BACKGROUND Duodenal gastrointestinal stromal tumor(DGIST)is a rare tumor with a specific anatomic site and biological characteristics.As the incidence of lymph node metastasis is very low,the main treatment method is ...BACKGROUND Duodenal gastrointestinal stromal tumor(DGIST)is a rare tumor with a specific anatomic site and biological characteristics.As the incidence of lymph node metastasis is very low,the main treatment method is surgery.Two main surgical techniques(local resection and Whipple)are performed in patients with DGISTs.The critical question is which surgical technique to choose.AIM To identify factors influencing the choice of surgery for DGISTs.METHODS The clinicopathological data of patients with DGISTs who underwent surgery between January 1999 and January 2021 were analyzed.We used the Student’s ttest or Mann-Whitney U-test and theχ2 test or Fisher’s exact test to determine the differences between the two groups of patients.Furthermore,we used logistic analysis to identify the relevant factors and independent factors related to the type of surgery.The Kaplan-Meier method was used to analyze the patient’s survival information and Cox regression analysis was performed to determine prognostic risk factors.RESULTS Overall,86 patients were analyzed,including 43 men(50%)and 43 women(50%).We divided the patients into two groups based on surgical technique(local resection or Whipple surgery).There were no differences in the age,mitotic figures,and complications between the two groups;however,the tumor size,tumor location,risk grade,postoperative hospital stay,and abdominal drainage time were significantly different.Based on univariate logistic analysis,the Whipple procedure was chosen if the tumor size was≥5.0 cm,the tumor was located in the descending part of the duodenum,or the risk grade was medium or high.In our research,the five-year overall survival rate of patients was more than 90%.We also describe two DGIST patients with liver metastases at first diagnosis and analyzed their management in order to provide advice on complicated cases.CONCLUSION The Whipple procedure was performed if the primary tumor was in the descending part of the duodenum,tumor size was≥5.0 cm,or the tumor risk grade was medium or high.展开更多
BACKGROUND Endoscopic resection for duodenal gastrointestinal stromal tumors(GISTs)is still considered a great challenge with a high risk of complications,including perforation,bleeding,tumor rupture,and residual tumo...BACKGROUND Endoscopic resection for duodenal gastrointestinal stromal tumors(GISTs)is still considered a great challenge with a high risk of complications,including perforation,bleeding,tumor rupture,and residual tumor.AIM To assess the effectiveness and safety of endoscopic resection for duodenal GISTs.METHODS Between January 2010 and January 2022,11 patients with duodenal GISTs were treated with endoscopic resection.Data were extracted for the incidence of complete resection,bleeding,perforation,postoperative infection,recurrence,and distant metastasis.RESULTS The incidence of successful complete resection of duodenal GISTs was 100%.Three cases(27.3%)had suspected positive margins,and the other 8 cases(72.7%)had negative vertical and horizontal margins.Perforation occurred in all 11 patients.The success rate of perforation closure was 100%,while 1 patient(9.1%)had suspected delayed perforation.All bleeding during the procedure was managed by endoscopic methods.One case(9.1%)had delayed bleeding.Postoperative infection occurred in 6 patients(54.5%),including 1 who developed septic shock and 1 who developed a right iliac fossa abscess.All 11 patients recovered and were discharged.The mean hospital stay was 15.3 d.During the follow-up period(14-80 mo),duodenal stenosis occurred in 1 case(9.1%),and no local recurrence or distant metastasis were detected.CONCLUSION Endoscopic resection for duodenal GISTs appears to be an effective and safe minimally invasive treatment when performed by an experienced endoscopist.展开更多
BACKGROUND: Duodenal gastrointestinal stromal tumors, which are rare, comprise 3%-5% of all gastrointestinal stromai tumors. We present a case of a metastatic duodenal gastrointestinal stromal tumor that was successfu...BACKGROUND: Duodenal gastrointestinal stromal tumors, which are rare, comprise 3%-5% of all gastrointestinal stromai tumors. We present a case of a metastatic duodenal gastrointestinal stromal tumor that was successfully treated by simultaneous tight hemihepatectomy and pancreaticoduodenectomy. METHODS: A 50-year-old woman was admitted to our department for the treatment of a possible metastatic duodenal gastrointestinal stromal tumor (GIST). At laparotomy a large duodenal tumor was found displacing the head of the pancreas. A 3 cm in diameter lesion in the posterior aspect of segment Ⅷ of the liver was also noted. Simultaneous right hepatectomy and pancreaticoduodenectomy were performed. RESULTS: Histological examination revealed a high grade metastatic duodenal GIST strongly positive for c-kit, CD34, and vimentin. The patient had no additional therapy. A follow-up of 21 months showed that the patient is very well and there is no evidence of recurrent diseases. CONCLUSIONS: Malignant stromai tumors of the duodenum are rarely encountered. They are usually slow growing, and may be amenable to curative surgery, even after occurrence of metastases. Resection of localized liver metastasis is still advocated when feasible, since imatinib does not provide a complete or long-term response. Combined surgical resection is an efficacious treatment for patients with metastatic duodenal gastrointestinal stromal tumor.展开更多
BACKGROUND Liver metastasis of duodenal gastrointestinal stromal tumor(GIST)is rare.Most reports mainly focus on its treatment and approaches to surgical resection,while details on its contrast-enhanced ultrasound(CEU...BACKGROUND Liver metastasis of duodenal gastrointestinal stromal tumor(GIST)is rare.Most reports mainly focus on its treatment and approaches to surgical resection,while details on its contrast-enhanced ultrasound(CEUS)findings are lacking.The diagnosis and imaging modalities for this condition remain challenging.CASE SUMMARY A 53-year-old Chinese man presented with mild signs and symptoms of the digestive tract.He underwent routine examinations after GIST surgery.Magnetic resonance imaging showed a 2.3 cm hepatic space-occupying lesion.All the laboratory test results were within normal limits.For further diagnostic confirmation,we conducted high frame rate CEUS(H-CEUS)and found a malignant perfusion pattern.Heterogeneous concentric hyper-enhancement,earlier wash-in than the liver parenchyma,and two irregular vessel columns could be observed at the periphery of the lesion during the arterial phase.Ultrasound-guided puncture biopsy was used to confirm the diagnosis of the lesion as liver metastasis of duodenal GIST.Imatinib was prescribed after biopsy,and the patient’s clinical course was monitored.CONCLUSION H-CEUS is useful for detecting microcirculation differences,wash-in patterns,and vascular morphogenesis and diagnosing liver metastasis of duodenal GIST.展开更多
Introduction: The coexistence of synchronic duodenal gastrointestinal stromal tumor (GIST) and neuroendocrine tumor in a patient with neurofibromatosis type 1 (NF1) is extremely rare, and only eight cases were describ...Introduction: The coexistence of synchronic duodenal gastrointestinal stromal tumor (GIST) and neuroendocrine tumor in a patient with neurofibromatosis type 1 (NF1) is extremely rare, and only eight cases were described in the literature. Clinical Case: This is a rare case of a 38-year-old female patient with NF1 who developed synchronic GIST and neuroendocrine tumor, which were both in the second portion of the duodenum. The first symptoms were abrupt digestive bleeding and anemia. Upper digestive endoscopy revealed two tumors, sizes 2.5 and 3.0 cm, in the second portion of duodenum, with biopsies identifying a GIST and a neuroendocrine tumor. Therapeutic decision was to proceed to surgical resection, and Whipple’s procedure was indicated. Surgical procedure was performed with good outcome. Currently the patient has excellent quality of life and maintains follow up for thirty months without recurrence. Discussion: Long-term disease-free survival and excellent quality of life are reported when these tumors are fully resected in this context. However, it is not always easy to access the gastrointestinal tract, especially the small intestine, and proceed to the histopathologic diagnosis of these tumors. Conclusion: It is important to be aware of the possibility of the coexistence of various tumors in the NF1 scenario for adequate screening, staging, and surgical treatment of these patients, as good prognosis can be achieved when such tumors are detected and treated properly.展开更多
Introduction: This study investigated the incidence, surgical management and outcome of Gastrointestinal Stromal Tumors (GIST) in Upper Egypt. Methods: A retrospective review of all GIST patients admitted a South Egyp...Introduction: This study investigated the incidence, surgical management and outcome of Gastrointestinal Stromal Tumors (GIST) in Upper Egypt. Methods: A retrospective review of all GIST patients admitted a South Egypt Cancer Institute between Jan. 2010 and Dec. 2015 was conducted. Patients’ demographics, clinical presentation, tumor characteristics, radiological, pathological and immunohistochemical findings, surgical procedures, recurrence and mortality were recorded. Results: A total of 36 GIST patients were identified, stomach was the most common site (27.8%) followed by the small intestine (19.4%) and the large intestine (16.7%). The mean age at time of diagnosis as 52.8 ± 14.4 (ranged from 17 to 76 years). Of these 36 cases, 20 (55.6%) cases were males and 16 (44.4%) cases were females with a ratio of 1.2:1. About 22 cases (61.1%) presented with primary tumors, eight cases (22.2%) had primary tumors and metastases, three cases (8.35) presented with recurrent mass, whereas one case (2.2%) presented either with recurrent mass and metastases or metastases only. The majority of cases (22) had tumorsize >5 cm. Patients were stratified as high, intermediate, low and very low risk (50.6%, 30.6%, 11.1% and 2.8%, respectively). Almost all the cases were surgically managed and 75% were completely resectable. During follow up (average 26.5 months), 22 patients showed complete recovery, 7 had recurrent or metastatic disease and 2 died due to liver metastasis. Conclusion: The incidence of GIST in Upper Egypt is apparently low. Surgical resection is the preferred choice of treatment. The demographic data of GIST patients in South Egypt Cancer institute were similar to those published in the literature. Other prospective studies are required to assess the prognosis and the effect of treatment.展开更多
Introduction: Colorectal gastrointestinal stromal tumors (GISTs) mesenchymal tumor is very uncommon. GISTs effect mostly on the stomach and small intestine and rarely occur in the colon, rectum and esophagus, that ori...Introduction: Colorectal gastrointestinal stromal tumors (GISTs) mesenchymal tumor is very uncommon. GISTs effect mostly on the stomach and small intestine and rarely occur in the colon, rectum and esophagus, that originating from precursors of the interstitial cells that originate of Cajal. The symptoms of gastrointestinal stromal tumor depend on the site and size of the tumor, and may include abdominal pain, gastrointestinal bleeding or signs of obstruction;small tumors may, however, be asymptomatic. Some of the patients with gastrointestinal stromal tumor have bloody stools, obstruction and abdominal pain as the commonest manifestation. Immunocytochemical staining for CD117 is helpful in confirming the diagnosis. Case presentation: We report 3 new cases of GISTs: two occurred at the rectal and the other at descending Colon. Two cases are over 50 years of age and, and all cases the chief complain of bowel obstruction, abdominal pain in two cases, and one case with anemia and urine retention. All the patients were operated and were permormed pathology examinatiom. All case ware positive result for immunocytochemical staining CD117. All cases we had presented had size more than 5 cm are considered as unfavorable prognostic factors to Fletcher criteria, all patients scheduled for chemotherapy with Glivec but just one patient continued to used Glivec. Post surgery follows up one patient post milles with urinary incontinence complaints found and that patients are trained to CIC (intermittent catheterization). Conclusion: Colorectal gastrointestinal stromal tumors are very rare and can present as mass abdomen. Resection and chemotherapy are the treatment of choice.展开更多
Gastrointestinal stromal tumor (GIST) is the most common mesenchymal malignancy of the gastrointestinal tract.GISTs may coexist with different types of cancer,either synchronous or metachronous (1).Most GISTs deve...Gastrointestinal stromal tumor (GIST) is the most common mesenchymal malignancy of the gastrointestinal tract.GISTs may coexist with different types of cancer,either synchronous or metachronous (1).Most GISTs develop in a sporadic fashion,but familial occurrence,such as neurofibromatosis and Carney-triad,has also been reported (2).The overall frequency of second tumors in different series varied from 4.5% to 33%.The most frequent types of GIST-associated cancers were gastrointestinal carcinomas (47%),lymphoma/leukemia (7%),carcinomas of prostate (9%),breast (7%),kidney (6%),lung (5%),female genital tract (5%),carcinoid tumors (3%),soft tissue and bone sarcomas (3%),malignant melanoma (2%) and seminoma (1%) (1,3-5).展开更多
Epithelioid gastrointesinal tumors (GISTs) are less likely to have c-kit gene mutations (and express CD117) than spindle cell GISTs. CD117 negative/c-kit negative GISTs can have platelet-derived growth factor alpha (P...Epithelioid gastrointesinal tumors (GISTs) are less likely to have c-kit gene mutations (and express CD117) than spindle cell GISTs. CD117 negative/c-kit negative GISTs can have platelet-derived growth factor alpha (PDGFRα) gene mutations, overexpress PDGFRα protein and respond to imatinib mesylate. Many cases of CD117-negative/CD117-weakly positive, c-kit mutation negative and PDGFRα mutation positive myxoid epithelioid GISTs and one case of CD117-positive GIST with chondro-myxoid features mimicking chondrosarcoma have been reported. We report a case of myxoid epithelioid GIST with predominance of chondroid and chordoid areas resembling an extraskeletal myxoid chondrosarcoma that was strongly positive for CD117, PDGFRα and DOG1 (Discovered on GIST 1) by immunohistochemistry, but lacked c-kit and PDGFRα gene mutations. It is possible that CD117 is strongly positive if a myxoid epithelioid GIST has chondroid/chordoid appearance, but a larger study is needed to confirm this association. CD117 expression in GISTs is important, because GISTs showing CD117 positivity respond to imatinib. No comment can be made about the prognostic significance of chondroid/chordoid appearance in the GISTs.展开更多
Introduction: Majority of mesenchymal tumors of gastrointestinal tract are Gastrointestinal Stromal Tumor (GIST). It is, however, a rare tumor, accounting for less than 1% of primary gastrointestinal (GI) neoplasms. T...Introduction: Majority of mesenchymal tumors of gastrointestinal tract are Gastrointestinal Stromal Tumor (GIST). It is, however, a rare tumor, accounting for less than 1% of primary gastrointestinal (GI) neoplasms. Though, these tumors are refractory to conventional chemotherapy or radiotherapy but show a good response to targeted adjuvant chemotherapy with tyrosine kinase inhibitors following surgical resection. Case Report: we report here a case of primary Extra-GIST tumor arising from mesentry of small bowel near duodeno-jejunal junction in a 69 years old male patient. The patient presented with a palpable mass in upper abdomen for past 15 days. On examination, a non-tender mobile lump of size around 17 × 10 cm, with bosselated surface and firm in consistency was palpable involving epigastric, left hypochondrium and umbilical region. Contrast enhanced computed tomography of abdomen revealed a heterogenous mesentric mass. On surgical intervention a mass was found involving mesentery near dudenojejunal junction without involvement of gastrointestinal tract. Complete surgical resection of the tumor was done and adjuvant chemotherapy with Imatinib mesylate was started as HPE revealing GIST with mitotic index of >10/50 HPF and 17 × 10 cm size placed the patient in high risk category. Patient was discharged on 12th of post-operative day with advice of regular follow-up. Conclusion: GIST occurrence is not restricted to bowel but can involve unusual sites also. The mainstay of treatment remains surgical resection with adequate margin. In cases where tumour has malignant potential (high mitotic figures on histopathology) adjuvent treatment with tyrosine kinase may prevent or delay relapse.展开更多
BACKGROUND Gastrointestinal stromal tumors(GISTs)are mesenchymal tissue tumors originating from Cajal cells,presenting diverse clinical manifestations due to the different sizes,locations,and growth patterns of the le...BACKGROUND Gastrointestinal stromal tumors(GISTs)are mesenchymal tissue tumors originating from Cajal cells,presenting diverse clinical manifestations due to the different sizes,locations,and growth patterns of the lesions.Duodenum is an uncommon site of GISTs,more with gastrointestinal obstruction and bleeding as the first symptoms.Ectopic duodenal varix,as a rare varix occurring outside the gastroesophageal region,is the main type of heterotopic varices and an unusual cause of gas-trointestinal hemorrhage.The etiology is mainly seen in liver cirrhosis,portal hypertension,vasculitis,portal vein embolism and obstruction caused by various factors.Reports of duodenal stromal tumor combined with ectopic variceal hemorrhage are rarely seen;however,when it occurs,the situation can sometimes be urgent and life-threatening,especially when traditional endoscopy and imaging fail to detect the lesion timely.CASE SUMMARY We report a 52-year-old female patient who had no obvious inducement to develop black stool.Gastroscopy in a local hospital revealed that the duodenal horizontal ectopic varices were ruptured and bleeding.After metal clamping hemostasis,she still had gastrointestinal bleeding and was transferred to our hospital.Gastroscopy showed that active bleeding was still seen in the horizontal part of duodenum,and suspicious submucosal eminence was seen in the bleeding part.Abdominal computed tomography showed a huge stromal tumor of duodenum,specimens were pathologically confirmed after surgery.After a 3-mo follow-up,no gastrointestinal hemorrhage and complications occurred.CONCLUSION Ectopic variceal hemorrhage is rare but sometimes fatal.It may be combined with stromal tumor,which can be diagnosed by multiple methods.Endoscopic and surgical treatment are effective.展开更多
Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor and has a malignant potential. The clinical presentation with pneumoperitoneum and peritonitis is extremely rare. We report a case of a 40-yea...Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor and has a malignant potential. The clinical presentation with pneumoperitoneum and peritonitis is extremely rare. We report a case of a 40-year-old male presented with symptoms of acute abdomen. Radiological work-up confirmed pneumoperitoneum. Emergency laparatomy and complete resection were performed. The final diagnosis revealed perforated GIST originating from the jejunum. If an abdominal mass presents with pneumoperitoneum and peritonitis, jejunal GIST should be considered in diagnosis. A complete radical resection followed by postoperative adjuvant chemotheraphy with Imatinib is recommended.展开更多
Gastrointestinal stromal tumors (GISTs) account for approximately 2% of gastric cancers and can be challenging to diagnose due to the difficulty sampling tissue from these lesions. Current biopsy methods are inadequat...Gastrointestinal stromal tumors (GISTs) account for approximately 2% of gastric cancers and can be challenging to diagnose due to the difficulty sampling tissue from these lesions. Current biopsy methods are inadequate for determining the mitotic index, a major prognostic factor of these tumors. We sought to evaluate the ability of a prototype submucosal biopsy forceps to make a histologic diagnosis and mitotic index determination in cases of resected gastric GISTs. After obtaining informed consent and surgical resection of three gastric GISTs, an investigational submucosal biopsy forceps and sheath was passed under direct visualization into the central portion of the tumors (in the ex vivo setting) and biopsies were obtained. The tumor was then processed for standard histology. A gastrointestinal pathologist, blinded to the mitotic index of the full specimen, evaluated the research biopsies. The results from the submucosal forceps biopsies were then compared with the standard histology results. n all 3 cases, the submucosal forceps biopsies confirmed the diagnosis of a GIST. In two out of three cases, the submucosal forceps biopsies accurately classified the risk for progressive disease (based on the mitotic index) when compared to standard histology. In one case, the research biopsy mitotic index would have resulted in an underestimation of tumor risk (biopsies categorized the lesion as “very low” rather than “moderate” risk).展开更多
Objective:The aim of the study was to review the clinical records of 122 patients with gastrointestinal stromal tumors(GISTs) and analyze their clinicopathologic and immunohistochemical characteristics.Methods:The med...Objective:The aim of the study was to review the clinical records of 122 patients with gastrointestinal stromal tumors(GISTs) and analyze their clinicopathologic and immunohistochemical characteristics.Methods:The medic records of 122 patients with GISTs during the periods from January 2002 to May 2010 were reviewed.All tumors were confirmed by histological and immunohistochemical analyses.Results:The tumors occurred in 59 males and 63 females,ranging from 25 to 77 years.Of all cases,46 cases originated from stomach,42 from small intestine,17 from colon and rectum and 9 from retroperitoneal cavity and 4 cases from extra-gastrointestinal site.Liver was the most common organ that tumors metastases involved.Immunohistochemically,there were 114 tumors being positive for CD117 while 8 tumors negative for it.The frequencies of CD34 positive were higher in the stomach and rectum(89.1% and 86.7% respectively) than in the small intestine(64.3%,P < 0.05).Higher expression of SMA was in the tumors located in small intestine(54.8%) while the expressions of SMA in the gastric and rectal tumors were relatively low(21.7% and 20.0% respectively,P < 0.05).Conclusion:Gastrointestinal stromal tumors can occur in the gastrointestinal tract as well as in the extra-gastrointestinal sites.The frequencies of CD34 and SMA expression vary significantly with different locations.展开更多
The coronavirus disease 2019(COVID-19)pandemic profoundly affected the management and treatment of patients with malignancies.Based on the progress reported in the literature,we reviewed the recommendations for treatm...The coronavirus disease 2019(COVID-19)pandemic profoundly affected the management and treatment of patients with malignancies.Based on the progress reported in the literature,we reviewed the recommendations for treatment and vaccination in patients with gastrointestinal stromal tumor(GIST)during COVID-19.We focus on whether there is a risk and what could be the possible effects of vaccinating patients with GIST/cancer.Since the situation is quickly changing,and the health services have been severely disrupted,the diagnosis,treatment and recommendations for vaccination of these patients against COVID-19 are still not updated.The approval of vaccines in the pandemic gave hope that we would soon be able to return to a more normal life.However,the oncology community needs to adapt and provide the most effective treatment and care models for patients with rare cancer,such as GIST.Collecting data on the impact of vaccination in patients with GIST/cancer also will be beneficial in expanding knowledge about the future planning of treatment strategies and optimizing care in the event of a subsequent pandemic.展开更多
AIM: To evaluate the results of segmental duodenectomy (SD) and pancreaticoduodenectomy (PD) for duodenal gastrointestinal stromal tumor (GIST) and help clinicians with surgical management. METHODS: All patients who u...AIM: To evaluate the results of segmental duodenectomy (SD) and pancreaticoduodenectomy (PD) for duodenal gastrointestinal stromal tumor (GIST) and help clinicians with surgical management. METHODS: All patients who underwent surgery for non-metastatic GIST of the duodenum in a single institution since 2000 were prospectively followed up. Seven patients (median age 51 years, range: 41-73 years) were enrolled: five underwent SD and two underwent PD. RESULTS: All the patients had a complete resection (R0), with no postoperative morbidity and mortality. Among the SD group, GIST was classified as low risk in two patients, intermediate risk in two, and high risk in one, according to the Fletcher scale, (vs two high risk patients in the PD group). With a median followup of 41 (18-85) mo, disease-free survival (DFS) rateswere 100% after SD and 0% after PD (P < 0.05). The median DFS was 13 mo in the PD group. CONCLUSION: Whenever associated with clear surgical margins, SD is a reliable and curative option for most duodenal GISTs, and is compatible with longterm DFS.展开更多
Gastrointestinal stromal tumors (GISTs) occur most frequently in the stomach.Diagnosis of gastric GIST is not always clear before surgery.Flexible endoscopy may suggest the nature of the lesion (a bulky tumor with ...Gastrointestinal stromal tumors (GISTs) occur most frequently in the stomach.Diagnosis of gastric GIST is not always clear before surgery.Flexible endoscopy may suggest the nature of the lesion (a bulky tumor with preserved mucosa); however,biopsy is rarely diagnostic.Therefore,diagnostic medication with safe drugs may provide a feasible way under such conditions after an informed consent is obtained.Based on the excellent efficacy of imatinib mesylate (IM) in the treatment of GIST,we successfully applied it in the diagnostic medication of two patients with clinically suspected gastric stromal tumors.In conclusion,the diagnostic medication with IM can be an alternative option for patients with suspected GIST that can not be confirmed pathologically.展开更多
AIM: To determine the safety and efficacy of endoscopic duodenal stent placement in patients with malignant gastric outlet obstruction.METHODS: This prospective, observational, multicenter study included 39 consecutiv...AIM: To determine the safety and efficacy of endoscopic duodenal stent placement in patients with malignant gastric outlet obstruction.METHODS: This prospective, observational, multicenter study included 39 consecutive patients with malignant gastric outlet obstruction. All patients underwent endoscopic placement of a nitinol, uncovered, selfexpandable metal stent. The primary outcome was clinical success at 2 wk after stent placement that was defined as improvement in the Gastric Outlet Obstruction Scoring System score relative to the baseline.RESULTS: Technical success was achieved in all duodenal stent procedures. Procedure-related complications occurred in 4 patients(10.3%) in the form of mild pneumonitis. No other morbidities or mortalitieswere observed. The clinical success rate was 92.3%. The mean survival period after stent placement was 103 d. The mean period of stent patency was 149 d and the patency remained acceptable for the survival period. Stent dysfunction occurred in 3 patients(7.7%) on account of tumor growth.CONCLUSION: Endoscopic management using duodenal stents for patients with incurable malignant gastric outlet obstruction is safe and improved patients' quality of life.展开更多
BACKGROUND: Gastrointestinal stromal tumors are the most common mesenchymal tumors of the gastrointestinal tract and occur rarely in the duodenum. Splenic angiosarcoma is an aggressive neoplasm with an extremely poor ...BACKGROUND: Gastrointestinal stromal tumors are the most common mesenchymal tumors of the gastrointestinal tract and occur rarely in the duodenum. Splenic angiosarcoma is an aggressive neoplasm with an extremely poor prognosis. METHODS: We report a case of a 70-year-old man hospitalized for abdominal pain in the upper quadrants, dyspepsia and nausea, previously treated for Hodgkin lymphoma 30 years ago. Abdominal CT showed a solid nodular lesion in the third portion of the duodenum, the presence of retropancreatic, aortic and caval lymph nodes, and four nodular splenic masses. 111 In-octreotide scintigraphy revealed pathological tissue accumulation in the duodenal region, and in the retropancreatic, retroduodenal, aortic and caval lymph nodes, suggesting a nonfunctioning neuroendocrine peripancreatic tumor. RESULTS: At exploratory laparotomy, an exophytic soft tumor was found originating from the third portion of the duodenum. Pancreas-preserving duodenectomy with duodenojejunostomy, splenectomy and lymphnodectomy of retropancreatic aortic and caval lymph nodes were performed. Pathological evaluation and immunohistochemical studies showed the presence of a duodenal gastrointestinal stromal tumor with low mitotic activity and a well-differentiated angiosarcoma localized to the spleen and invading lymph nodes.CONCLUSIONS: We speculated that the angiosarcoma and duodenal gastrointestinal stromal tumors of this patient were due to the treatment of Hodgkin lymphoma with radiotherapy 30 years ago. Pancreas-preserving segmental duodenectomy can be used to treat non-malignant neoplasms of the duodenum and avoid extensive surgery. Splenectomy is the treatment of choice for localized angiosarcomas but a strict follow-up is mandatory because of the possibility of recurrence.展开更多
基金Supported by CAMS Initiative for Innovative Medicine,No.2016-I2M-1-007.
文摘BACKGROUND Duodenal gastrointestinal stromal tumor(DGIST)is a rare tumor with a specific anatomic site and biological characteristics.As the incidence of lymph node metastasis is very low,the main treatment method is surgery.Two main surgical techniques(local resection and Whipple)are performed in patients with DGISTs.The critical question is which surgical technique to choose.AIM To identify factors influencing the choice of surgery for DGISTs.METHODS The clinicopathological data of patients with DGISTs who underwent surgery between January 1999 and January 2021 were analyzed.We used the Student’s ttest or Mann-Whitney U-test and theχ2 test or Fisher’s exact test to determine the differences between the two groups of patients.Furthermore,we used logistic analysis to identify the relevant factors and independent factors related to the type of surgery.The Kaplan-Meier method was used to analyze the patient’s survival information and Cox regression analysis was performed to determine prognostic risk factors.RESULTS Overall,86 patients were analyzed,including 43 men(50%)and 43 women(50%).We divided the patients into two groups based on surgical technique(local resection or Whipple surgery).There were no differences in the age,mitotic figures,and complications between the two groups;however,the tumor size,tumor location,risk grade,postoperative hospital stay,and abdominal drainage time were significantly different.Based on univariate logistic analysis,the Whipple procedure was chosen if the tumor size was≥5.0 cm,the tumor was located in the descending part of the duodenum,or the risk grade was medium or high.In our research,the five-year overall survival rate of patients was more than 90%.We also describe two DGIST patients with liver metastases at first diagnosis and analyzed their management in order to provide advice on complicated cases.CONCLUSION The Whipple procedure was performed if the primary tumor was in the descending part of the duodenum,tumor size was≥5.0 cm,or the tumor risk grade was medium or high.
基金Medical Science and Technology Project of Zhejiang Province,No.2021PY083Program of Taizhou Science and Technology Grant,No.22ywb09+3 种基金Major Research Program of Taizhou Enze Medical Center Grant,No.19EZZDA2Open Project Program of Key Laboratory of Minimally Invasive Techniques&Rapid Rehabilitation of Digestive System Tumor of Zhejiang Province,No.21SZDSYS01 and 21SZDSYS09Key Technology Research and Development Program of Zhejiang Province,No.2019C03040Program of Taizhou Science and Technology Grant,No.1901ky18.
文摘BACKGROUND Endoscopic resection for duodenal gastrointestinal stromal tumors(GISTs)is still considered a great challenge with a high risk of complications,including perforation,bleeding,tumor rupture,and residual tumor.AIM To assess the effectiveness and safety of endoscopic resection for duodenal GISTs.METHODS Between January 2010 and January 2022,11 patients with duodenal GISTs were treated with endoscopic resection.Data were extracted for the incidence of complete resection,bleeding,perforation,postoperative infection,recurrence,and distant metastasis.RESULTS The incidence of successful complete resection of duodenal GISTs was 100%.Three cases(27.3%)had suspected positive margins,and the other 8 cases(72.7%)had negative vertical and horizontal margins.Perforation occurred in all 11 patients.The success rate of perforation closure was 100%,while 1 patient(9.1%)had suspected delayed perforation.All bleeding during the procedure was managed by endoscopic methods.One case(9.1%)had delayed bleeding.Postoperative infection occurred in 6 patients(54.5%),including 1 who developed septic shock and 1 who developed a right iliac fossa abscess.All 11 patients recovered and were discharged.The mean hospital stay was 15.3 d.During the follow-up period(14-80 mo),duodenal stenosis occurred in 1 case(9.1%),and no local recurrence or distant metastasis were detected.CONCLUSION Endoscopic resection for duodenal GISTs appears to be an effective and safe minimally invasive treatment when performed by an experienced endoscopist.
文摘BACKGROUND: Duodenal gastrointestinal stromal tumors, which are rare, comprise 3%-5% of all gastrointestinal stromai tumors. We present a case of a metastatic duodenal gastrointestinal stromal tumor that was successfully treated by simultaneous tight hemihepatectomy and pancreaticoduodenectomy. METHODS: A 50-year-old woman was admitted to our department for the treatment of a possible metastatic duodenal gastrointestinal stromal tumor (GIST). At laparotomy a large duodenal tumor was found displacing the head of the pancreas. A 3 cm in diameter lesion in the posterior aspect of segment Ⅷ of the liver was also noted. Simultaneous right hepatectomy and pancreaticoduodenectomy were performed. RESULTS: Histological examination revealed a high grade metastatic duodenal GIST strongly positive for c-kit, CD34, and vimentin. The patient had no additional therapy. A follow-up of 21 months showed that the patient is very well and there is no evidence of recurrent diseases. CONCLUSIONS: Malignant stromai tumors of the duodenum are rarely encountered. They are usually slow growing, and may be amenable to curative surgery, even after occurrence of metastases. Resection of localized liver metastasis is still advocated when feasible, since imatinib does not provide a complete or long-term response. Combined surgical resection is an efficacious treatment for patients with metastatic duodenal gastrointestinal stromal tumor.
基金Supported by the Guide Project for Key Research and Development Foundation of Liaoning Province,No.2019JH8/10300008the 345 Talent Projectthe Liaoning Baiqianwan Talents Program.
文摘BACKGROUND Liver metastasis of duodenal gastrointestinal stromal tumor(GIST)is rare.Most reports mainly focus on its treatment and approaches to surgical resection,while details on its contrast-enhanced ultrasound(CEUS)findings are lacking.The diagnosis and imaging modalities for this condition remain challenging.CASE SUMMARY A 53-year-old Chinese man presented with mild signs and symptoms of the digestive tract.He underwent routine examinations after GIST surgery.Magnetic resonance imaging showed a 2.3 cm hepatic space-occupying lesion.All the laboratory test results were within normal limits.For further diagnostic confirmation,we conducted high frame rate CEUS(H-CEUS)and found a malignant perfusion pattern.Heterogeneous concentric hyper-enhancement,earlier wash-in than the liver parenchyma,and two irregular vessel columns could be observed at the periphery of the lesion during the arterial phase.Ultrasound-guided puncture biopsy was used to confirm the diagnosis of the lesion as liver metastasis of duodenal GIST.Imatinib was prescribed after biopsy,and the patient’s clinical course was monitored.CONCLUSION H-CEUS is useful for detecting microcirculation differences,wash-in patterns,and vascular morphogenesis and diagnosing liver metastasis of duodenal GIST.
文摘Introduction: The coexistence of synchronic duodenal gastrointestinal stromal tumor (GIST) and neuroendocrine tumor in a patient with neurofibromatosis type 1 (NF1) is extremely rare, and only eight cases were described in the literature. Clinical Case: This is a rare case of a 38-year-old female patient with NF1 who developed synchronic GIST and neuroendocrine tumor, which were both in the second portion of the duodenum. The first symptoms were abrupt digestive bleeding and anemia. Upper digestive endoscopy revealed two tumors, sizes 2.5 and 3.0 cm, in the second portion of duodenum, with biopsies identifying a GIST and a neuroendocrine tumor. Therapeutic decision was to proceed to surgical resection, and Whipple’s procedure was indicated. Surgical procedure was performed with good outcome. Currently the patient has excellent quality of life and maintains follow up for thirty months without recurrence. Discussion: Long-term disease-free survival and excellent quality of life are reported when these tumors are fully resected in this context. However, it is not always easy to access the gastrointestinal tract, especially the small intestine, and proceed to the histopathologic diagnosis of these tumors. Conclusion: It is important to be aware of the possibility of the coexistence of various tumors in the NF1 scenario for adequate screening, staging, and surgical treatment of these patients, as good prognosis can be achieved when such tumors are detected and treated properly.
文摘Introduction: This study investigated the incidence, surgical management and outcome of Gastrointestinal Stromal Tumors (GIST) in Upper Egypt. Methods: A retrospective review of all GIST patients admitted a South Egypt Cancer Institute between Jan. 2010 and Dec. 2015 was conducted. Patients’ demographics, clinical presentation, tumor characteristics, radiological, pathological and immunohistochemical findings, surgical procedures, recurrence and mortality were recorded. Results: A total of 36 GIST patients were identified, stomach was the most common site (27.8%) followed by the small intestine (19.4%) and the large intestine (16.7%). The mean age at time of diagnosis as 52.8 ± 14.4 (ranged from 17 to 76 years). Of these 36 cases, 20 (55.6%) cases were males and 16 (44.4%) cases were females with a ratio of 1.2:1. About 22 cases (61.1%) presented with primary tumors, eight cases (22.2%) had primary tumors and metastases, three cases (8.35) presented with recurrent mass, whereas one case (2.2%) presented either with recurrent mass and metastases or metastases only. The majority of cases (22) had tumorsize >5 cm. Patients were stratified as high, intermediate, low and very low risk (50.6%, 30.6%, 11.1% and 2.8%, respectively). Almost all the cases were surgically managed and 75% were completely resectable. During follow up (average 26.5 months), 22 patients showed complete recovery, 7 had recurrent or metastatic disease and 2 died due to liver metastasis. Conclusion: The incidence of GIST in Upper Egypt is apparently low. Surgical resection is the preferred choice of treatment. The demographic data of GIST patients in South Egypt Cancer institute were similar to those published in the literature. Other prospective studies are required to assess the prognosis and the effect of treatment.
文摘Introduction: Colorectal gastrointestinal stromal tumors (GISTs) mesenchymal tumor is very uncommon. GISTs effect mostly on the stomach and small intestine and rarely occur in the colon, rectum and esophagus, that originating from precursors of the interstitial cells that originate of Cajal. The symptoms of gastrointestinal stromal tumor depend on the site and size of the tumor, and may include abdominal pain, gastrointestinal bleeding or signs of obstruction;small tumors may, however, be asymptomatic. Some of the patients with gastrointestinal stromal tumor have bloody stools, obstruction and abdominal pain as the commonest manifestation. Immunocytochemical staining for CD117 is helpful in confirming the diagnosis. Case presentation: We report 3 new cases of GISTs: two occurred at the rectal and the other at descending Colon. Two cases are over 50 years of age and, and all cases the chief complain of bowel obstruction, abdominal pain in two cases, and one case with anemia and urine retention. All the patients were operated and were permormed pathology examinatiom. All case ware positive result for immunocytochemical staining CD117. All cases we had presented had size more than 5 cm are considered as unfavorable prognostic factors to Fletcher criteria, all patients scheduled for chemotherapy with Glivec but just one patient continued to used Glivec. Post surgery follows up one patient post milles with urinary incontinence complaints found and that patients are trained to CIC (intermittent catheterization). Conclusion: Colorectal gastrointestinal stromal tumors are very rare and can present as mass abdomen. Resection and chemotherapy are the treatment of choice.
文摘Gastrointestinal stromal tumor (GIST) is the most common mesenchymal malignancy of the gastrointestinal tract.GISTs may coexist with different types of cancer,either synchronous or metachronous (1).Most GISTs develop in a sporadic fashion,but familial occurrence,such as neurofibromatosis and Carney-triad,has also been reported (2).The overall frequency of second tumors in different series varied from 4.5% to 33%.The most frequent types of GIST-associated cancers were gastrointestinal carcinomas (47%),lymphoma/leukemia (7%),carcinomas of prostate (9%),breast (7%),kidney (6%),lung (5%),female genital tract (5%),carcinoid tumors (3%),soft tissue and bone sarcomas (3%),malignant melanoma (2%) and seminoma (1%) (1,3-5).
文摘Epithelioid gastrointesinal tumors (GISTs) are less likely to have c-kit gene mutations (and express CD117) than spindle cell GISTs. CD117 negative/c-kit negative GISTs can have platelet-derived growth factor alpha (PDGFRα) gene mutations, overexpress PDGFRα protein and respond to imatinib mesylate. Many cases of CD117-negative/CD117-weakly positive, c-kit mutation negative and PDGFRα mutation positive myxoid epithelioid GISTs and one case of CD117-positive GIST with chondro-myxoid features mimicking chondrosarcoma have been reported. We report a case of myxoid epithelioid GIST with predominance of chondroid and chordoid areas resembling an extraskeletal myxoid chondrosarcoma that was strongly positive for CD117, PDGFRα and DOG1 (Discovered on GIST 1) by immunohistochemistry, but lacked c-kit and PDGFRα gene mutations. It is possible that CD117 is strongly positive if a myxoid epithelioid GIST has chondroid/chordoid appearance, but a larger study is needed to confirm this association. CD117 expression in GISTs is important, because GISTs showing CD117 positivity respond to imatinib. No comment can be made about the prognostic significance of chondroid/chordoid appearance in the GISTs.
文摘Introduction: Majority of mesenchymal tumors of gastrointestinal tract are Gastrointestinal Stromal Tumor (GIST). It is, however, a rare tumor, accounting for less than 1% of primary gastrointestinal (GI) neoplasms. Though, these tumors are refractory to conventional chemotherapy or radiotherapy but show a good response to targeted adjuvant chemotherapy with tyrosine kinase inhibitors following surgical resection. Case Report: we report here a case of primary Extra-GIST tumor arising from mesentry of small bowel near duodeno-jejunal junction in a 69 years old male patient. The patient presented with a palpable mass in upper abdomen for past 15 days. On examination, a non-tender mobile lump of size around 17 × 10 cm, with bosselated surface and firm in consistency was palpable involving epigastric, left hypochondrium and umbilical region. Contrast enhanced computed tomography of abdomen revealed a heterogenous mesentric mass. On surgical intervention a mass was found involving mesentery near dudenojejunal junction without involvement of gastrointestinal tract. Complete surgical resection of the tumor was done and adjuvant chemotherapy with Imatinib mesylate was started as HPE revealing GIST with mitotic index of >10/50 HPF and 17 × 10 cm size placed the patient in high risk category. Patient was discharged on 12th of post-operative day with advice of regular follow-up. Conclusion: GIST occurrence is not restricted to bowel but can involve unusual sites also. The mainstay of treatment remains surgical resection with adequate margin. In cases where tumour has malignant potential (high mitotic figures on histopathology) adjuvent treatment with tyrosine kinase may prevent or delay relapse.
文摘BACKGROUND Gastrointestinal stromal tumors(GISTs)are mesenchymal tissue tumors originating from Cajal cells,presenting diverse clinical manifestations due to the different sizes,locations,and growth patterns of the lesions.Duodenum is an uncommon site of GISTs,more with gastrointestinal obstruction and bleeding as the first symptoms.Ectopic duodenal varix,as a rare varix occurring outside the gastroesophageal region,is the main type of heterotopic varices and an unusual cause of gas-trointestinal hemorrhage.The etiology is mainly seen in liver cirrhosis,portal hypertension,vasculitis,portal vein embolism and obstruction caused by various factors.Reports of duodenal stromal tumor combined with ectopic variceal hemorrhage are rarely seen;however,when it occurs,the situation can sometimes be urgent and life-threatening,especially when traditional endoscopy and imaging fail to detect the lesion timely.CASE SUMMARY We report a 52-year-old female patient who had no obvious inducement to develop black stool.Gastroscopy in a local hospital revealed that the duodenal horizontal ectopic varices were ruptured and bleeding.After metal clamping hemostasis,she still had gastrointestinal bleeding and was transferred to our hospital.Gastroscopy showed that active bleeding was still seen in the horizontal part of duodenum,and suspicious submucosal eminence was seen in the bleeding part.Abdominal computed tomography showed a huge stromal tumor of duodenum,specimens were pathologically confirmed after surgery.After a 3-mo follow-up,no gastrointestinal hemorrhage and complications occurred.CONCLUSION Ectopic variceal hemorrhage is rare but sometimes fatal.It may be combined with stromal tumor,which can be diagnosed by multiple methods.Endoscopic and surgical treatment are effective.
文摘Gastrointestinal stromal tumor (GIST) is the most common mesenchymal tumor and has a malignant potential. The clinical presentation with pneumoperitoneum and peritonitis is extremely rare. We report a case of a 40-year-old male presented with symptoms of acute abdomen. Radiological work-up confirmed pneumoperitoneum. Emergency laparatomy and complete resection were performed. The final diagnosis revealed perforated GIST originating from the jejunum. If an abdominal mass presents with pneumoperitoneum and peritonitis, jejunal GIST should be considered in diagnosis. A complete radical resection followed by postoperative adjuvant chemotheraphy with Imatinib is recommended.
文摘Gastrointestinal stromal tumors (GISTs) account for approximately 2% of gastric cancers and can be challenging to diagnose due to the difficulty sampling tissue from these lesions. Current biopsy methods are inadequate for determining the mitotic index, a major prognostic factor of these tumors. We sought to evaluate the ability of a prototype submucosal biopsy forceps to make a histologic diagnosis and mitotic index determination in cases of resected gastric GISTs. After obtaining informed consent and surgical resection of three gastric GISTs, an investigational submucosal biopsy forceps and sheath was passed under direct visualization into the central portion of the tumors (in the ex vivo setting) and biopsies were obtained. The tumor was then processed for standard histology. A gastrointestinal pathologist, blinded to the mitotic index of the full specimen, evaluated the research biopsies. The results from the submucosal forceps biopsies were then compared with the standard histology results. n all 3 cases, the submucosal forceps biopsies confirmed the diagnosis of a GIST. In two out of three cases, the submucosal forceps biopsies accurately classified the risk for progressive disease (based on the mitotic index) when compared to standard histology. In one case, the research biopsy mitotic index would have resulted in an underestimation of tumor risk (biopsies categorized the lesion as “very low” rather than “moderate” risk).
文摘Objective:The aim of the study was to review the clinical records of 122 patients with gastrointestinal stromal tumors(GISTs) and analyze their clinicopathologic and immunohistochemical characteristics.Methods:The medic records of 122 patients with GISTs during the periods from January 2002 to May 2010 were reviewed.All tumors were confirmed by histological and immunohistochemical analyses.Results:The tumors occurred in 59 males and 63 females,ranging from 25 to 77 years.Of all cases,46 cases originated from stomach,42 from small intestine,17 from colon and rectum and 9 from retroperitoneal cavity and 4 cases from extra-gastrointestinal site.Liver was the most common organ that tumors metastases involved.Immunohistochemically,there were 114 tumors being positive for CD117 while 8 tumors negative for it.The frequencies of CD34 positive were higher in the stomach and rectum(89.1% and 86.7% respectively) than in the small intestine(64.3%,P < 0.05).Higher expression of SMA was in the tumors located in small intestine(54.8%) while the expressions of SMA in the gastric and rectal tumors were relatively low(21.7% and 20.0% respectively,P < 0.05).Conclusion:Gastrointestinal stromal tumors can occur in the gastrointestinal tract as well as in the extra-gastrointestinal sites.The frequencies of CD34 and SMA expression vary significantly with different locations.
文摘The coronavirus disease 2019(COVID-19)pandemic profoundly affected the management and treatment of patients with malignancies.Based on the progress reported in the literature,we reviewed the recommendations for treatment and vaccination in patients with gastrointestinal stromal tumor(GIST)during COVID-19.We focus on whether there is a risk and what could be the possible effects of vaccinating patients with GIST/cancer.Since the situation is quickly changing,and the health services have been severely disrupted,the diagnosis,treatment and recommendations for vaccination of these patients against COVID-19 are still not updated.The approval of vaccines in the pandemic gave hope that we would soon be able to return to a more normal life.However,the oncology community needs to adapt and provide the most effective treatment and care models for patients with rare cancer,such as GIST.Collecting data on the impact of vaccination in patients with GIST/cancer also will be beneficial in expanding knowledge about the future planning of treatment strategies and optimizing care in the event of a subsequent pandemic.
文摘AIM: To evaluate the results of segmental duodenectomy (SD) and pancreaticoduodenectomy (PD) for duodenal gastrointestinal stromal tumor (GIST) and help clinicians with surgical management. METHODS: All patients who underwent surgery for non-metastatic GIST of the duodenum in a single institution since 2000 were prospectively followed up. Seven patients (median age 51 years, range: 41-73 years) were enrolled: five underwent SD and two underwent PD. RESULTS: All the patients had a complete resection (R0), with no postoperative morbidity and mortality. Among the SD group, GIST was classified as low risk in two patients, intermediate risk in two, and high risk in one, according to the Fletcher scale, (vs two high risk patients in the PD group). With a median followup of 41 (18-85) mo, disease-free survival (DFS) rateswere 100% after SD and 0% after PD (P < 0.05). The median DFS was 13 mo in the PD group. CONCLUSION: Whenever associated with clear surgical margins, SD is a reliable and curative option for most duodenal GISTs, and is compatible with longterm DFS.
基金supported by WU JIEPING Medical Foundation(No.WJP-320.6700.09010)
文摘Gastrointestinal stromal tumors (GISTs) occur most frequently in the stomach.Diagnosis of gastric GIST is not always clear before surgery.Flexible endoscopy may suggest the nature of the lesion (a bulky tumor with preserved mucosa); however,biopsy is rarely diagnostic.Therefore,diagnostic medication with safe drugs may provide a feasible way under such conditions after an informed consent is obtained.Based on the excellent efficacy of imatinib mesylate (IM) in the treatment of GIST,we successfully applied it in the diagnostic medication of two patients with clinically suspected gastric stromal tumors.In conclusion,the diagnostic medication with IM can be an alternative option for patients with suspected GIST that can not be confirmed pathologically.
文摘AIM: To determine the safety and efficacy of endoscopic duodenal stent placement in patients with malignant gastric outlet obstruction.METHODS: This prospective, observational, multicenter study included 39 consecutive patients with malignant gastric outlet obstruction. All patients underwent endoscopic placement of a nitinol, uncovered, selfexpandable metal stent. The primary outcome was clinical success at 2 wk after stent placement that was defined as improvement in the Gastric Outlet Obstruction Scoring System score relative to the baseline.RESULTS: Technical success was achieved in all duodenal stent procedures. Procedure-related complications occurred in 4 patients(10.3%) in the form of mild pneumonitis. No other morbidities or mortalitieswere observed. The clinical success rate was 92.3%. The mean survival period after stent placement was 103 d. The mean period of stent patency was 149 d and the patency remained acceptable for the survival period. Stent dysfunction occurred in 3 patients(7.7%) on account of tumor growth.CONCLUSION: Endoscopic management using duodenal stents for patients with incurable malignant gastric outlet obstruction is safe and improved patients' quality of life.
文摘BACKGROUND: Gastrointestinal stromal tumors are the most common mesenchymal tumors of the gastrointestinal tract and occur rarely in the duodenum. Splenic angiosarcoma is an aggressive neoplasm with an extremely poor prognosis. METHODS: We report a case of a 70-year-old man hospitalized for abdominal pain in the upper quadrants, dyspepsia and nausea, previously treated for Hodgkin lymphoma 30 years ago. Abdominal CT showed a solid nodular lesion in the third portion of the duodenum, the presence of retropancreatic, aortic and caval lymph nodes, and four nodular splenic masses. 111 In-octreotide scintigraphy revealed pathological tissue accumulation in the duodenal region, and in the retropancreatic, retroduodenal, aortic and caval lymph nodes, suggesting a nonfunctioning neuroendocrine peripancreatic tumor. RESULTS: At exploratory laparotomy, an exophytic soft tumor was found originating from the third portion of the duodenum. Pancreas-preserving duodenectomy with duodenojejunostomy, splenectomy and lymphnodectomy of retropancreatic aortic and caval lymph nodes were performed. Pathological evaluation and immunohistochemical studies showed the presence of a duodenal gastrointestinal stromal tumor with low mitotic activity and a well-differentiated angiosarcoma localized to the spleen and invading lymph nodes.CONCLUSIONS: We speculated that the angiosarcoma and duodenal gastrointestinal stromal tumors of this patient were due to the treatment of Hodgkin lymphoma with radiotherapy 30 years ago. Pancreas-preserving segmental duodenectomy can be used to treat non-malignant neoplasms of the duodenum and avoid extensive surgery. Splenectomy is the treatment of choice for localized angiosarcomas but a strict follow-up is mandatory because of the possibility of recurrence.