Gastrointestinal Stromal Tumor Presenting Research Articles

An Extremely Rare Calcified Pancreatic Head Mass Causing Painless Jaundice: Get the GIST of It

Purpose: This is a case report of an atypical presentation of a very rare disease. A 45-year-old male reported 2 months history of itching, progressive jaundice, and 15-pound weight loss. He had no abdominal pain or gastrointestinal bleeding. His labs showed normal hemoglobin, total bilirubin of 8.5 mg/dL, and a carbohydrate antigen 19.9 (CA 19-9) of 25 units/mL. Abdominal CT showed a 5.3-cm calcified mass in the pancreatic head causing obstruction of the intrapancreatic common bile duct (CBD) with proximal dilation. The pancreatic duct was normal. The mass showed peripheral hyperenhancement. Retrograde cholangiography revealed a malignant appearing distal CBD stricture; brushings for cytology were negative. Endoscopic ultrasound (EUS) guided fine needle biopsy showed clusters of spindle cells. Immunohistochemistry was positive for CD117, and negative for CD34, desmin, and S100, consistent with gastrointestinal stromal tumor (GIST). There was no evidence of metastasis on EUS or PET-CT. He underwent a modified pancreaticoduodenectomy, which confirmed a GIST arising from the duodenal wall 1 cm away from the major papilla, obstructing the CBD and abutting the pancreatic parenchyma without invasion into the pancreas. Final pathology confirmed a low grade GIST; the mitotic rate was less than 1 mitosis per 50 high power fields. GIST calcification is rare before treatment. Duodenal GIST causing obstructive jaundice is uncommon, with two case reports of major papilla GISTs and two case reports of patients with neurofibromatosis-1. The largest series of duodenal GIST reported 22 patients and none had jaundice. Our patient did not have neurofibromatosis. His duodenal GIST did not involve the major papilla. It compressed the common bile duct and caused jaundice. It did not involve the pancreatic parenchyma and did not cause pancreatic ductal dilation, a finding that may be suggestive of an alternate diagnosis to pancreatic adenocarcinoma, which often is ductal in origin and causes pancreatic ductal obstruction and upstream dilation. Duodenal GISTs represent less than 5% of gastrointestinal GISTs and 30% of primary duodenal cancers. The majority arise from the second portion of the duodenum, similar to our patient. The aim of surgery is curative resection; in our patient a modified Whipple accomplished clear surgical margins. Mitotic activity is the most important prognostic factor. Prognosis is significantly better than in pancreatic cancer, with over 85% survival at 3 years. This is the first report of a calcified duodenal gastrointestinal stromal tumor presenting as a pancreatic head mass causing painless jaundice. Both calcifications and jaundice are very unusual features of this rare disease.

Stomach GIST Presenting as a Liver Abscess

Gastrointestinal stromal tumor (GIST) is the most common nonepithelial neoplasm of the gastrointestinal (GI) tract, constituting only 1 % of primary GI malignancies [1–4]. GIST is not restricted to the GI tract and may have extra gastrointestinal involvement [1]. Common primary sites include the stomach (50–70%); small intestine (30–45%); colon, rectum, and anus (10–15 %); mesentery and peritoneum (<10 %); and the esophagus (<5 %) [5–7]. Initially presenting as a liver abscess and Streptococcus intermedius bacteremia, we describe a rare case of GIST which also exhibited synchronous primary rectal and prostate adenocarcinoma tumors.

Multiple Liver Abscess Formation and Primary Gastrointestinal Stromal Tumor

Gastrointestinal stromal tumors are the most common mesenchymal tumors of the gastrointestinal tract. The introduction of a number of small molecule tyrosine kinase inhibitors has revolutionized the management of metastatic disease. Surgery is the mainstay of management for localized disease. Patients with high risk tumors are treated with adjuvant imatinib. We report the rare presentation of a localized primary small bowel gastrointestinal stromal tumor in association with multiple liver abscesses. Cystic liver lesions should be fully evaluated in gastro intestinal tumor patients to exclude an infective cause. Treatment with intravenous antibiotics resulted in clinical and radiological improvement of the liver abscesses. The small bowel tumor was treated with surgical resection.

Gastrointestinal Stromal Tumor of the Tongue

Objective: To report a case of a gastrointestinal stromal tumor presenting as a recurrent tongue mass&#x0D; Methods:&#x0D; Study Design: Case Report&#x0D; Setting: Tertiary Public Hospital&#x0D; Participant: One patient&#x0D; Results: A 40-year-old Filipino soldier from Camarines Sur presented with a recurrent tongue mass two years after excision of a progressively-enlarging, firm, fixed, non-tender mass at the left posterior third of the tongue diagnosed as a Schwannoma. Repeat surgery involved partial glossectomy via midline mandibular swing. Final histopathologic report after immunohistochemical studies for CD117, SMA, and S100 was extraintestinal gastrointestinal stromal tumor of the tongue. The patient was started on the c-kit tyrosine kinase inhibitor Imatinib with no recurrence 10 months post treatment.&#x0D; Conclusion: Gastrointestinal stromal tumor may be considered when presented with a recurrent tongue mass despite complete surgical resection. Surgical removal is curative for most lesions. Post-operative chemotherapy with the use of Imatinib is valuable.&#x0D; Keywords: recurrent tongue mass, gastrointestinal stromal tumor, immunohistochemistry, c-kit tyrosine kinase inhibitor

A rare presentation of gastrointestinal stromal tumors as small bowel perforation: A single institution based clinical experience of three cases

Gastrointestinal stromal tumors (GIST) of intestine presenting as acute abdomen due to perforation of the tumor is extremely rare.We present three cases of jejunal GIST which presented as acute abdomen resulting from perforation of the tumor. All the three cases underwent emergency laparotomy and resection of the tumor with clear margin. Histopathological examination was compatible with GIST and c-Kit/CD 117 was positive in immunohistochemistry. All the three patients received adjuvant chemotherapy with imatinib.

Duodenal GIST presenting as large pancreatic head mass: an uncommon presentation.

Duodenal gastrointestinal stromal tumors (GIST) are uncommon. They usually present with gastrointestinal bleed, upper abdominal pain or mass abdomen. Tumor arising from the second part of duodenum can be wrongly diagnosed as pancreatic mass.We present a case of a thirty-year-old male who came with chief complaint of mass upper abdomen. On laparotomy there was a 15x10 cm mass arising from the whole of anterior surface head of pancreas and was attached with the second part of duodenum for about 1-2 cm only. Patient underwent pancreaticoduodenectomy and histopathology revealed that tumor was arising from the duodenal wall.Duodenal gastrointestinal stromal tumors can grow exophytically into large mass and involve the pancreas without infiltrating microscopically and present as pancreatic head mass.

An unusual case of duodenal beaking

A 35-year-old female presented with complaints of several episodes of malaena and frank blood in the stools for the past 1 year. The frequency of these episodes had increased in the past 2 months. The patient complained of extreme weakness and palpitations. There was no history of abdominal pain, nausea, vomiting or haematemesis. There was no history of fever, jaundice, weight loss or constipation. Physical examination was unremarkable except for the presence of severe pallor. Digital rectal examination revealed black tarry stools staining the glove. There was no palpable growth; first degree haemorrhoids were visible at the 3 o'clock position; no active bleeding was discernible. Her haemoglobin was 4 g dl−1, packed cell volume 12.5%, total leukocyte count 7900 cells mm−3and platelet count 5.34×105 mm−3. Peripheral smear showed features of anaemia. Total serum bilirubin was 2.5 mg dl−1 (direct bilirubin 1.2 mg dl−1). The rest of the liver function tests were within normal limits. HBsAg (the surface antigen of the hepatitis B virus) was negative and human immunodeficiency virus was non-reactive. A chest radiograph demonstrated mild cardiomegaly and the lung fields were clear. Colonoscopy was within normal limits. An upper gastrointenstinal endoscopy was performed, which showed a normal stomach and first part of duodenum. The endoscope could not be negotiated into the third part of the duodenum. A barium meal examination was done for further evaluation of the upper gastrointestinal tract. Two radiographs of an upper gastrointestinal barium series are shown (Figure 1). Figure 1 (a) Barium meal upper gatsrointestinal tract: smooth narrowing or “beaking” at the junction of the second and third parts of the duodenum, with mucosal folds running parallel to the lumen and a small C-loop of the duodenum. (b) Barium ...

A Gastrointestinal Stromal Tumor (GIST) Presenting as a Dieulafoyʼs Lesion

Purpose: An 81-year-old female presented with melena and lightheadedness. She was recently discharged 4 days prior with similar symptoms. Endoscopic gastroduodenoscopy (EGD) on prior admission found a 5 mm clean based gastric ulcer with no stigmata of recent bleed. Pulse was 112 otherwise vitals were stable. Hemoglobin (Hgb) was 5.5 g/dl (9 g/dl on recent discharge). After transfusion, repeat EGD now revealed a large amount of fresh blood in the stomach. The previously visualized gastric ulcer was again without stigmata of bleeding. An active bleeding site could not be found despite thorough EGD examination. Emergent arterial angiography revealed aneurysmal changes in the splenic and short gastric arteries, with no early venous return which is the angiographic definition of a Dieulafoy's lesion. A gastric mucosal blush along the greater curvature near the fundus was noted. No active bleeding was seen but the aneurysms were embolized. Her Hgb continued to decline so computed tomography angiography (CTA) of her abdomen was done which revealed a 6.8 cm hypovascular eroding mass possibly arising from the left adrenal gland into the gastric fundic region (Figure A). Repeat EGD identified in this area a nipple-like protuberance without ulceration, consistent with a Dieulafoy's lesion. The lesion was clipped to provide hemostasis. Endoscopic ultrasound (EUS) of this area revealed a large mass near the left adrenal gland with both cystic and solid components. Needle biopsy results revealed spindle cell neoplasm consistent with a GIST. The patient subsequently underwent exploratory laparotomy revealing a 7.8 cm exophytic submucosal lesion arising from the greater curve of the stomach near the gastroesophageal junction which was resected with clean margins. Pathology again confirmed the diagnosis of a GIST and was positive for KIT (CD117). The patient was started on imatinib therapy after discharge and was doing well on subsequent follow-up. We demonstrate in this case report the diagnostic and therapeutic challenges of this rare scenario, a GIST presenting as a Dieulafoy's lesion.Figure: (A) CTA with lobular mass invading the posterior wall of the stomach.

Surgical second-look in high risk gastrointestinal stromal tumor of small intestine: A case report

INTRODUCTIONThe peritoneum is one of the most common sites of distant gastrointestinal stromal tumor (GIST) metastases. In particular, GIST arising from the small intestine with resected minimal synchronous macroscopic peritoneal carcinomatosis or with primary tumor rupture has a higher risk of developing peritoneal recurrence. Current clinical practice does not envisage second-look surgery in GIST patients at high risk of developing peritoneal recurrence, and no literature data are available. PRESENTATION OF CASEWe describe a 45-year-old woman who underwent emergency surgical resection of jejunal GIST presenting with spontaneous tumor rupture, synchronous ovarian and minimal macroscopic peritoneal involvement, and subsequent second-look surgery after 13 months of imatinib treatment. DISCUSSIONSecond-look surgery confirmed a 2.6cm lesion close to the mesenteric border of the fourth jejunal loop, and 11 peritoneal lesions with a macroscopic necrotic aspect related to treatment response. After conversion to an open procedure, a segmental jejunal resection was performed with removal of all peritoneal lesions and macroscopic radical cytoreduction. CONCLUSIONSecond-look surgery in selected GIST patients may be performed after at least 12 months of medical treatment with tyrosine-kinase inhibitors to identify those patients with limited peritoneal disease not disclosed by instrumental imaging who could undergo radical cytoreduction of peritoneal lesions.

Small duodenal gastrointestinal stromal tumor presenting with acute bleeding misdiagnosed as hemobilia: Two case reports

Only 3-5% of gastrointestinal stromal tumors (GISTs) are located in the duodenum. They are associated with an increased risk of fatal gastrointestinal bleeding, which is a primary manifestation. A small GIST (less than 2 cm in size) is easily confused with a duodenal papilla. In the 2 cases presented in this study, endoscopic examination alone resulted in a misdiagnoses of hemobilia. To the best of our knowledge, this is the first case report of this type in the literature. Patient 1 is a 66-year-old male who was admitted to The Second Affiliated Hospital of Dalian Medical University with syncope. Emergent upper gastrointestinal endoscopy identified bleeding from what appeared to be a duodenal papilla, and the patient was diagnosed with hemobilia. However, the angiography did not support this result. To save the patient's life, an exploratory laparotomy was conducted. We identified a small tumor in the descending part of the duodenum and a wedge resection was successfully conducted. The final diagnosis was duodenal GIST with no further risk to the patient. Patient 2 is a 71-year-old female who was admitted to the hospital diagnosed with hemobilia. The patient underwent a barium swallow examination and a contrast-enhanced computed tomography (CT) scan, in which a tumor on the descending part of the duodenum was identified. Patient 2 also underwent a wedge resection. The final diagnosis was duodenal GIST with no further risk to the patient. Gastroscopy may be a useful procedure for duodenal GIST diagnosis; however, the endoscopic findings may be confused with hemobilia when the tumor diameter is less than 2 cm in size and heavily accompanied with blood clots. A barium swallow examination and contrast-enhanced CT may prevent a misdiagnosis of hemobilia.

Multiple Primary Gastrointestinal Stromal Tumors Presenting in Jejunum and Ileum

Multiple primary gastrointestinal stromal tumors (GIST) are an infrequent finding. Benign and malignant tumors could coexist in the same patient. We discuss one case of a benign jejunal GIST and a malignant ileal GIST coexisting in the same patient and present their radiological characteristics.

Atypical Presentation of Gastrointestinal Stromal Tumours—A Case Report

Gastrointestinal stromal tumors (GISTs) are benign mesenchymal tumors of the gastrointestinal tract (GIT). Their clinical presentations are variable. We report a case of a 31-year-old man who presented with pain in the abdomen and vomiting. CT abdomen revealed a large exophytic mass in the epigastrium with enhancement pattern similar to hemangioma. No relationship of the mass could be made out with the adjacent structures on CT, histopathology proved it to be a GIST.

A rare case of gastrointestinal stromal tumor presenting with closed perforation of the small intestine

Gastrointestinal stromal tumors are very rare mesenchymal tumors of the gastrointestinal tract with variable clinical presentations depending on the tumor size and anatomic site. The currently used diagnostic modalities cannot establish a preoperative diagnosis with 100% certainty. The treatment of choice includes complete surgical excision and/or chemotherapy. In this paper, we present a very rare case of gastrointestinal stromal tumor presenting with closed perforation of the small intestine. The patient was successfully managed with surgical excision of the tumor with segmental bowel resection and adjuvant chemotherapy. Although rare, it must be kept in mind that gastrointestinal stromal tumors can also present with closed bowel perforation.

Treatment Guidelines for Gastrointestinal Stromal Tumors in Children and Young Adults

Gastrointestinal stromal tumor (GIST) occurring in the pediatric population has a unique biology and natural history when compared to GIST occurring in adults. As a result of these unique features, management of GIST in children may need to differ from management of GIST in adults. GIST is a very rare disease in children. Consequently prospective clinical trials have not been performed. A review of the biology, clinical presentation and natural history of GIST in children is presented. Guidelines for diagnostic evaluation and management of GIST in children are presented. The presented guidelines are based on clinical experience and data from case reports and case series.

Gastrointestinal Stromal Tumor Arising in the Rectovaginal Septum

To describe a rare case of gastrointestinal stromal tumor (GIST) outside the gastrointestinal tract and arising in the rectovaginal septum with literature review. This study involved a 29-year-old woman, gravida 0, with a GIST presenting as an asymptomatic tumor in the rectovaginal septum. The tumor was excised locally, and the patient received imatinib as adjuvant therapy. She is free of disease 2 years after surgery. To our knowledge, only 5 cases of GIST presenting as rectovaginal mass have been reported previously. Local excision is the definitive treatment and the use of imatinib shows promising results. Mitotic activity, cellularity, and necrosis are the main prognostic factors. Long-term follow-up is necessary.Different physicians involved in the evaluation of a mass arising in the rectovaginal septum should be alert of GIST as differential diagnosis, even in young women.

Gastrointestinal stromal tumor presenting with prominent calcification

We present a rare case of a gastrointestinal stromal tumor (GIST) in the stomach with prominent calcification at presentation. A 61-year-old woman visited our hospital because of epigastric discomfort. A spherical calcified lesion with a diameter of about 30 mm was incidentally shown in the left upper quadrant on an abdominal X-ray. Computed tomography demonstrated that the tumor was growing from the upper gastric body, with calcification in the peripheral ring area. A laparoscopic partial gastrectomy was performed, and the resected specimen revealed a well-circumscribed tumor with exophytic growth from the gastric muscularis propria. Microscopic examination revealed spindle-shaped tumor cells with calcification and hemorrhage. Additionally, positive immunoreactivity of the tumor to KIT and CD34 and a low mitotic index resulted in the diagnosis of very low risk GIST. There are a few case reports of heavily calcified GIST, although solitary or punctate calcification of primary GIST has been reported in several case series. Dystrophic calcification of necrotic or degenerative tissue is the supposed cause of primary calcified GISTs. In contrast, appearance of calcification after administration of imatinib mesylate, which may be one indicator of disease response, is possibly caused by a different mechanism.

Gastrointestinal stromal tumour of the rectum: a review of surgical treatment, outcomes and the role of imatinib

s / International Journal of Surgery 10 (2012) S1–S52 S24 ABSTRACTS 0269: GASTROINTESTINAL STROMAL TUMOUR OF THE RECTUM: A REVIEW OF SURGICAL TREATMENT, OUTCOMES AND THE ROLE OF IMATINIB M.J. Wilkinson, J.E.F. Fitzgerald, D.C. Strauss, A.J. Hayes, J.M. Thomas. The Royal Marsden Hospital NHS Foundation Trust, London, UK Aims: Gastrointestinal stromal tumours (GISTs) of the rectum are rare, accounting for only 0.1% of all rectal tumours. This study investigates the presentation, management and outcomes of rectal GISTs at a specialist unit. Methods: Retrospective cohort study analysing a prospectively maintained database at a tertiary referral centre from Jan 2001 Jan 2012. Results: A total of 14 patients (6 female, 8 male), presented with a primary rectal GIST. Commonest presenting symptoms were rectal bleeding (n1⁄46) and tenesmus (n1⁄46). Median tumour size at presentation: 8cm (range 2 12cm). 12 patients received neoadjuvant imatinib; median reduction in tumour size 2.8cm (range 0.5 5.6cm); p 1⁄4 0.001. Surgical resection was performed in 6 of the 14 patients (2 patients declined surgery and 6 are continuing imatinib to downsize). Complete macroscopic clearance was obtained in 100% of patients. On follow up, 12 patients are alive without metastases: median follow-up 31.3 months. There were 2 deaths from unrelated causes. The remaining 5 patients operated on are disease free (median DFS 1⁄4 36.2 months). Conclusions: Biopsy is essential in establishing the diagnosis. Neoadjuvant imatinib substantially downsizes rectal GISTS which may permit less invasive surgery. Favourable outcomes can be achieved for rectal GISTs in specialist centres. 0283: A PROPOSED STANDARD FOR PRE-OPERATIVE LAPAROSCOPIC COLORECTAL CANCER RESECTION ENDOSCOPIC TATTOOING. IDENTIFICATION OF MODIFIABLE PRACTICES AT AN ENHANCED RECOVERY CANCER CENTRE Ajay Sud, Arkeliana Tase, Elinor Baker, Santanu Bhattacharjee, Shiva Dindyal, Stefano Andreani. Whipps Cross University Hospital, London, UK Aims: The National Bowel Cancer Screening Program specifies a 100% target for tattooing of suspected malignant lesions. There remains no all-inclusive guideline for colorectal tattooing. We aim to identify factors contributing to suboptimal practice. Methods: The data collected incorporated retrospective analysis of all 144 colorectal surgery patients at Whipps Cross Hospital whom underwent oncological colorectal resections for tenmonths from January 2008 and six months from June 2010. Results: In 2008 and 2010, 39% and 52% respectively, of our patients received pre-operative tattooing. In 2008 and 2010, 30% and 50% respectively of lesions were only documented to be distally tattooed. The mean number of days between their pre-operative endoscopy to surgery in 2010 was 69 days. In 2008 consultant gastroenterologists tattooed 70% of suspect lesions, but by 2010 this reduced to 36%. Only 40%were underwent solely distal tattooing, and 22% of ulcerating lesions were tattooed. Conclusions: Surgeons are the direct recipients of suboptimal tattooing. They are best placed to lead the colonoscopy community to ensure efficacious tattooing practices, enabling optimal uncomplicated oncological resection. The standard for practice should be a recent distal ‘360-degree' tattoo with one vial per 30 degrees, to all suspicious lesions, irrespective to the endoscopic morphology. 0316: IROBOT INITIALIZING A ROBOTIC COLORECTAL SERVICE Faira Eldriana Rizal, Benjamin Stubbs, P. Mathur, Colin Elton, Daren Francis. Department of Coloproctology, Barnet and Chase Farm Hospital,

Laparoscopic resection of gastric gastrointestinal stromal tumors presenting as left adrenal tumors

Gastrointestinal stromal tumors (GISTs) are rare gastrointestinal malignancies. They are rarely seen near the urinary tract. In a literature review, only one case of GIST presenting as a left adrenal tumor was reported. We report two documented cases of gastric GISTs mimicking left adrenal tumors which were successfully treated with pure laparoscopic adrenalectomy and wedge resection of the stomach by excising the tumor from the stomach with serial firing of endoscopic gastrointestinal staplers. The surgical margins were clear, and the patients recovered smoothly. No adjuvant therapy with imatinib was prescribed. During the surveillance for 9 mo and 44 mo respectively, no tumor recurrence and metastasis were documented. Laparoscopic tumor excision, when adhering to the principles of surgical oncology, seems feasible and the prognosis is favorable for such tumors.

A Gastrointestinal Stromal Tumor Presenting as an Emergency: a Case Report

A Gastrointestinal Stromal Tumor Presenting as an Emergency: a Case Report

A Rare Case of Gastrointestinal Stromal Tumor Presenting as a Large Retroperitoneal Abscess Communicating with the Gastric Lumen

A Rare Case of Gastrointestinal Stromal Tumor Presenting as a Large Retroperitoneal Abscess Communicating with the Gastric Lumen