Giant GIST of the mesocolon: report of a case

Gastrointestinal stromal tumors are rare neoplasms arising from mesenchymal cells of the gastrointestinal tract, that strongly express a class III receptor tyrosine kinase, called KIT, due to some mutations in the KIT proto-oncogene. Two thirds of GISTs are found in the stomach, 20% to 50% in the small bowel (one third in the duodenum), and 5% to 15% in colon and rectum; GISTs, however, may rarely be found also in the oesophagus, omentum, mesentery or the retroperitoneum. Their treatment is strictly surgical, and only R0 resection can achieve good RESULTS: Treatment with Imatinib seems to be promising in case of unresectable or metastatic GIST, even if some trials are studying its effects after curative resection. GIST of the mesocolon are rare, and as in the other locations, require extensive surgery. The Autohrs report a case of giant malignant GIST arising from transverse mesocolon, treated by en-bloc resection of the tumor with a segment of transverse colon and great omentum.     

十二指肠间质瘤的临床分析

目的 探讨十二指肠间质瘤局部切除的手术安全性及疗效.方法 对12例直径＜4 cm,肿瘤位置距离十二指肠乳头1cm以上的十二指肠间质瘤采用局部切除,并回顾性分析患者的临床病理资料.结果 术后本组患者均无吻合口漏发生,切缘无肿瘤细胞残留.术后病理学检查均为低度危险间质瘤.随访9～38个月,无复发患者.结论 十二指肠间质瘤的局部切除创伤小、恢复快,该手术方式安全可靠.

Abstract：

Objective To evaluate the safety and effect of local resection of the duodenum for the treatment of gastrointestinal stromal tumors(GISTs). Methods In this study, 12 duodenal GIST cases in which tumors located more than 1.0 cm away from the major duodenal papilla, underwent simple local resection of the duodenum along with the tumors measuring at 4 cm in diameter or less. The clinical data were retrospectively analyzed. Results After simple local resection of the duodenum for GISTs, no stomal leak occurred and the margin was all negative. Pathologically tumors were all low risk grade. There was no recurrence during a follow-up of 9-38 months. Conclusions Local resection of the duodenum is safe and efficient in the treatment of duodenal GISTs should the tumors locat more than 1.0 cm away from the papilla.     

Large gangliocytic paraganglioma of the duodenum: A rare entity

Gangliocytic paragangliomas are rare tumors that almost exclusively occur within the second portion of the duodenum. Although these tumors generally have a benign clinical course, they have the potential to recur or metastasize to regional lymph nodes. The case report presented here describes a 57-year-old female patient with melena, progressive asthenia, anemia, and a mass in the second-third portion of the duodenum that was treated by local excision. The patient was diagnosed with a friable bleeding tumor. The histologic analysis showed that the tumor was a 4 cm gangliocytic paraganglioma without a malignant cell pattern. In the absence of local invasion or distant metastasis, endoscopic resection represents a feasible, curative therapy. Although endoscopic polypectomy is currently considered the treatment of choice, it is not recommended if the size of the tumor is > 3 cm and/or there is active or recent bleeding. Patients diagnosed with a gangliocytic paraganglioma should be closely followed-up for possible local recurrence.     

A gastrointestinal stromal tumor of the third portion of the duodenum treated by wedge resection: A case report

A 65-year old woman was admitted to our hospital with abdominal pain. Computed tomography showed a tumor measuring about 3 cm in diameter with no metastatic lesion or signs of local infiltration. Gastroduodenal endoscopy revealed the presence of a submucosal tumor in the third portion of the duodenum and biopsy revealed tumor cells stained positive for c-kit. These findings were consistent with gastrointestinal stromal tumors (GISTs) and we performed a wedge resection of the duodenum, sparing the pancreas. The postoperative course was uneventful and she was discharged on day 6. Surgical margins were negative. Histology revealed a GIST with a diameter of 3.2 cm and < 5 mitoses/50 high power fields, indicating a low risk of malignancy. Therefore, adjuvant therapy with imatinib was not initiated. Wedge resection with primary closure is a surgical procedure that can be used to treat low malignant potential neoplasms of the duodenum and avoid extensive surgery, with significant morbidity and possible mortality, such as pancreatoduodenectomy.     

Duodenal gastrointestinal stromal tumor: clinicopathological characteristics,surgical outcomes,long term survival and predictors for adverse outcomes

Background

Gastrointestinal stromal tumors (GISTs) occur rarely in the duodenum. Because of their low incidence, data on long-term survival and prognostic factors are limited. The aims of this study were to present the authors' experiences in the diagnosis and treatment of this disease and to evaluate long-term surgical outcomes.

Methods

Clinical data from 22 consecutive patients with duodenal GISTs surgically managed from May 1999 to August 2011 were retrospectively studied. A pooled analysis was done by systematically reviewing other case series reported in the English literature. Recurrence-free survival and independent predictors of adverse outcomes were analyzed using the Kaplan-Meier method and multivariate Cox regression.

Results

Duodenal GISTs had a mild male predominance (68.2%), occurring primarily in older adults (median age, 58 years), with a frequency of 7.49% among all GISTs. Clinical presentations were nonspecific, with gastrointestinal bleeding and abdominal pain or discomfort being the most common symptoms. The tumors were located mainly in the second portion of the duodenum, in 14 patients (63.6%), with a median size of 3.75 cm (range, 1.4 to 14). All patients underwent curative surgical resection, including 9 pancreaticoduodenectomy, 3 segmental duodenectomy, and 10 local resection. Eighteen patients were alive without evidence of recurrence after a median follow-up period of 67.5 months (range, 3 to 118). The 1-year, 2-year, and 3-year rates of recurrence-free survival were 95%, 89.5%, and 86.7%, respectively. Kaplan-Meier analysis and log-rank tests showed that surgical pattern, mitosis, and risk grade were significantly associated with recurrence-free survival (P < .05 for all). However, only high mitosis was a significant predictive factor for adverse outcomes on multivariate analysis (hazard ratio, 16.414; 95% confidence interval, 1.914 to 140.756; P = .011).

Conclusions

Duodenal GIST is an unusual neoplasm with favorable survival after curative resection. Mitotic activity was more influential than tumor size and risk grade in predicting adverse outcomes. All patients with duodenal GISTs require long-term follow-up, because late relapse can occur even if the tumor has low malignant potential.     

Surgical options in the treatment of GIST of the upper portion of the stomach. Report of two cases

Gastrointestinal stromal tumors are rare neoplasms arising from mesenchymal precursor cells of the gastrointestinal tract that may differentiate towards the interstitial cells of Cajal, pacemaker cells regulating autonomous motility of G.I. tract. Grading of GIST has been proven to be as difficult as their classification. Two thirds of GISTs are located in the stomach, 20-50% in the small bowel (one third in the duodenum), and 5-15% in colon and rectum; GISTs, however, may rarely be found also in the oesophagus, omentum, mesentery or the retroperitoneum. The distribuition of these tumors in the stomach is: pars media, 40%; antrum, 25%; pylorus, 20%; in less than 15%, GISTs location is next to the EGJ, in the cardia and in the iundus. The upper gastric third location of GISTs is not common, so their surgical management has been not yet well investigated. Total gastrectomy is considered the therapy of choice for the GIST located next to the EGJ, but wedge resection could be considered a surgical option in selected cases. The Authors describe 2 cases of GIST located just under the upper portion of the stomach and discuss about the different surgical options for GISTs of this region.     

十二指肠间质瘤18例的临床特征和外科治疗

目的探讨十二指肠间质瘤的临床特征、外科治疗方法及效果。方法回顾性分析复旦大学附属中山医院1995-2004年间收治的18例十二指肠间质瘤患者的临床资料。结果本组患者临床表现为黑便者7例,腹痛6例,饱胀5例,贫血3例。分别经上消化道钡餐造影、胃镜、内镜超声及CT诊断,肿瘤位于十二指肠降部13例,水平部2例,球部2例,升部1例;肿瘤直径2.0-9.0(平均4.8)cm。肿瘤恶性15例(83.3%),交界性2例(11.1%),良性1例(5.6%);梭形细胞型14例,混合型3例,上皮细胞型1例。9例十二指肠降部肿瘤者行胰十二指肠切除术,术后病理检查均无淋巴结转移;4例十二指肠降部和1例球部肿瘤者行肿瘤及十二指肠壁局部切除术;2例十二指肠水平部和1例升部肿瘤者行十二指肠节段切除术:另有1例十二指肠球部肿瘤者行胃远端大部切除术。15例术后获得17个月至8年的随访,1、3年生存率分别为100%和86.7%。结论十二指肠间质瘤恶性多见。手术治疗为主。手术方式的选择取决于肿瘤的位置和大小。     

Management and Outcome of Gastrointestinal Stromal Tumors of the Duodenum

Background

Duodenal gastrointestinal stromal tumors (GISTs) are uncommon and relatively small subset of GISTs whose optimal surgical procedure has not been well defined. We conducted this study to present the surgical experience in our institution and to analyze the postoperative outcome of duodenal GISTs.

Methods

A retrospective clinicopathologic analysis was performed for nine duodenal GIST patients who underwent surgery from May 2001 to April 2009. The median follow-up period was 22 months (range: 13–61 months).

Results

A total of nine patients (six males/three females) with a median age of 52 years (range: 45-73 years) were treated. The most common presentation was abdominal pain (45%), and the second portion of duodenum (45%) was most common dominant site. All of the patients underwent limited resection: there were seven wedge resections with primary closures (five open/two laparoscopic) and two segmental resections with end-to-end duodenojejunostomies. The median tumor size was 3.5 cm (range: 1.9-5.5 cm), and the mitotic count was less than fivemitoses/50 high power fields (HPF) in all cases. None patients had neoadjuvant or adjuvant therapy. All of the patients were alive and disease-free.

Conclusion

We obtained excellent disease-free survival following limited resection with clear margins. Limited resection should be considered a treatment option for duodenal GIST.     

Two case of bilateral approach in laparoscopic pancreas-sparing distal duodenectomy for duodenal neoplasms arising from the distal duodenum

IntroductionLaparoscopic pancreas-sparing distal duodenectomy is a less invasive surgical therapy; however, the anatomical complexity of the duodenum increases the difficulty of laparoscopic procedures. We introduce our technique for laparoscopic pancreas-sparing distal duodenectomy for distal duodenal tumors.Presentation of casesA first patient was 47-year-old woman who had 30 mm of duodenal tumor which located in third portion of duodenum. A second patient was 66-year-old man who had 35 mm of submucosal tumor which located in the third portion of duodenum. Laparoscopic pancreas-sparing duodenectomy was performed using bilateral approach for both cases. We began by dissecting an avascular area on the right side of the transverse mesocolon to mobilize the second and third portions of the duodenum with the uncinate process of the pancreas. Next, from the left side, the jejunum and the fourth portion of the duodenum were fully mobilized orally from the surrounding tissue, connecting the dissection plane with the right-side area. The jejunum and duodenum were cut with a linear stapler. Intracorporeal reconstruction was performed in an overlapped manner. We performed this procedure in two patients. Operative time was 326 and 370 min, respectively. Patients were discharged on postoperative days 9–12 without postoperative complications.DiscussionDuodenal tumors are found increasingly often because of developments in endoscopic technology and techniques; therefore, establishing safe surgical procedures for duodenal tumor excision is imperative. Our surgical approach was simple and safe procedure.ConclusionLaparoscopic pancreas-sparing distal duodenectomy with a bilateral approach is a useful approach without wide mobilization of duodenum.     

Multiple gastrointestinal stromal tumors in neurofibromatosis type 1: Report of a case

This report presents a case of multiple gastrointestinal stromal tumors (GIST) with neurofibromatosis type 1 (NF1). A 68-year-old woman was admitted to the hospital because of a tumor close to the head of the pancreas. Imaging studies revealed submucosal tumors of the duodenum. The retroperitoneal tumor was diagnosed before surgery. Besides the main tumor in the duodenum, multiple small submucosal tumors were found in the duodenum and upper part of the jejunum during the operation. All of these tumors were resected. The histological diagnosis of all these tumors was GISTs. These tumors were immunohistochemically positive for KIT, but they demonstrated no mutation in c-kit exons 9, 11, 13, and 17, and platelet-derived growth factor receptor α exons 12 and 18. No recurrence occurred for a year after surgery.     

Diffuse Large B-cell Lymphoma in the Ampulla of Vater Causing Obstructive Jaundice: Report of a Case

We report a case of diffuse large B-cell lymphoma (DLBCL) in the ampulla of Vater, causing painless obstructive jaundice in a 78-year-old woman. Duodenal endoscopy revealed a mass in the ampulla of Vater and narrowing of the second portion of the duodenum, although diagnosing DLBCL from an endoscopic biopsy was impossible because there were several kinds of leukocytes in the infiltrate. We performed pylorus-preserving pancreatoduodenectomy to establish a histological diagnosis, relieve the obstructive jaundice, and remove the narrowed second portion of the duodenum. Histological and immunohistochemical examination of the surgically resected specimen confirmed a diagnosis of DLBCL. Chemotherapy is the mainstay of treatment for DLBCL; however, surgery still plays an important role when the histological diagnosis cannot be established preoperatively and when complications are not amenable to nonsurgical therapy.     

Duodenal stenosis caused by cystic dystrophy in heterotopic pancreas: Report of a case

We herein describe the first reported case of duodenal stenosis caused by cystic dystrophy in heterotopic pancreas (CDHP) in Asia. A 63-year-old man was admitted to the hospital presenting with nausea and vomiting of 2 days’ duration. Laboratory examinations showed an elevation in both the serum amylase level (275 IU/l) and white blood cell count (13 600/μl). A 3-cm-diameter tumor close against the duodenum was pointed out from the results of computed tomography (CT) and magnetic resonance cholangiopancreatography (MRCP); the tumor contained a cystic and solid component. Endoscopic examinations and an upper gastrointestinal series showed stenosis of the second portion of the duodenum without any mucosal change. The tumor was considered to be located at the submucosal layer of the second duodenum. The biopsy specimen of the duodenum revealed no malignancy. We strongly doubted the presence of a malignant submucosal tumor in the duodenum based on the findings of diagnostic imaging, and a pancreaticoduodenectomy was thus performed. Histopathologically, the lesion was diagnosed to be CDHP. The postoperative course was uneventful. The patient was symptomatic but was free of any symptoms after surgery. He continues to be regularly followed up on an outpatient basis and has had no recurrence of symptoms. This case demonstrates the need to consider CDHP in the differential diagnosis as a rare cause of duodenal stenosis.     

Solitary malignant schwannoma of the duodenum with metastasis to the liver: Report of a case

Solitary malignant schwannoma of the duodenum is extremely rare. In fact, only two previous reports on successful radical resection of a solitary malignant schwannoma of the duodenum have been documented in the Japanese literature, and there are none in the English literature. We report herein the unique case of a 64-year-old man with a malignant schwannoma of the duodenum in whom both the primary lesion and a metachronous metastatic lesion were successfully resected. The patient initially underwent a Whipple procedure for a hypervascular tumor in the head of the pancreas. The tumor was composed of palisading spindle cells and scattered S-100 protein-positive cells with prominent mitotic figures, and was diagnosed as a malignant schwannoma of the duodenum. A second admission, 3 years later, revealed the presence of a solitary hypervascular hepatic tumor in the anterior segment of the right lobe of the liver. The hepatic tumor was resected and confirmed, microscopically and immunohistochemically, as a metastasis from the malignant schwannoma of the duodenum. The patient is alive 3 years and 6 months after his initial operation. To our knowledge no other report on the successful radical resection of both primary and metastatic lesions has been documented. This case report therefore highlights the importance of performing follow-up examinations focusing on the liver, and encourages an aggressive surgical attitude for the treatment of this unusual disease.     

Lymphangioma of the duodenum: Report of a case

A case of lymphangioma of the duodenum with a successful preoperative diagnosis is reported herein. A 76-year-old man who complained of tarry stool was found to have a hemorrhagic polypoid tumor in the third portion of the duodenum. The tumor was diagnosed histologically as cavernous lymphangioma by endoscopic biopsy. Since such a tumor is essentially benign, a partial resection of the duodenum including the tumor was performed. Therefore, an endoscopic biopsy seems to be valuable in the diagnosis of duodenal lymphangioma.     

原发性十二指肠恶性肿瘤的诊断和治疗：附54例报告

目的：探讨改善原发性十二指肠恶性肿瘤的诊断和治疗方法。方法：回顾性分析6年间收治原发性十二指肠恶性肿瘤54例的临床资料。结果：肿瘤位于球部6例（11.1%）、降部44例（81.5%），其中乳头部38例（占降部的86.4%，总数的70.4%），水平部2例（3.7%），升部2例（3.7%）。腺癌50例（92.6%）。主要症状有黄疸、上腹部痛、体重下降伴纳差、腹胀、消化道梗阻等。上腹部压痛72.0%，无阳性体征20.0%，腹块8.0%。术前合并胆囊病变率37.0%。术前经十二指肠镜、ERCP确诊率为94.4%及77.8%。术前确诊为原发性十二指肠癌15例（27.8%）,胰十二指肠切除38例，节段性十二指肠切除1例，姑息性手术9例，失去手术时机6例。手术切除率88.9%，根治性切除率72.2%。行胰十二指肠切除3年和5年生存率分别为40.6%和21.9%;姑息性手术3~24个月死亡，未行手术者半年内死亡。结论：十二指肠恶性肿瘤以降部乳头最多见，以腺癌为主，进展期症状复杂，腹部体症特异性表现低，易合并胆囊病变，术前确诊率低。十二指肠镜和ERCP是诊断的首选方法。胰十二指肠切除可延长生存期。     

Mucinous Carcinoma of the Duodenum Associated with Hereditary Nonpolyposis Colorectal Cancer: Report of a Case

We herein report a rare case of primary mucinous carcinoma of the duodenum associated with hereditary nonpolyposis colorectal cancer (HNPCC). A 50-year-old man known to have HNPCC based on the Amsterdam criteria I was admitted because of the presence of a duodenal tumor. Duodenoscopy revealed an ulcerated tumor in the posterior wall of the second portion of the duodenum and the malignancy was confirmed by a biopsy. He underwent a pylorus-preserving pancreaticoduodenectomy with a regional lymph node dissection. The histological diagnosis was mucinous carcinoma of the duodenum with lymph node metastasis. High-frequency microsatellite instability (MSI-H) was identified in both the colon and a duodenal specimen based on a microsatellite assay. A germline mutation in the hMSH2 gene was also identified. Even though extracolonic malignancies are associated with HNPCC, duodenal cancer is nevertheless very rare, and only two cases have been reported over the past 20 years. The present case is therefore only the third such case and the patient is herein described with a brief review of the literature.     

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目的 探讨胃肠道间质瘤的临床表现、诊断、治疗和预后。方法 回顾性分析1999年4月至2002年7月收治的15例胃肠道间质瘤的临床和病理资料。结果 主要临床表现为腹部不适(7／15)、消化道出血(6／15)。病变部位为胃13例，十二指肠l例，小肠1例。14例行手术治疗。15例镜下均见梭形细胞，其中良性9例，交界性4例，恶性l例，胃癌伴良性间质瘤l例。免疫组化CDll7( )73％，CD34( )73％，SMA(-)73％，S—100(-)67％。随访率100％，病人均存活。结论 内镜和影像学检查是发现胃肠道间质瘤病变的主要手段，术前确诊仍较困难，手术切除是病理确诊和治疗的主要方法。     

A very rare case of duodenal hemolymphangioma presenting with iron deficiency anemia

INTRODUCTION

Intraabdominal lymphangiomas account for less than 5% of all lymphangiomas and small intestinal hemolymphangioma is a very rare benign tumor.

PRESENTATION OF CASE

Here we describe the first case of primary ulcerated duodenal hemolymphangioma in a 24-year-old woman, causing occult bleeding from gastrointestinal tract. She presented with an unexplained refractory iron-deficiency anemia and gastroduodenoscopy revealed an ulcerated and polypoid lesion of the second portion of the duodenum. Partial resection of the duodenum was thus performed and the final pathological diagnosis was hemolymphangioma.

DISCUSSION

There were only two reports, one of a hemolymphangioma of the pancreas invading to the duodenum and another of a small intestinal hemolymphangioma, presenting with gastrointestinal bleeding until May 2012.

CONCLUSION

The aim of this case report is to highlight the difficulty in making an accurate preoperative diagnosis and describe the surgical management of an unusual location for a very rare tumor. To arrive at a definitive diagnosis and exclude malignancy, partial resection of the duodenum was considered to be the required treatment.     

胃肠道间质瘤的诊治：附100例报告

目的:探讨胃肠道间质瘤的临床病理诊断与治疗方法.方法:回顾性分析100例胃肠道间质瘤的临床病理资料.结果:CT平扫探查发现所有患者肿块均为单发,79例良性肿块可见软组织密度肿块,密度均匀;21例恶性肿块内可见大小不等的低密度坏死区,呈分叶状.双期增强扫描见肿块腔内侧大小不一的溃疡,其中7例恶性间质瘤的溃疡程度大而深;增强扫描肿块呈不均匀明显强化,囊变坏死区无强化.病变发生在胃部56例,十二指肠26例,小肠10例,结肠及直肠6例,肠系膜2例.术中标本的病理学检测发现由梭形瘤细胞形成的细胞团巢是胃肠道间质瘤的特征排列.所有病例均完整切除,均未发现淋巴结转移,术中及术后均无死亡,平均住院时间15.2 d.随访期6～24个月,其中5例恶性肿瘤患者死亡,均死于肝转移;28例复发(28.0％),其中肿瘤类型:交界性肿瘤12例,恶性肿瘤16例;复发部位:局部复发16例,腹膜复发12例;再次进行切除手术后随访3个月,均存活.结论:胃肠道间质瘤可根据其临床表现、CT等影像学检查以及病理学检测等明确诊断,早行手术切除效果良好.     

Double common bile duct: a case report and a review of the Japanese literature

BACKGROUND: A double common bile duct (DCBD) is a rare congenital anomaly. We report the case of a 60-year-old Japanese female, whose common bile duct divided into 2 channels and both channels opened individually into the second portion of the duodenum. This is the fourth reported case of DCBD with a choledochal cyst and pancreaticobiliary maljunction (PBM). METHODS: A review of the literature revealed that DCBD is more frequently diagnosed in Oriental people. We reviewed 47 cases of DCBD reported in the Japanese literature. RESULTS: Among these, cholelithiasis was found in 27.7%, a choledochal cyst in 10.6%, PBM in 29.8%, and cancers in 25.5%. Cancer and PBM were the 2 most serious concomitant conditions. The incidence and type of complicating cancer and PBM varied according to the site of the opening of the accessory common bile duct (ACBD). Concomitant gastric cancer was frequently noted when the ACBD opened into the stomach, whereas cancer of the biliary system was common when the ACBD opened into the second portion of the duodenum or the pancreatic duct. PBM was observed only in those patients in whom the ACBD opened into the second portion of the duodenum or the pancreatic duct. Therefore the treatment and prognosis of DCBD is influenced by the site of opening of the ACBD. CONCLUSIONS: In DCBD, the opening site of the ACBD was considered to have close implications for the type of concomitant cancer and concomitant PBM that would appear.