Gastrointestinal Stromal Tumor Presenting Research Articles

S3646 Gastrointestinal Stromal Tumors Presenting as Recurrent Gastrointestinal Bleeding

S3646 Gastrointestinal Stromal Tumors Presenting as Recurrent Gastrointestinal Bleeding

Uncommon presentation of duodenal gastrointestinal stromal tumor as large paraduodenal cyst: case report

Uncommon presentation of duodenal gastrointestinal stromal tumor as large paraduodenal cyst: case report

Primary small-bowel gastrointestinal stromal tumor presenting with severe anemia: A report of two cases

Gastrointestinal stromal tumors are rare mesenchymal neoplasms that commonly occur in the stomach and small intestine and can occur anywhere from the esophagus to the anus. We are reporting two cases with identical presentations of severe anemia and abdominal discomfort, which revealed the diagnosis of primary small-bowel gastrointestinal stromal tumour (GIST), on computed tomography (CT), as standard endoscopy investigations failed to reveal the cause for the anemia. Both patients underwent surgical resection for the lesions and histopathological examination confirmed the diagnosis of GIST. After surgical management, hemoglobin levels of both patients improved, and were discharged with uneventful postoperative course. These case reports stress the importance of contrast-enhanced computed tomography (CT) scans, for investigating anemia, along with the standard endoscopy tests.

A rare presentation of jejunal GIST: A case report

Gastrointestinal stromal tumors (GISTs) are rare tumors that constitute 1% of all GI tract tumors. Jejunal GISTs are the rarest subtype. We present a middle-aged gentleman, who presented with pain in the right lower abdomen. On abdominal examination, a lump was palpable in the right iliac fossa (RIF). Contrast-enhanced computed tomography of the abdomen revealed an extraluminal soft-tissue mass in the ileum. Computed Tomography-guided core needle biopsy from the lump was consistent with GIST, which was confirmed on immunohistochemistry. Mutation analysis revealed exon 11 mutations. Due to the proximity of GIST to the rectum and urinary bladder, the patient was started on imatinib therapy. After 3 months of treatment, imatinib therapy had to be stopped due to skin reactions. Restaging was done with a positron-emission tomography scan, which demonstrated a soft-tissue mass likely arising from the ileocecal region in the RIF abutting the ascending colon without any significant decrease in size. On exploration, a well-circumscribed mobile extraluminal lobulated mass was seen arising from the antimesenteric border of the jejunum. Histopathologic examination showed GIST, which was confirmed on immunohistochemistry. We report this case to emphasize keeping small bowel GIST as an unusual differential diagnosis of a RIF lump. Furthermore, not all patients can tolerate imatinib treatment, hence exon mutation study is important, and surgery should be considered if it is deemed resectable.

Clinical and Morphological Characteristics of Gastrointestinal Stromal Tumor.

Introduction: Gastrointestinal stromal tumors (GIST) are a rare form of cancer located within the gastrointestinal (GI) tract, defined as tumors with spindle, epithelioid, or occasionally pleomorphic cells. They originate in the interstitial cells of Cajal, with the function of "pacemaker" of gastrointestinal motility. Their behavior is dictated by changes in the c-kit/PDGFRA gene, which is often highlighted by immunolabeling. Methods: We report the clinical, macroscopic, microscopic, and immunohistochemical characteristics of consecutive patients diagnosed with GIST who underwent surgical removal of the tumor in our department between 2008-2022. Results: We included 20 consecutive patients. The presentation was considered a surgical emergency requiring immediate surgical intervention in most subjects. The most common localization is the small intestine (n=9, 45%), followed by the stomach (n=7, 35%), colon (n=3, 15%), and peritoneum (n=1, n=5%). Histologically, the tumors were predominantly mixed (n=10, 50%) followed by spindle type (n=8, 40%) and epithelioid - 2 cases (10%). Conclusion: The clinical presentation of GISTs remains heterogeneous, and the diagnosis is predominantly postsurgical, using complex immunohistochemistry analysis. The tumor size and number of mitoses are strongly associated with the long-term prognosis.

Gastrointestinal Stromal Tumor (GIST) presenting as acute abdomen: Image in surgery

Gastrointestinal stromal tumors originating from the small bowel are uncommon. We present the case of a 45-year-old man with no prior medical or surgical history who presented to our hospital's emergency department with abdominal pain, vomiting, inability to pass gas, and no bowel movements. Physical examination revealed abdominal distention and tenderness. His white blood cell count was 10.9 x 10^3/µl, and hemoglobin levels were 13.3 g/dl.

Gastrointestinal stromal tumor in North Africa and the middle east: updates in presentation and management from an 11-year retrospective cohort

ABSTRACT Objectives This study described the epidemiological, clinical, and survival profiles of patients with gastrointestinal stromal tumor (GIST) in North Africa and the Middle East (AfME). Methods This regional, multicenter, observational, retrospective study collected 11-year data on demographics, medical history, disease characteristics, current treatment approaches of GIST, the safety of the most common tyrosine kinase inhibitors (TKIs), second cancers, and survival status. Results Data of 201 eligible patients were analyzed: mean age was 56.9 ± 12.6 years; 111 (55.2%) patients were men, 21 (10.4%) patients had previous personal malignancy. The most common clinical presentation of GIST was dysphagia [92 (45.8%) patients]. The stomach was the most common primary site in 120 (60.7%) patients, 171 (85.1%) patients had localized disease at diagnosis. 198 (98.5%) GIST cases were CD117/CD34-positive. Imatinib was used in the neoadjuvant (18/21 patients), adjuvant (85/89 patients), and first-line metastatic treatment (28/33 patients) settings. The most common non-hematological toxicity associated with TKIs was vomiting in 32/85 (37.6%) patients. Overall, 100 (49.8%) patients (95%CI: 42.8–56.7%) were alive and disease-free while 30 (14.9%) patients were alive with active disease. Conclusion Presentation of GIST in our AfME population is consistent with global reports, being more frequent in patients >50 years old and having the stomach as the most common primary site. Unlike what is usually reported, though, we did have more patients with lymphatic spread of the disease. Despite the global trend and advances in the treatment of GIST according to molecular profile, this is still far to happen in our population given the lack of access to molecular profiles and the high associated cost.

Is Bleeding Gastrointestinal Stromal Tumor Associated With Higher MortalityandMorbidity?

Introduction Gastrointestinal stromal tumor (GIST) is the most common primary mesenchymal tumor of the gastrointestinal tract. The clinical presentation of GIST varies widely and ranges from being asymptomatic to being a life-threatening emergency in the form of gastrointestinal bleeding or bowel obstruction. Multiple prognostic factors have been identified for GIST, including, most importantly, larger tumor size (>5 cm), higher mitotic activity (>5 per 50 high-power fields), rupture of the mass, site of the mass, and personal history of GIST. Objective In this paper, we aim to study bleeding in gastrointestinal tumors as a predictor for morbidity and mortality and investigate possible factors influencing bleeding in GIST. Method A retrospective study design was used. Electronic medical records of 39 patients diagnosed with GIST in Prince Sultan Military Medical City in Riyadh between January 2015 and December 2020 were retrieved. Normal variables were presented as mean and standard deviation, while non-normal variables were presented as median and interquartile range. Student t-test or Mann-Whitney test was used to compare quantitative data when appropriate. The distribution of recurrence and survival was plotted with the Kaplan-Meier curveand compared between the two groups (bleeding and non-bleeding groups) with the log-rank test. Factors affecting recurrence and mortality were assessed using univariable Cox regression. Result A total of 39 cases of GIST tumors were included in this study. Patients were categorized according to the presentation into two groups: patients with no bleeding (n= 28) and those presented with bleeding (n= 11). The gender distribution was equal. The mean age was 58.14± 12.46 in patients with non-bleeding GIST and 58.18± 16.01 in patients with bleeding GIST. The most common location of GIST was the stomach (22 cases, 56%). Neither group had any significant differences regarding age (P-value = 0.993), gender (P-value = 0.648), tumor location (P-value = 0.057), size (P-value = 0.250), cluster of differentiation (CD) markers, histological types (P-value = 0.692), and shape (P-value = 0.079). There was no significant difference in recurrence between both groups (log-rank P = 0.972). There was no significant difference in survival between both groups (Log-rank P = 0.506). Conclusion We concluded that bleeding GIST was not a significant predictor for recurrence rate or higher mortality. This can help in the debate of whether bleeding GIST should be added as an independent factor in risk stratification. Despite that, further studies are needed to identify more variables that can add more accuracy to the pre-existing risk stratification systems.

Unusual presentation of a large GIST: a challenging diagnostic dilemma

Introduction: Gastrointestinal stromal tumors (GISTs) are rare neoplasms of the gastrointestinal tract associated withhigh rates of malignant transformation. Most GISTs present asymptomatically. They are best identified by computedtomography (CT) scan and most stain positive for CD 117 (C-Kit), CD34, and/ or DOG-1. There have been many riskstratification classifications systems which are calculated based on tumor size, mitotic rate, location and perforation. Case presentation: A 42 years old man presented with dysphagia and weight loss for 2.5 months. On examination, hewas severely anaemic having a huge intra-abdominal lump. He underwent laparotomy followed by removal of tumormass with partial left lobectomy of the Liver. Conclusion:We present a case of GIST of unusual location and presentation pattern. In general, only completeresection of tumor can lead to cure, although recurrence is common after surgery. Bangladesh Crit Care J March 2023; 11 (1): 54-60

Gastrointestinal Stromal Tumor in Patients Presenting to Khyber Teaching Hospital, Peshawar

Objective: To determine the frequency of presentations of gastrointestinal stromal tumor in patients presenting to Khyber Teaching Hospital, Peshawar. Study Design: Descriptive Cross Sectional Place and Duration of study: Department of General Surgery, MTI-KTH Peshawar. From 1st November, 2020 to 30 April, 2021. Methods: All patients were evaluated for their presentations (Abdominal Pain, Abdominal mass, Vomiting, Anemia, Weight loss and Hematemesis as per operational definition. All this data was noted and recorded on especially designed proforma. Results: As per frequencies and percentages for variable presentation, 42 (59.2%) patients had abdominal pain, 38 (53.5%) patients had abdominal mass, 36 (50.7%) patients had vomiting, 31 (43.7%) patients had anemia, 22 (31.0%) patients had weight loss while 11 (15.5%) patients had hematemesis. Conclusion: Our study demonstrated abdominal pain as the most common presentation of gastrointestinal stromal tumor in our local population as local evidence which was followed by abdominal mass and vomiting. Keywords: Abdominal Pain, Gastrointestinal Stromal Tumor, Vomiting

Small bowel gastrointestinal stromal tumor presenting as a life-threatening emergency

Gastrointestinal stromal tumor (GIST) is a rare stromal neoplasm, which represents the most common mesenchymal tumor of the gastrointestinal tract. It is characterized by indolent clinical symptoms, although it can present as a life-threatening emergency. Here in, we present a case of primary small bowel GIST treated at our department. A 58-year-old male patient presented to our emergency department with a diffuse abdominal pain of acute onset. Patient presented with symptoms and signs of acute intestinal obstruction. Imaging studies revealed a mass at mid ileum, with multiple air fluid levels. Intra-operatively, a mass of 12×9 cm was found at mid ileum on antimesenteric border with 3600 volvulus of adjacent ileum. En bloc resection was performed. Histopathology report was suggestive of gastrointestinal stromal tumor.

A rare case of primary gastrointestinal stromal tumour of the stomach presenting as perforation peritonitis

Presentation of gastrointestinal stromal tumours (GISTs) as perforation peritonitis is a rare entity with only few cases reported in literature. Here we present a case of a 50-year-old male with acute abdomen and signs of peritonitis. Abdominal radiography revealed dilated small bowel loops in the absence of any free intraperitoneal air. Exploratory laparotomy revealed a growth arising from the stomach wall at the distal part of the body along the greater curvature. Wide excision of the lesion was done with rent closure. Histopathological examination further confirmed the diagnosis of a GIST of the stomach. Post operatively patient received Imatinib therapy and is doing well on follow up. Although rare, GISTs should be considered in the differential diagnosis of perforated GI lesions.

False-positive 123I-metaiodobenzylguanidine scintigraphy in a patient with a gastrointestinal stromal tumor presenting as a left adrenal incidentaloma.

A 47-year-old man was diagnosed with a left adrenal incidentaloma at 40 years of age. The tumor had irregular margins and grew from 18 mm to 30 mm in maximum diameter over 7 years. On computed tomography scan, the mass appeared to localize within the tip of the lateral limb of the left adrenal gland, and between the left adrenal gland and the posterior wall of the stomach. The plasma corticotropin and cortisol concentrations and the 24-h urine fractionated metanephrine levels were normal. 123I-metaiodobenzylguanidine scintigraphy showed tumor avidity consistent with a hormonally inactive pheochromocytoma. A laparoscopic left adrenalectomy was performed; however, no tumor was present in the resected specimen. Abdominal computed tomography postoperatively showed that the tumor remained intact and appeared to connect to the posterior wall of the stomach. A laparotomy was performed and the tumor was removed. The tumor was localized to the intraperitoneal space and isolated from the posterior wall of the stomach. The pathological diagnosis was a gastrointestinal stromal tumor. Clinicians need to be aware of the limitations of diagnostic imaging studies in diagnosing non-functioning adrenal incidentalomas, which require a pathological analysis for the final diagnosis. Moreover, clinicians need to provide patients with sufficient informed consent when deciding on treatment strategies. Anatomic structures and tumors that develop in neighboring tissues to the adrenal glands may be confused with primary adrenal tumors. 123I- metaiodobenzylguanidine (MIBG) scintigraphy is specific for diagnosing pheochromocytomas and paragangliomas; however, it has been reported that 123I-MIBG may accumulate in neuroendocrine tumors as well as other tumors. Clinicians should recognize the limitations of imaging studies and the uncertainty of an imaging-based preoperative diagnosis.

Gastrointestinal stromal tumor (GIST) presenting as a multilocular cystic intra-abdominal mass in a dog

BackgroundGastrointestinal stromal tumor (GIST) is a malignant mesenchymal neoplasm described in humans, dogs, and cats. A hallmark of diagnosis for GISTs is positive immunohistochemical labelling with c-Kit (CD117). The differentiation of GIST from other mesenchymal neoplasms of the gastrointestinal tract is pivotal to allow for initiation of appropriate treatment. In humans, cystic GIST has been described, though this has not been reported in dogs. In humans, the cystic form of GIST has been associated with a favorable prognosis. In the present paper, we report a case of multilocular cystic GIST in a dog, which has not previously been described in this species.Case presentationA ten-year-old, male-entire Maltese terrier mix breed dog presented with a large cystic mural mass of the duoedenum and orad jejunum. Histopathology and positive immunohistochemical staining with CD117 confirmed a diagnosis of GIST. No evidence of metastasis was detected on routine staging with abdominal sonography and thoracic radiography at the time of diagnosis. Surgical resection was performed and toceranib therapy was initiated post-operatively. Metastasis was documented 251 days after surgery on computed tomography. Due to clinical deterioration, the patient was humanely euthanised 370 days after surgical excision.ConclusionsThere are few differential diagnoses for large multilocular cystic intra-abdominal masses in dogs. This case presents a previously undescribed presentation of gastrointestinal stromal tumor in the dog as a predominantly multilocular cystic mass. It remains unclear if the cystic form of GIST may represent a favorable prognosis in dogs.

Giant gastrointestinal stromal tumour presenting with small bowel strangulation.

Ileal Gastrointestinal tumor are usually asymptomatic and small bowel obstruction is a rare condition.

S3605 Gastrointestinal Stromal Tumor Presenting as a Fistulizing Cavitary Lesion

Introduction: Gastrointestinal stromal tumors (GISTs) are the most common mesenchymal neoplasms of the stomach. GISTs can vary from small, asymptomatic lesions managed with surveillance to large lesions presenting with life-threatening complications, including gastrointestinal hemorrhage. Complications such cavitary lesions and luminal GI fistulas have been reported but are exceedingly rare. Case Description/Methods: A 65 year-old woman developed mild, constant abdominal pain. Her past medical history includes HTN, GERD, COPD and 50 pack-year smoking history. One month after symptom onset, she was admitted to an outside hospital with upper GI bleed. EGD was reportedly normal at that time. She was readmitted one month later with recurrent anemia. CT scan showed a large gastric diverticulum or possible cavitary lesion. EGD was repeated and showed an opening to a large diverticulum or cavity distal to the gastroesophageal junction with ulcerated cavity wall. Pathology showed ulcerated gastric mucosa and granulation tissue with atypical spindle cells. Biopsies were negative for H. pylori. She was then admitted to our hospital for severe anemia and work-up of suspected GIST. A third EGD showed a 2 cm fistula to a 10 cm necrotic cavity on the fundus and proximal greater curvature of the gastric body. Multiple biopsies of the cavity wall were taken. Biopsies were positive for spindle cell-type GIST (CD117+, CD34+, Cam 5.2-). She then had a feeding jejunostomy placed due to difficulty tolerating PO intake. She was treated with pantoprazole and imatinib and discharged with Medical Oncology and Surgery follow-up for resection. (Figure) Discussion: The most common site of origin for GISTs is the stomach. Radiographic appearance is variable and can include endoluminal, exophytic or dumbbell-shaped pattern and there is frequently necrosis. Other imaging findings include calcifications, cyst formation, cystic degeneration and rarely, cavitary lesions and fistulas. In fact, there are only 2 case reports in the last 10 years of GISTs presenting as a gastric fistula or a gastrobronchial fistula. CT findings including: hemorrhage, necrosis, calcifications, intralesional cavitation, cystic degeneration, size >10cm, irregular margins and heterogeneous enhancement all portend a poor prognosis. Enlarged feeding or draining vessels can predict relapse. Our patient’s case shows that it is important to have a high index of suspicion for diagnosis of GIST, particularly in the setting of atypical presentation.Figure 1.: Upper GI endoscopy image of the interior of the GIST cavity.

S3416 Gastrointestinal Stromal Tumor Presenting as Small Bowel Obstruction in the Setting of Neurofibromatosis Type 1

S3416 Gastrointestinal Stromal Tumor Presenting as Small Bowel Obstruction in the Setting of Neurofibromatosis Type 1

Presentation and Management of Gastrointestinal Stromal Tumour (Gist) Among Sudanese Patients from April 2015 to April 2019

Background: Gastrointestinal stromal tumour is one of the common alimentary tract tumour and it have different anatomical site of occurrence; there are international data that are common to occur in the stomach but no local data about the common site in Sudanese patients and the presentation. Objective: To asses presentation and management of gastrointestinal stromal tumour among Sudanese patients

CASE REPORT ON RARE OUTCOME OF A RETROPERITONEAL MASS: MANAGEMENT CHALLENGES AND LESSONS LEARNT

Background. Gastrointestinal stromal tumours (GIST) are non-epithelial mesenchymal solid neoplasm with varied presentation. The study reports the case of a retroperitoneal GIST in a 21‑year‑old male presented with an abdominal lump for six months. The lesion was initially thought to be a retroperitoneal sarcoma. Exploratory laparotomy revealed an abdominopelvic mass covering the entire right side of abdomen and pelvis. The tumour was adherent to the terminal ileum and ascending colon. There were dense adhesions between the retroperitoneum with involvement of the middle third of the right ureter. The tumour was resected with right hemicolectomy with ileotransverse anastomosis. Post-operative histopathology revealed it as high-grade spindle cell type GIST. The patient is presently on post-operative chemotherapy with Imatinib mesylate. Objective. Atypical presentations of GIST are seldom discussed but frequently encountered in clinical practice. This article depicts different challenges the surgeon has to face while diagnosing such atypical entity. Methods. Case report of atypical GIST presenting as retroperitoneal lump. Results. The patient underwent surgical resection and is presently on post-operative chemotherapy with good overall outcome for a one year follow up. Conclusions. GISTs presenting as retroperitoneal lumps are very rare, they should be considered in their differential diagnosis of an atypical retroperitoneal mass.

Hemothorax due to a ruptured esophageal gastrointestinal stromal tumor: case report

BackgroundEsophageal gastrointestinal stromal tumors (GISTs) are rare tumors of the gastrointestinal interstitium, and comprise less than 0.7% of all GISTs. The presentation of esophageal GIST is relatively benign, commonly characterized by symptoms of dysphagia and gastrointestinal bleed. On the contrary, it is highly unusual for these tumors to present as surgical emergencies.Case presentationHere, we describe a case of hemothorax secondary to the rupture of a massive (19 cm) esophageal GIST in a 79-year-old male. The patient presented with mild back pain, vomiting, and hypotension. A CT scan revealed significant mediastinal enlargement and left hemothorax. We conducted an emergency thoracotomy which revealed a 19 × 15 × 7 cm ruptured esophageal tumor that was bleeding profusely into the left thoracic cavity. Piecemeal resection without esophagectomy was performed to achieve hemostasis. Pathological evaluation of resected tissue confirmed the diagnosis of GIST. The patient was provided adjuvant imatinib therapy and remains progression-free at the 10-month follow-up.ConclusionsTo the best of our knowledge, this is the first reported case of life-threatening hemothorax caused by a ruptured esophageal GIST. Findings from this case may aid in the diagnosis and management of these rare tumors.