A large gastrointestinal stromal tumor of the duodenum treated by partial duodenectomy with Roux-en-Y duodenojejunostomy: a case report

INTRODUCTION: Duodenal gastrointestinal stromal tumors are uncommon and a relatively small subset of gastrointestinal stromal tumors whose optimal surgical procedure has not been well defined. Because submucosal spread and local lymph node involvement are infrequent in gastrointestinal stromal tumors, wide margins with routine lymph node dissection may not be required. Various surgical procedures for duodenal gastrointestinal stromal tumor, pancreatoduodenectomy, pancreas-sparing duodenectomy, segmental duodenectomy, or local resection, have been described depending on the size and exact site of the lesion. CASE PRESENTATION: We present the case of a 65-year-old African woman with a giant gastrointestinal stromal tumor involving the second and third portion of the duodenum successfully treated by partial duodenectomy with duodenojejunostomy. This surgical technique is ideal when a gastrointestinal stromal tumor does not involve the ampulla because it avoids a pancreatoduodenectomy, and has not been previously described for the management of this malignancy. Duodenal gastrointestinal stromal tumor should be suspected in any patient with a duodenal wall mass. CONCLUSIONS: Gastrointestinal stromal tumors of the duodenum should be suspected in any patient with a duodenal wall mass. Extramural growth and central ulceration with or without bleeding should alert the endoscopist to the possibility of a duodenal gastrointestinal stromal tumor diagnosis

Unusual appearance of a pendulated gastric tumor : always think of GIST

Objective. To investigate the clinicopathological characteristics of gastrointestinal stromal tumor (GIST) with significant cystic changes and to assess the molecular genetic characteristics. Methods. In a 68-year-old man, a large abdominal tumoral mass was discovered incidentally. Computed tomography (CT) and magnetic resonance imaging (MRI) confirmed the presence of a large cystic lesion with multiple contrast-enhancing septae and papillary projections. No clear connection with any of the surrounding organs was identified. Malignancy could not be excluded, and surgery was indicated. During surgery, the large mass was found to be attached by a narrow stalk to the large curvature of the stomach. Results. The histological features and immunohistiochemical profile of the tumor cells (positivity for CD117 and CD34) were consistent with a gastrointestinal stromal tumor with a high risk of progressive disease according to the Fletcher classification. Diagnosis was confirmed by mutational analysis; this demonstrated mutation in exon 14 of PDGFRA. During the followup of 97 months, the patient had a cancer-free survival. Conclusions. This case demonstrates that gastrointestinal stromal tumors (GISTs) with extensive cystic degeneration should be considered in the differential diagnosis of a cystic abdominal mass

Gastrointestinal stromal tumor

<p>Abstract</p> <p>Background</p> <p>GISTs are a subset of mesenchymal tumors and represent the most common mesenchymal neoplasms of GI tract. However, GIST is a recently recognized tumor entity and the literature on these stromal tumors has rapidly expanded.</p> <p>Methods</p> <p>An extensive review of the literature was carried out in both online medical journals and through Athens University Medical library. An extensive literature search for papers published up to 2009 was performed, using as key words, GIST, Cajal's cells, treatment, Imatinib, KIT, review of each study were conducted, and data were abstracted.</p> <p>Results</p> <p>GIST has recently been suggested that is originated from the multipotential mesenchymal stem cells. It is estimated that the incidence of GIST is approximately 10-20 per million people, per year.</p> <p>Conclusion</p> <p>The clinical presentation of GIST is variable but the most usual symptoms include the presence of a mass or bleeding. Surgical resection of the local disease is the mainstay therapy. However, therapeutic agents, such as Imatinib have now been approved for the treatment of advanced GISTs and others, such as everolimus, rapamycin, heat shock protein 90 and IGF are in trial stage demonstrate promising results for the management of GISTs.</p

Gastrointestinal stromal tumors with pseudocystic change mimicking a pancreatic tumor: two case reports

<p>Abstract</p> <p>Introduction</p> <p>Cystic lesions of the upper abdomen normally develop from pancreatic tissue. The differential diagnoses include neoplastic and non-neoplastic lesions. Pseudocystic tumors that secondarily involve the pancreas are very rare and may lead to diagnostic pitfalls.</p> <p>Case presentation</p> <p>A 51-year-old woman and a 65-year-old man, both German, presented with abdominal cystic lesions suspected to be pancreatic pseudocysts. Both tumors were classified as gastrointestinal stromal tumors, epithelioid subtype. In one case, tumor origin in the gastric wall was confirmed by relaparotomy. In the other case, a point mutation in <it>PDGFRalpha</it> gene, exon 18 proved the diagnosis of gastrointestinal stromal tumor. The tumors were resected and both patients are still alive and disease-free.</p> <p>Conclusions</p> <p>The differential diagnosis of cystic lesions in the upper abdomen must include gastrointestinal stromal tumors with pseudocystic change. The origin of a large cystic gastrointestinal stromal tumor may be difficult to determine. Epithelioid tumor pattern, weak or absent KIT expression and detection of <it>PDGFRalpha</it> mutation are typical diagnostic parameters of gastric gastrointestinal stromal tumors.</p

Gastrointestinal stromal tumor (GIST) of the Treitz’s angle– a very rare cause of high bowel obstruction

Gastrointestinal stromal tumors (GIST) are somewhat rare gastrointestinal tumors - approximately 1% to 3% incidence, but they are the most common mesenchymal neoplasms of the gastrointestinal tract. GISTs are usually found in the stomach or small intestine but can occur anywhere within the gastrointestinal tract, even in extremely uncommon locations like duodeno-jejunal flexure. Only 3% – 5% of GISTs are located in the duodenum and tumors occurring in the angle of Treitz are even rarer, most published studies being case reports. These tumors have a size ranging from small lesions to large masses and can cause digestive bleeding or high bowel obstruction. This paper is a case presentation illustrating an emergency situation involving a high bowel obstruction caused by a small tumor with an unusual location in the Treitz’s angle. A large percentage of duodenal GISTs are localized in the third and fourth part of the duodenum and may not be found through standard upper endoscopy; only the barium study of the upper gastrointestinal tract highlights the obstruction point. Preoperative diagnosis is difficult but non-invasive imaging techniques like ultrasonography and computed tomography of the abdomen can be helpful. Recently, targeted therapy with inhibitors of tyrosine kinase receptors (IMATINIB) has been introduced for the management of advanced and metastatic tumors. In our opinion the surgical resection with curative intent is the treatment of choice

A large gastrointestinal stromal tumor of the duodenum: a case report

<p>Abstract</p> <p>Introduction</p> <p>Gastrointestinal stromal tumors of the duodenum are uncommon. They can reach a large size. Diagnosis can be elusive and managing them can be difficult. Our case report aims to increase awareness and highlight some issues related to the diagnosis and management of duodenal gastrointestinal stromal tumors.</p> <p>Case presentation</p> <p>We present the case of a 38-year-old Middle Eastern woman with a large, slowly-growing gastrointestinal stromal tumor of the duodenum. Her complaints were minor epigastric discomfort and swelling. A pancreaticoduodenectomy with complete tumor excision was performed. She was doing very well with no evidence of disease recurrence when she was last seen 34 months after her operation.</p> <p>Conclusion</p> <p>Gastrointestinal stromal tumors of the duodenum should be suspected in any patient with a duodenal wall mass. Extramural growth and central ulceration with or without bleeding should alert the endoscopist to the possibility of a duodenal gastrointestinal stromal tumor diagnosis. There is more than one surgical approach available; however, complete surgical excision, with negative margins, is the absolute requirement. Preoperative imatinib mesylate can be considered in unresectable or borderline resectable cases.</p

Generation of orthotopic patient-derived xenografts from gastrointestinal stromal tumor.

BackgroundGastrointestinal stromal tumor (GIST) is the most common sarcoma and its treatment with imatinib has served as the paradigm for developing targeted anti-cancer therapies. Despite this success, imatinib-resistance has emerged as a major problem and therefore, the clinical efficacy of other drugs has been investigated. Unfortunately, most clinical trials have failed to identify efficacious drugs despite promising in vitro data and pathological responses in subcutaneous xenografts. We hypothesized that it was feasible to develop orthotopic patient-derived xenografts (PDXs) from resected GIST that could recapitulate the genetic heterogeneity and biology of the human disease.MethodsFresh tumor tissue from three patients with pathologically confirmed GISTs was obtained immediately following tumor resection. Tumor fragments (4.2-mm3) were surgically xenografted into the liver, gastric wall, renal capsule, and pancreas of immunodeficient mice. Tumor growth was serially assessed with ultrasonography (US) every 3-4 weeks. Tumors were also evaluated with positron emission tomography (PET). Animals were sacrificed when they became moribund or their tumors reached a threshold size of 2500-mm3. Tumors were subsequently passaged, as well as immunohistochemically and histologically analyzed.ResultsHerein, we describe the first model for generating orthotopic GIST PDXs. We have successfully xenografted three unique KIT-mutated tumors into a total of 25 mice with an overall success rate of 84% (21/25). We serially followed tumor growth with US to describe the natural history of PDX growth. Successful PDXs resulted in 12 primary xenografts in NOD-scid gamma or NOD-scid mice while subsequent successful passages resulted in 9 tumors. At a median of 7.9 weeks (range 2.9-33.1 weeks), tumor size averaged 473 ± 695-mm³ (median 199-mm3, range 12.6-2682.5-mm³) by US. Furthermore, tumor size on US within 14 days of death correlated with gross tumor size on necropsy. We also demonstrated that these tumors are FDG-avid on PET imaging, while immunohistochemically and histologically the PDXs resembled the primary tumors.ConclusionsWe report the first orthotopic model of human GIST using patient-derived tumor tissue. This novel, reproducible in vivo model of human GIST may enhance the study of GIST biology, biomarkers, personalized cancer treatments, and provide a preclinical platform to evaluate new therapeutic agents for GIST

Laparoscopic resection of gastric GISTs. Where do we stand now? A single-centered experience

Introduction. Gastrointestinal stromal tumors (GISTs) represent a rare type of gastrointestinal neoplasms. Resection with negative margins has been established as a mainstay treatment, but laparoscopic resections are still open to debate. Material and method. This retrospective study was conducted at a single institution, with data collected over 2 years (01.01.2017-01.01.2019). The variables examined were age, tumor location with regard to the gastric wall, the results of the intraoperative endoscopy, intraoperative and postoperative complications, the surgical technique, and histopathological reports. Results. We identified 12 relevant cases, of which 8 were females and 4 males. The average tumor diameter was 2.3 cm. The majority of the lesions were located on the anterior gastric wall (8 cases), on the small curvature (2 cases), and in the pyloric region (2 cases). Intraoperative endoscopy was performed successfully in 10 cases in order to identify the lesions and guide the resection. The average operative time was 120 minutes and the average hospital stay was 5 days. The gastric wall with the lesion was resected using an Ultrasonic device, a 2-cm oncological safety margin was preserved. Conclusion. Complete surgical resection independent from the tumor size represents the current optimal treatment. From a surgical point of view, these tumors must be considered malignant and the surgeon must respect principles of oncological surgery. Maintaining tumor integrity at dissection is critical for the patient’s long-term prognosis. Laparoscopic resection independent of the tumor size is feasible

Surgical treatment of gastrointestinal stromal tumors of the duodenum. A literature review

Background: Gastrointestinal stromal tumors (GIST) are the most frequent mesenchymal tumours in the digestive tract. The duodenal GIST (dGIST) is the rarest subtype, representing only 4–5% of all GIST, but up to 21% of the resected ones. The diagnostic and therapeutic management of dGIST may be difficult due to the rarity of this tumor, its anatomical location, and the clinical behavior that often mimic a variety of conditions; moreover, there is lack of consent for their treatment. This study has evaluated the scientific literature to provide consensus on the diagnosis of dGIST and to outline possible options for surgical treatment. Methods: An extensive research has been carried out on the electronic databases MEDLINE, Scopus, EMBASE and Cochrane to identify all clinical trials that report an event or case series of dGIST. Results: Eighty-six studies that met the inclusion criteria were identified with five hundred forty-nine patients with dGIST: twenty-seven patients were treated with pancreatoduodenectomy and ninety-six with only local resection (segmental/wedge resections); in four hundred twenty-six patients it is not possible identify the type of treatment performed (pancreatoduodenectomy or segmental/wedge resections). Conclusions: dGISTs are a very rare subset of GISTs. They may be asymptomatic or may involve symptoms of upper GI bleeding and abdominal pain at presentation. Because of the misleading clinical presentation the differential diagnosis may be difficult. Tumours smaller than 2 cm have a low biological aggressiveness and can be followed annually by endoscopic ultrasound. The biggest ones should undergo radical surgical resection (R0). In dGIST there is no uniformly adopted surgical strategy because of the low incidence, lack of experience, and the complex anatomy of the duodenum. Therefore, individually tailored surgical approach is recommended. R0 resection with 1–2 cm clear margin is required. Lymph node dissection is not recommended due to the low incidence of lymphatic metastases. Tumor rupture should be avoided