.5 ^, 6

Methods

A 20-year-old female was referred to our institution because of a duodenal submucosal mass. Computer tomography and endoscopy revealed a 3.8-cm–sized mass that was ～2 cm from AOV. A minimally invasive and function-preserving resection was scheduled.

Results

Meticulous dissection of the duodenum from the pancreatic head was a critical point. Even small breakages of vessels could provoke massive bleeding, possibly resulting in the surgeon’s view being obstructed, longer operating times, or a decreased chance of performing a minimally invasive and limited resection. Therefore, an especially meticulous and careful dissection was performed. An upper gastrointestinal series revealed no leakage, and the patient received a soft diet on postoperative day 3. The patient was discharged on postoperative day 8. Pathologic examination reported a low-risk GIST group.

Conclusions

Although clearly malignant tumors are not suitable for this approach due to poor oncologic outcomes, laparoscopic pancreas-preserving subtotal duodenectomy is a feasible and effective strategy to treat benign or borderline tumors. This approach will offer successful oncologic results and laparoscopic merits. We feel that this demonstration would advocate clinical feasibility of minimally invasive and function-preserving resections in well-selected duodenal GISTs.     

Giant Gastrointestinal Stromal Tumor of the Stomach in An Elderly Patient

We report a case of giant gastrointestinal stromal tumor (GIST) of the stomach of 17 cm in diameter detected in an 88-year-old Caucasian female. An en-block resection of the mass requiring gastric and transverse colon resection was carried out. Pathological examination evidenced a smooth multycistic giant gastric GIST measuring 17 × 13 × 9 and weighing 1,630 g. At immunohistochemistry, the specimen was c-kit positive, CD34-positive, SMA-negative S100-negative, desmin-negative, CD31-negative, HMB45-negative, and calponin-negative. It was diagnosed as an uncommitted GIST at high risk for malignancy.