Gastrointestinal Stromal Tumor (GIST) Presenting as a Huge Pelvic Mass.
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ABSTRACT:
SUBMITTER: Lee T
PROVIDER: S-EPMC3859154 | biostudies-other | 2013 Jan
REPOSITORIES: biostudies-other
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Project description:A 71-year-old man was referred for an anterior chest wall mass. Chest computed tomography (CT) and positron emission tomography-CT suggested a malignant tumor. Surgical biopsy through a vertical subxiphoid incision revealed an extra-gastrointestinal stromal tumor (EGIST). En bloc resection of the tumor, including partial resection of the sternum, costal cartilage, pericardium, diaphragm, and peritoneum, was performed. Pathologic evaluation revealed a negative resection margin and confirmed the tumor as an EGIST. On postoperative day 17, the patient was discharged without any complications. At the 2-week follow-up, the patient was doing well and was asymptomatic.
| S-EPMC5548212 | biostudies-other
Project description:Tumor rupture is an important risk factor predictive of recurrence after macroscopically complete resection of gastrointestinal stromal tumors (GISTs), and an indication for defined interval or even lifelong adjuvant therapy with imatinib according to guidelines. However, there is no consensus or universally accepted definition of the term 'tumor rupture', and, consequently, its incidence varies greatly across reported series. Without predefined criteria, the clinical significance of rupture has also been difficult to assess on multivariate analysis of retrospective data. We reviewed the relevant literature and international guidelines, and, based on the Oslo criteria, proposed the following six definitions for 'tumor rupture': (1) tumor fracture or spillage; (2) blood-stained ascites; (3) gastrointestinal perforation at the tumor site; (4) microscopic infiltration of an adjacent organ; (5) intralesional dissection or piecemeal resection; or (6) incisional biopsy. Not all minor defects of tumor integrity should not be classified as rupture, i.e. mucosal defects or spillage contained within the gastrointestinal lumen, microscopic tumor penetration of the peritoneum or iatrogenic damage only to the peritoneal lining, uncomplicated transperitoneal needle biopsy, and R1 resection. This broad definition identifies GIST patients at particularly high risk of recurrence in population-based cohorts; however, its applicability in other sarcomas has not been investigated. As the proposed definition of tumor rupture in GIST has limited evidence based on the small number of patients with rupture in each retrospective study, we recommend validating the proposed definition of tumor rupture in GIST in prospective studies and considering it in clinical practice.
| S-EPMC6510879 | biostudies-literature
Project description:IntroductionGastrointestinal stromal tumors (GISTs) are mesenchymal tumors that occur along the alimentary tract, and are most commonly found in the stomach. Rarely, these tumors can occur in the small bowel, and when located in the duodenum or proximal jejunum, they may require challenging reconstruction of the alimentary tract. Patients with GISTs often present with non-specific abdominal pain or symptoms of obstruction, hemorrhage, and less commonly perforation.Presentation of caseA 46-year-old male presented to the hospital with a one-day history of left upper quadrant pain with fevers and chills. Physical examination was significant for signs of peritonitis, and laboratory results revealed leukocytosis and lactic acidosis. CT abdomen showed a large soft tissue mass in the proximal jejunum. In the operating theater he was found to have a perforated jejunal tumor. Pathology report revealed a 13cm GIST, pT4N0M0, Stage IIIa. He had an uneventful recovery and was discharged nine days after surgery.DiscussionProximal jejunal GISTs are a rare entity and when present, perforation is unlikely. Pathological diagnosis of GISTs are relies on immunohistochemistry demonstrating c-KIT or CD34 positivity. The prognosis of GISTs are dependent on the size and the mitotic index. Definitive treatment of non-metastatic GISTs is R0 resection. When located in the duodenum or proximal jejunum, resection can be very challenging and may require clinical expertise in order to safely perform complex alimentary tract reconstruction.ConclusionFurther investigation is required in order to determine best practice management for patients who present with proximal GISTs.
| S-EPMC7381426 | biostudies-literature
Project description:Accounting for less than one percent of all GI tumors, gastrointestinal stromal tumors are uncommon neoplasms that arise from the intestinal cells of Cajal. They can arise anywhere along the gastrointestinal tract, but most often arise from the stomach or small bowel. Usually, they are small and present with vague symptoms such as abdominal pain and are found incidentally. They usually don't involve other structures unless they metastasize. Here, we present a case of small bowel GIST. The patient is a 72-year-old male who was seen in the clinic with symptoms of abdominal pain for 4 months along with intermittent gastrointestinal bleeding for 6 years. Imaging studies revealed a mass in the small bowel with no signs of obstruction. During surgery, a 9 cm by 9 cm small bowel mass was identified with adhesions to the appendix, omentum, and a segment of small bowel. There was also a hemoperitoneum. En bloc resection was performed with clean margins. Histopathology report showed malignant gastrointestinal stromal tumor with focal involvement of adjacent small bowel loop.
| S-EPMC7494819 | biostudies-literature
Project description:BackgroundSynovial sarcoma presenting in the mediastinum is exceedingly rare. Furthermore, data addressing optimal therapy is limited. Herein we present a case where an attempt to downsize the tumor to a resectable state with chemotherapy was employed.Case presentationA 32 year female presented with massive pericardial effusion and unresectable huge mediastinal mass. Computed axial tomography scan - guided biopsy with adjunctive immunostains and molecular studies confirmed a diagnosis of synovial sarcoma. Following three cycles of combination Ifosfamide and doxorubicin chemotherapy, no response was demonstrated. The patient refused further therapy and had progression of her disease 4 months following the last cycle.ConclusionSynovial sarcoma presenting with unresectable mediastinal mass carry a poor prognosis. Up to the best of our knowledge there are only four previous reports where primary chemotherapy was employed, unfortunately; none of these cases had subsequent complete surgical resection. Identification of the best treatment strategy for patients with unresectable disease is warranted. Our case can be of benefit to medical oncologists and thoracic surgeons who might be faced with this unique and exceedingly rare clinical scenario.
| S-EPMC3701506 | biostudies-literature
Project description:Despite significant treatment advances over the past decade, metastatic gastrointestinal stromal tumor (GIST) remains largely incurable. Rare diseases, such as GIST, individually affect small groups of patients but collectively are estimated to affect 25 to 30 million people in the United States alone. Given the costs associated with the discovery, development, and registration of new drugs, orphan diseases such as GIST are often not pursued by mainstream pharmaceutical companies. As a result, "drug repurposing" or "repositioning," has emerged as an alternative to the traditional drug development process. In this study, we screened 796 U.S. Food and Drug Administration (FDA)-approved drugs and found that two of these compounds, auranofin (Ridaura) and fludarabine phosphate, effectively and selectively inhibited the proliferation of GISTs, including imatinib-resistant cells. One of the most notable drug hits, auranofin, an oral, gold-containing agent approved by the FDA in 1985 for the treatment of rheumatoid arthritis, was found to inhibit thioredoxin reductase activity and induce reactive oxygen species (ROS) production, leading to dramatic inhibition of GIST cell growth and viability. Importantly, the anticancer activity associated with auranofin was independent of imatinib-resistant status, but was closely related to the endogenous and inducible levels of ROS. Coupled with the fact that auranofin has an established safety profile in patients, these findings suggest for the first time that auranofin may have clinical benefit for patients with GIST, particularly in those suffering from imatinib-resistant and recurrent forms of this disease.
| S-EPMC3707936 | biostudies-literature
Project description:Gastrointestinal stromal tumors (GIST) are the most common mesenchymal soft tissue sarcoma of the gastrointestinal tract. The management of locally advanced or metastatic unresectable GIST involves detecting KIT, PDGFR, or other molecular alterations targeted by imatinib and other tyrosine kinase inhibitors. The role of immunotherapy in soft tissue sarcomas is growing fast due to multiple clinical and pre-clinical studies with no current standard of care. The potential therapies include cytokine-based therapy, immune checkpoint inhibitors, anti-KIT monoclonal antibodies, bi-specific monoclonal antibodies, and cell-based therapies. Here we provide a comprehensive review of the immunotherapeutic strategies for GIST.
| S-EPMC8306810 | biostudies-literature
Project description:Gastrointestinal stromal tumors (GISTs) are rare entities, which, however, represent the most common mesenchymal tumor of the gastrointestinal tract. The discovery of gain of function mutations on KIT and PDGFRA receptor genes led to a deep revolution in the knowledge of this tumor. This paved the way to the introduction of imatinib and other tyrosine-kinase inhibitors (TKIs), which terrifically revolutionized the prognosis of GIST patients. Currently, it is well established that tumor mutational status is the main player in clinical outcome; however, with the research advances, it has been slowly understood that GIST landscape is more complex than expected and the TKIs available are not effective for all the GIST subtypes. For this reason, in the era of tailored/personalized medicine, each GIST patient should be considered individually and genetic consult should be the first step to take in consideration in the therapeutic decision making process.
| S-EPMC9019173 | biostudies-literature
Project description:Gastrointestinal stromal tumors (GISTs) are the most frequent mesenchymal tumors of the gastrointestinal tract. The discovery that these tumors, formerly thought of smooth muscle origin, are indeed better characterized by specific activating mutation in genes coding for the receptor tyrosine kinases (RTKs) CKIT and PDGFRA and that these mutations are strongly predictive for the response to targeted therapy with RTK inhibitors has made GISTs the typical example of the integration of basic molecular knowledge in the daily clinical activity. The information on the mutational status of these tumors is essential to predict (and subsequently to plan) the therapy. As resistant cases are frequently wild type, other possible oncogenic events, defining other "entities," have been discovered (e.g., succinil dehydrogenase mutation/dysregulation, insuline growth factor expression, and mutations in the RAS-RAF-MAPK pathway). The classification of disease must nowadays rely on the integration of the clinico-morphological characteristics with the molecular data.
| S-EPMC4291900 | biostudies-literature
Project description:Constitutive activating mutations in KIT and platelet-derived growth factor receptor ? ( PDGFR?) are heavily involved in the pathobiology of gastrointestinal stromal tumors (GISTs). This disease has served as an effective "proof-of-concept" model for targeting gain-of-function kinase mutations in cancer. This review discusses the current standard of care in terms of pharmacotherapy in the management of localized and metastatic GISTs.
| S-EPMC5600003 | biostudies-other