Refractory hypoglycaemia in a localised gastrointestinal stromal tumour: Case report

IntroductionGIST and NICTH are mesenchymal in origin however there are very few reports of GIST associated with NICTH which is a para neoplastic syndrome, generally diagnosed when a tumour induced hypoglycaemia is noted.Case presentationA 46 years old female with prime complain of awareness of a mass in the upper abdomen was admitted for evaluation and further management. Detailed investigation revealed the mass to be gastrointestinal stromal tumour. On the day of admission patient was found to be hypoglycaemic which didn't resolve even after 10% glucose infusion. A growth hormone releasing peptide-2 (GHRP-2) assay was carried out which showed an excessive reaction of basal growth hormone however corticotropin releasing hormone (CRH) tests were within normal limits. She was suspected to be Non Islet cell tumour hypoglycaemia (NICTH) and hypoglycaemia resolved upon administering dexamethasone. Later she underwent chemotherapy and surgical resection after which her blood sugar levels were within normal limits.DiscussionExpression of big IGF-II on the surface of GIST be it metastatic or nonmetastatic can cause refractory hypoglycaemia and can be fatal if left untreated.ConclusionClinicians should be aware of refractory hypoglycaemia in patients with large GIST's as glucocorticoid therapy may prove to be extremely useful and lifesaving even before considering any forms of definitive management of the tumour.     

Perisacral gastrointestinal stromal tumor with intracranial metastasis. Case report

A 68-year-old woman presented with an extremely rare intracranial metastasis from a gastrointestinal stromal tumor (GIST) manifesting as left hemiparesis 2 years after resection of a sacral tumor adjacent to the coccygeal bone. Magnetic resonance imaging revealed an intracranial tumor in the right parietal lobe. Craniotomy was performed to completely remove the tumor. Although the tumor was located extra-axially, only internal carotid angiography showed mass staining. Seven months after surgery, the tumor recurred. Repeat craniotomy was performed to remove the recurrent tumor. Immunohistochemical analysis showed that the tumor cells were positive for c-kit and CD34, and the tumors were identified as intracranial metastasis of GIST. Following the second intracranial surgery, the patient developed severe lower back pain caused by metastatic tumor invading the lumbar spine and ureter. To avoid surgical complications and to reduce tumor volume, imatinib mesylate (Gleevec) was administered. The severe pain was relieved, although the tumor was not reduced. In this case, the extra-axial tumor was fed only by the internal carotid artery.     

Varied presentations of gastrointestinal stromal tumour

Gastrointestinal stromal tumours (GIST) are soft tissue tumours arising from the mesenchyma in the gastrointestinal tract. These are rare tumours. However, over the past few years with the better understanding of the pathogenesis of GIST and better imaging facilities, the diagnosis is made more frequently. The characteristic diagnostic feature of GIST is the expression of CD34 and receptor tyrosine kinase KIT, CD117 by these tumours. The use of tyrosine kinase inhibitor imatinib mesylate has led to improved outcome. The presentation of GIST however remains non-specific, and varies depending upon the size and the organ of origin. We present a series of four cases of GIST with varied presentation.     

Intestinal stromal tumors, rare cause of lower gastrointestinal bleeding. Case report

Gastrointestinal stromal tumors represent a heterogeneous group of mesenchymal tumors that can develop throughout the gastrointestinal tract. We present the clinical case of a patient with such malignancies occurred in the proximal jejunum and clinically manifested by a severe digestive hemorrhage with hemorrhagic shock and severe posthemorrhagic anemia. Pre-operative diagnosis was possible only through the Entero-MRI. The evolution was favorable after surgical extirpation. It's being discussed particular aspects of this lesion's entity and post-operative optimal attitude in this case.     

A ruptured gastrointestinal stromal tumour of the transverse mesocolon: a case report

We discuss the long history of a patient still alive with a primary gastrointestinal stromal tumour (GIST) of the transverse mesocolon: initially it presented as an acute complication, namely a haemorrhagic shock caused by rupture of the tumour and it recurred twice locally in the years following primary resection. Each time, a macroscopically complete resection could be achieved. Six years after the resection of the primary tumour, a liver metastasis was discovered, which was removed by radio frequency ablation (RFA). Eight months later, the patient developed a new liver metastasis and a retropancreatic mass deemed unresectable. He is treated with imatinib mesylate (Glyvec, Novartis, Vilvoorde, Belgium) since and has had a stable disease for 6 months. Gastrointestinal stromal tumours outside the gastrointestinal tract are rare. Until a few years ago the only possible therapy was surgical resection. Nowadays radio frequency ablation can be a solution for irresectable liver metastases, and imatinib mesylate can bring improvement for advanced and metastasized tumours. Whether this could also be useful as an adjuvant therapy has not yet been investigated, but it could be important knowing that 38% of the tumours recur locally and 15% metastasize. The most important prognostic factors for gastrointestinal stromal tumours are grading and complete resection.     

Gastric stromal tumor. Case report

Gastrointestinal stromal tumors (GIST) are the most common mesenchymal tumors of the gastro intestinal tract and express the c-kit proto-oncogene protein (CD-117). We report herein a case of a 58 years old male with upper abdominal pain and anaemia. Preoperative endoscopic and barium meal exams showed a submucosal tumor in the gastric fornix. Endoscopic biopsy revealed spindle-shaped cells with elongated nuclei specific of GIST. We performed a stapled resection of the gastric fornix associated with Hiss angle reconstruction. Postoperative histologic exam revealed the same spindle-shape cells characteristic of GIST with 2-5 mitosis/50HPF. Almost all of the cells showed immunoreactivity for c-kit, CD-34 and a Ki-67 labeling index of 30%. Postoperative course was non eventful and after 3 month the patient has no recurrence. CONCLUSIONS: GIST are rare gastric tumors and are usually associated with bleeding and abdominal pain. The preoperative diagnosis is usually established by endoscopy with biopsy or endoscopic ultrasonography fine needle aspiration and barium meal exam. Only immunoreactivity is able to differentiate GIST from other mesenchymal tumors. Surgical resection is a safe and effective treatment. The chemotherapy with tyrosine kinases competitive inhibitors (e.g. imatinib mesylate) is also recommended.     

Late recurrent adrenocortical carcinoma presenting radiologically as a gastrointestinal stromal tumour: A case report

Introduction

Adrenocortical carcinoma (ACC) is a rare malignancy with an estimated incidence of 1–2 per million people. It may recur, after complete surgical removal by local or distant metastasis.

Mucinous cystadenocarcinoma of pancreas mimicking gastrointestinal stromal tumor of stomach: Case report

IntroductionPancreatic mucinous cysadenocarcinoma is a rare neoplasm of pancreas which rarely presents as upper gastrointestinal bleed.Case reportA 45-year-old woman presented with history of repeated episodes of melena and abdominal pain. Upper gastrointestinal endoscopy revealed a growth in the body of the stomach likely to be a gastrointestinal stromal tumor (GIST). Endoscopic biopsy was inconclusive for GIST or malignancy. Computed tomography scan of the abdomen showed a well-defined multiloculated cystic lesion (11.8 × 11.1 × 6.4 cm) in the body and tail of the pancreas with involvement of the stomach. Preoperative diagnosis was a cystic neoplasm of the pancreas with tumor ingrowth into the stomach. The patient underwent enbloc resection of the stomach with distal pancreatectomy and splenectomy. Histopathological examination revealed low grade mucinous cystadenocarcinoma of the pancreas.DiscussionUpper gastrointestinal bleed as a manifestation of cystic neoplasms of pancreas are rarely reported. Although some cases of hemoperitoneum were reported due to rupture of tumor; luminal gastrointestinal bleed is even rarer. It may be due to involvement of porto-mesenteric axis with portal hypertension, direct invasion of the gastric wall with ulceration of the gastric mucosa, communication between the tumor and the main pancreatic duct, and from the infiltrating tumor.ConclusionOur case highlights the fact that mucinous cystadenocarcinoma of pancreas rarely can present with upper gastrointestinal hemorrhage, Moreover, if it is large enough to compress or invade the stomach, endoscopy may misdiagnose it as GIST.     

Surgery for gastrointestinal stromal tumour in the post-imatinib era

Gastrointestinal stromal tumour (GIST) is a rare tumour. Historically, surgery has been the only effective treatment. The prognosis of patients with gastrointestinal stromal tumour is poor. Even after apparently 'curative' surgical resection more than 50% of patients relapse. The development of an effective novel targeted therapy against GIST (imatinib mesylate) is a success story of molecular biology that has dramatically altered the management of patients with these tumours. However, as follow up of patients who have initially responded to imatinib has increased, it has become evident that such hopes of cure were premature because responses to imatinib are of limited duration. Unresolved issues include the role of imatinib as an induction (neo-adjuvant) therapy prior to surgery, or as adjuvant treatment after surgery, the role of surgery in patients with a differential or partial response and the role of surgery in patients with isolated metastatic disease. In the present paper the biology and natural history of GIST are reviewed, and the complexities of surgical management that exist in the context of an effective, but not curative, biological therapy, are addressed.     

Laparoscopic wedge resection for gastrointestinal stromal tumour of the duodenum

We report the case of a 40-year-old male patient who presented with melaena and acute anaemia. Endoscopic ultrasound examination revealed a lesion with a central depression measuring 2.5 cm, arising from the lateral wall of the second portion of the duodenum. Because of this rare location, a very invasive procedure (duodenopancreatectomy) might have been required for tumour resection. We avoided this operation and implemented an alternative solution. A laparoscopic wedge resection of the duodenal tumour was successfully completed. Operating time was 200 min and blood loss 50 ml. The patient was discharged on postoperative day 3, after an uneventful postoperative recovery. Histopathologically, the tumour was diagnosed as a low-risk gastrointestinal stromal tumour. Despite the fact that laparoscopic surgery requires more complex technique than open surgery and the acquisition of advanced laparoscopic skills depending on prior laparoscopic experience, laparoscopic wedge resection should be considered as a valid treatment for duodenal stromal tumour.