Hypercalcaemia in gastrointestinal stromal tumour and sarcoidosis: a case report.

BACKGROUND: Hypercalcaemia is a common manifestation of sarcoidosis but is sparingly described in gastrointestinal stromal tumours (GISTs). We describe a case of acute kidney injury and hypercalcemia resulting from simultaneous diagnosis of GIST and sarcoidosis, the presentation of which has not yet been reported. CASE PRESENTATION: A 61-year-old male presented with acute kidney injury and hypercalcemia, with elevated 1,25-dihydroxyvitamin D levels. Investigations demonstrated a large gastric antral mass which was resected and proven to be GIST. Histopathology of incidentally found liver nodules revealed non-necrotising epithelioid granulomas consistent with concomitant sarcoidosis. The hypercalcemia was successfully treated with bisphosphonate therapy, resection of the GIST and a four month course of corticosteroids, which was truncated due to a mycobacterial infection. CONCLUSIONS: Our case report is the first to describe hypercalcemia due to GIST and biopsy-proven sarcoidosis, thereby raising the possibility of a common pathophysiological pathway relating the two entities. We review the literature describing the mechanisms of hypercalcaemia in GIST and the association between GIST and sarcoidosis.

Gastrointestinal stromal tumor of the duodenum presenting with shock and massive upper and lower gastrointestinal bleeding: a case report and review of the literature.

BACKGROUND: Due to rarity of duodenal GISTs, clinicians have few information about its clinical features, diagnosis, management and prognosis. CASE REPORT: We report a case of promptly diagnosed duodenal GIST in a 61-year-old Egyptian man presented shocked with severe attack of hematemesis and melena. Upper gastroduodenal endoscopy was done and revealed a large ulcerating bleeding mass at first part of duodenum 4 hemo-clips were applied with good hemostasis. An exploratory laparotomy and distal gastrectomy, duodenectomy and gastrojejunostomy were performed. The morphology of the mass combined with immunohistochemistry was consistent with duodenal gastrointestinal stromal tumours (GISTs) of high risk type. The patient is on amatinib one tablet daily and he was well with no evidence of tumor recurrence. CONCLUSION: despite being rare, emergency presentation with sudden severe, life-threatening hemorrhagic shock duodenal GISTs might be a cause of potentially lethal massive combined upper and lower gastrointestinal bleeding which is the key feature of this rare and challenging tumor.

Adult Female Patient with Untreated Truncus Arteriosus Type I and Torsion of Small Bowel Gastrointestinal Stromal Tumor: A Rare Case Report.

BACKGROUND Persistent truncus arteriosus is a rare congenital cyanotic heart defect characterized by a single ventricular outflow tract. Without surgical intervention, it has a poor prognosis in infancy. Here, we report an adult female patient with uncorrected truncus arteriosus type I, who presented with acute-onset abdominal pain due to torsion of a small bowel gastrointestinal stromal tumor (GIST). CASE REPORT A 41-year-old woman came to our Emergency Department with acute-onset lower abdominal pain for 2 days. Congenital heart disease, truncus arteriosus, had been diagnosed at birth, and there had been no surgical intervention. Abdominal computed tomography revealed a 10×9×12-cm mixed-density mass in the pelvic capacity. Transthoracic echocardiography revealed a 33-mm ventricular septal defect. The ascending aorta originated mainly from the right ventricle, and the pulmonary artery originated from the beginning of the aorta (type I truncus arteriosus, according to Collett and Edwards classification). After a quick and detailed preoperative workup, the patient underwent tumor resection by open surgery with general anesthesia. CONCLUSIONS This is the first case to report emergency surgery for a patient with uncorrected persistent truncus arteriosus due to torsion of a small bowel GIST. A multidisciplinary team with deep understanding of the disease entity was crucial. By considering the fixed hemodynamic and respiratory physiology, overtreatment and unrealistic goals were avoided. Eventually, the patient was discharged after being hospitalized for 2 weeks.

Jejunojejunal intussusception induced by a gastrointestinal stromal tumor: a case report and literature review.

Intussusception is defined as the invagination of a proximal segment of the bowel into the adjoining or distal segment. In most adults with intussusception, there is a demonstrable lead point with a definite pathologic abnormality. The clinical features of intussusception include chronic intermittent abdominal pain, nausea and vomiting, constipation, and a palpable abdominal mass. The present case report describes a 62-year-old woman with a 2-week history of abdominal pain and 9-day history of vomiting. Clinical, imaging, and histologic evaluations revealed a jejunojejunal intussusception with a gastrointestinal stromal tumor as the lead point. A gastrointestinal stromal tumor should be considered as a possible lead point in adult patients with intussusception. The implication of reducing the intussusception prior to tumor resection requires further evaluation in view of the risk of venous embolism, including direct spread of malignant cells, in cases involving a large polypoid mass with a necrotic surface that extends to the serosa as shown by intraoperative examination. Accordingly, the rationale for adjuvant therapy with imatinib also requires further evaluation.

Incidental Bleeding Gastrointestinal Stromal Tumor Identified on 99m Tc-RBC Scintigraphy.

ABSTRACT: Various pathologies could lead to occult gastrointestinal (GI) bleeding. Here we report the case of a 73-year-old woman who presented with hematochezia and syncope, and was found to have a large bleeding GI stromal tumor incidentally from 99m Tc-RBC scintigraphy. This study was done after negative workup with CT angiography, colonoscopy, and capsule endoscopy for the source of GI bleeding. Final pathology confirmed the mass being a low-grade GI stromal tumor after exploratory laparotomy.

Colonic gastrointestinal stromal tumor (GIST) presenting with colocolonic intussusception: A rare case report.

An 8-year-old spayed female British bulldog was presented with vomiting, hyporexia, and large-bowel diarrhea. Abdominal ultrasound revealed a focal colonic mass with an intussusception located immediately oral to the mass. The intussusception encompassed the ascending and transverse colon and was non-reducible. Colonic resection and anastomosis were completed to include the intussusception and colonic mass. Histopathological examination of the mass demonstrated a spindle cell neoplasm arising within the muscular wall of the intussuscepted segment that obliterated normal architecture. Mild-to-moderate cytoplasmic immunoreactivity of the tumor cell population for CD117 and smooth muscle actin was consistent with a diagnosis of a gastrointestinal stromal tumor. The dog described herein remains alive and free of progressive disease at the time of writing. Key clinical message: The entire gastrointestinal tract should be evaluated in any animal with gastrointestinal symptoms. A gastrointestinal stromal tumor remains a plausible differential diagnosis, regardless of the intestinal segment affected, and tumorassociated intussusception is a rare but urgent clinical finding.

Tumeur stromale gastro-intestinale du côlon (GIST) présentant une invagination colocolique : un rapport de cas rare. Une femelle bouledogue anglais stérilisée de 8 ans a présenté des vomissements, une hyporexie et une diarrhée d'origine du gros intestin. L'échographie abdominale a révélé une masse colique focale avec une invagination située immédiatement oralement à la masse. L'intussusception englobait le côlon ascendant et transverse et était non réductible. La résection colique et l'anastomose ont été réalisées pour inclure l'intussusception et la masse colique. L'examen histopathologique de la masse a révélé un néoplasme à cellules fusiformes apparaissant dans la paroi musculaire du segment invaginé qui a oblitéré l'architecture normale. L'immunoréactivité cytoplasmique légère à modérée de la population de cellules tumorales pour le CD117 et l'actine des muscles lisses étaient compatibles avec un diagnostic de tumeur stromale gastro-intestinale. Le chien décrit ici est toujours vivant et exempt de maladie évolutive au moment de la rédaction.Message clinique clé :L'ensemble du tractus gastro-intestinal doit être évalué chez tout animal présentant des symptômes gastrointestinaux. Une tumeur stromale gastro-intestinale reste un diagnostic différentiel plausible, quel que soit le segment intestinal atteint, et l'intussusception associée à la tumeur est une constatation clinique rare mais urgente.(Traduit par Dr Serge Messier).

Multiple GISTand pheochromocytoma -A rare associationin neurofibromatosis type 1 / GIST múltipley feocromocitoma -Una asociación raraen neurofibromatosis tipo 1

We describe a case of coexistence of multiple gastrointestinal stromal tumors (GIST) and pheochromocytoma in a 32-year-old patient with neurofibromatosis type 1. The coexistence of these two conditions in patients with this syndrome is extremely rare.(AU)

Gastrointestinal stromal tumour-induced hypercalcaemia.

Hypercalcaemia is recognised as the most common oncological metabolic emergency, with several proposed underlying mechanisms. Nevertheless, hypercalcaemia has been rarely reported as a complication in patients with gastrointestinal stromal tumours (GISTs). GISTs are uncommon mesenchymal tumours of the gastrointestinal tract. There are only nine previous cases of hypercalcaemia occurring in patients with GIST reported in the literature. We report a case of a man in his 70s with a background of metastatic GIST on fourth-line treatment. The patient presented with new hypercalcaemia and acute kidney injury. Despite medical management, his calcium remained elevated and he deteriorated secondary to significant disease progression.

Multiple GIST and pheochromocytoma - A rare association in neurofibromatosis type 1.

We describe a case of coexistence of multiple gastrointestinal stromal tumors (GIST) and pheochromocytoma in a 32-year-old patient with neurofibromatosis type 1. The coexistence of these two conditions in patients with this syndrome is extremely rare.

Secondary amyloidosis with concomitant gastric GIST and gastric adenocarcinoma - A case report.

Secondary amyloidosis is a well-established entity and has been described in association with chronic inflammatory conditions such as rheumatoid arthritis, ankylosing spondylitis, bronchiectasis, tuberculosis, etc., It has also been reported in association with neoplasms such as Hodgkin's lymphoma, Waldenstrom's macroglobulinemia, renal cell carcinoma, lung carcinoma, etc. However, only a few case reports documenting the association of amyloidosis with gastrointestinal tumor (GIST) and gastric adenocarcinoma are available in the literature. Hereby, we report a case of a 74-year-old male who presented with colicky abdominal pain and vomiting. Ultrasonography revealed a common bile duct (CBD) stone and a small extra-luminal gastric mass. Endoscopic retrograde cholangiopancreatography (ERCP) was performed to remove the CBD stone which revealed an incidental finding of gastric ulcer. A biopsy was taken from the gastric ulcer which on histopathological examination was confirmed as adenocarcinoma leading onto total gastrectomy. During total gastrectomy, an inadvertent injury to the spleen led to simultaneous splenectomy. Multiple samples from the gastric ulcer, the extra-luminal gastric mass, and the spleen were subjected to histopathological examination. Gastric ulcer was confirmed as adenocarcinoma, gastric extra-luminal mass was confirmed as GIST, and splenic examination revealed widespread deposition of amyloid which on Congo-red stain imparted an apple-green birefringence on polarizing microscopy. To the best of our knowledge, this is the first-ever case of such an association where gastric adenocarcinoma occurred with concomitant gastric GIST and secondary amyloidosis of the spleen.

[Small intestinal hemorrhage in a patient with Neurofibromatosis type 1]. / Hemorragia de intestino delgado en un paciente con Neurofibromatosis tipo 1.

We present the case of a 32-year-old male patient with a history of Neurofibromatosis type 1, who presented with active small bowel bleeding, initially diagnosed by observing bleeding in ileoscopy, presenting with hemodynamic instability, abdominal angiotomography was performed, identifying a mass with contrast enhancement and active bleeding at the middle jejunum level, for which an angiography with arterial embolization of the branch that supplies said area is performed. With the patient stable, a double-balloon antegrade enteroscopy was performed, observing a subepithelial, ulcerated lesion, endoscopic tattooing was performed and finally surgery was sent for resection by laparoscopy. The pathology study was compatible with a jejunal gastrointestinal stromal tumor (GIST).

Glomus Tumor of the Stomach Presenting With Upper Gastrointestinal Bleeding: A Case Report.

Gastric glomus tumor is a rare mesenchymal tumor of the gastrointestinal tract, accounting for approximately 1% of all gastrointestinal soft tissue tumors. We describe a unique case of a 27-year-old female patient who presented with recurrent episodes of overt gastrointestinal bleeding requiring multiple blood transfusions. The patient was diagnosed with a gastric ulcer detected on esophagogastroduodenoscopy (EGD), which was grossly suggestive of an ulcerated gastrointestinal stromal tumor (GIST). Preoperative diagnosis was difficult, requiring laparoscopic robotic-assisted local wedge resection of the gastric mass. Pathological diagnosis and immunohistochemical (IHC) studies were consistent with a glomus tumor. We emphasize that the gastric glomus tumor might present with life-threatening recurrent gastrointestinal hemorrhage. In addition, it might mimic GIST and require surgical resection. Pathological diagnosis and IHC studies are needed to confirm the diagnosis.

[Metastatic brain lesion following gastrointestinal stromal tumor of the stomach complicated by coma.( A case report and literature review)]. / Metastaticheskoe porazhenie golovnogo mozga gastrointestinal'noi stromal'noi opukhol'yu zheludka, oslozhnivsheesya razvitiem komatoznogo sostoyaniya. (Opisanie sluchaya i obzor literatury).

The authors present an extremely rare case of metastatic brain lesion in a patient with gastrointestinal stromal tumor of the stomach. There are literature data on 23 cases of metastatic lesions of the brain, skull and soft tissues of the head in similar patients. Atypical localization of metastases can lead to some diagnostic difficulties, unreasonable cancellation of chemotherapy and delayed surgical treatment. A feature of our observation was postoperative coma determined by the features of the underlying disease.

Mortality in small bowel cancers and adenomas - A nationwide, population-based matched cohort study.

BACKGROUND: Small bowel adenocarcinoma (SBA), neuroendocrine tumors (NET) and gastrointestinal stromal tumors (GIST) are neoplastic lesions of the small bowel while small bowel adenomas are precursors of SBA. AIM: To examine mortality in patients diagnosed with SBA, small bowel adenomas, NET and GIST. METHODS: We performed a population-based matched cohort study encompassing all individuals with SBA (n = 2289), adenomas (n = 3700), NET (n = 1884) and GIST (n = 509) in the small bowel diagnosed at any of Sweden's 28 pathology departments between 2000 and 2016 (the "ESPRESSO study"). Each case was matched by sex, age, calendar year and county of residence to up to 5 comparators from the general population. Through Cox regression we estimated hazard ratios (HRs) and 95% confidence intervals (95%CIs) for death and cause-specific death adjusting for education. RESULTS: During follow-up until December 31, 2017, 1836 (80%) deaths occurred in SBA patients, 1615 (44%) in adenoma, 866 (46%) in NET and 162 (32%) in GIST patients. This corresponded to incidence rates of 295, 74, 80 and 62/1000 person-years respectively and adjusted HRs of 7.60 (95%CI=6.95-8.31), 2.21 (2.07-2.36), 2.74 (2.50-3.01) and 2.33 (1.90-2.87). Adjustment for education had a substantial impact on the HR for death in SBA but not for other neoplasias. The predominant cause of excess death was cancer in all groups. CONCLUSION: This study confirms earlier findings of increased death rates in patients with SBA and NET in a modern study population. We also demonstrate a more than 2-fold increased risk of death in both GIST and the SBA precursor adenoma.

[A case of multiple duodenal gastrointestinal stromal tumors associated with neurofibromatosis type 1].

Gastrointestinal stromal tumors (GISTs) associated with von Recklinghausen's disease (neurofibromatosis type 1 [NF1]) have different pathogenesis and characteristics from common GISTs. Furthermore, no treatment strategy for this type of GIST has been established. This study presents the case of a 76-year-old man previously diagnosed with NF1 who was later diagnosed with GISTs. A resection of the horizontal leg of the duodenum was performed, and no recurrence was observed 18 months after the surgery.

Rehabilitation of Social Communication Skills in Patients With Acquired Brain Injury With Intensive and Standard Group Interactive Structured Treatment: A Randomized Controlled Trial.

OBJECTIVE: To determine the efficacy of group interactive structured treatment (standard GIST) for improving social communication difficulties in a wider acquired brain injury (ABI) population compared to a waitlist control (WL). Secondary objectives were to (a) explore GIST across delivery formats by comparing the results to an intensive inpatient version of GIST (intensive GIST) and (b) compare the within-subject results for WL and intensive GIST. DESIGN: Randomized controlled trial with WL and repeated measures (pre- and posttraining, 3- and 6-month follow-ups). SETTING: Community and rehabilitation hospital. PARTICIPANTS: Forty-nine persons (27-74 years) with ABI and social communication difficulties (26.5% traumatic brain injury, 44.9% stroke, 28.6% other), minimum 12 months postinjury. INTERVENTION: Standard GIST (n=24) consisted of 12 weekly outpatient interactive group sessions (2.5 hours/session) and follow-up. Intensive GIST (n=18) consisted of 4 weeks with daily 4-hour inpatient group sessions (2 × 3 d/wk, 2 × 4 d/wk) and follow-up. MAIN OUTCOME MEASURES: La Trobe Questionnaire, a self-report questionnaire measuring social communication. Secondary measures: Social Communication Skills Questionnaire-Adapted, Goal Attainment Scale, Mind in the Eyes test, and questionnaires addressing mental and cognitive health, self-efficacy, and quality of life. RESULTS: When comparing the standard GIST and WL results, a trend of improvement was found for the main outcome, La Trobe Questionnaire, and a statistically significant improvement was found for the secondary outcome Social Communication Skills Questionnaire-Adapted. Comparing standard GIST and intensive GIST, improvement in social communication skills after both treatments was detected and maintained at 6-month follow-up. No statistically significant difference was found between groups. Goal attainment was achieved and maintained during follow-up for both standard and intensive GIST. CONCLUSIONS: Social communication skills were improved after both standard and intensive GIST, indicating that GIST can be delivered across treatment formats and to a wider ABI population.

Gastric volvulus associated with shrinkage of a gastrointestinal stromal tumor by neoadjuvant imatinib: a case report.

BACKGROUND: During neoadjuvant chemotherapy for giant gastrointestinal stromal tumors, changes in gastrointestinal stromal tumor size are rarely associated with events such as perforation and bleeding that require emergency surgery. Moreover, it is very rare for gastrointestinal stromal tumors to shrink and become mobile, resulting in gastric volvulus. Herein, we report a case of gastrointestinal stromal tumor shrinkage during neoadjuvant imatinib treatment, resulting in gastric volvulus that required surgery. To the best of our knowledge, this is the first reported occurrence of gastric volvulus during neoadjuvant imatinib treatment for a giant gastrointestinal stromal tumor. CASE PRESENTATION: A 58-year-old Japanese woman who was diagnosed with a giant gastric gastrointestinal stromal tumor and administered neoadjuvant imatinib presented to our hospital with complaints of abdominal pain and retching. Enhanced computed tomography revealed that the gastrointestinal stromal tumor had shrunk and shifted in position, and the stomach had organoaxially twisted. Accordingly, the patient was diagnosed with gastric volvulus caused by a gastric gastrointestinal stromal tumor. Conservative treatment did not improve the volvulus; hence, laparotomy was performed. The tumor developed from the lesser curvature of the stomach and caused rotation of the gastric body. The local gastric wall was resected. Histopathological examination confirmed the diagnosis of gastrointestinal stromal tumor. The patient received adjuvant imatinib for 3 years and has been alive for 5 years without recurrence. CONCLUSIONS: Gastric volvulus can be caused by the laxity of the ligaments that hold the stomach and gastric ptosis or esophageal hernia and diaphragmatic hernia; therefore, gastric gastrointestinal stromal tumors rarely cause gastric volvulus. However, a risk of torsion exists if the gastrointestinal stromal tumor develops extramural to lesser curvature and attains a certain size.

The Association between the Growth Pattern and Malignancy in the Formation of Ulceration in Gastric Gastrointestinal Stromal Tumor.

Objective In general, surface ulceration in gastric gastrointestinal stromal tumor (GIST) is considered a malignant feature; however, the mechanism underlying its formation has not been evaluated in detail. In this study, we analyzed the factors involved in ulceration using resected specimens of gastric GIST. Methods A total of 48 samples were retrospectively analyzed. We examined the association of surface ulceration of gastric GIST with the MIB-1 labeling index, mitotic number, tumor size, endoscopic ultrasound (EUS) findings and growth pattern on computed tomography (CT). Results The proportion of men was significantly higher in the ulceration group than in the non-ulceration group (p=0.04146), whereas age was not significantly different between the groups. Tumor was significantly larger in the ulceration group than in the non-ulceration group (p=0.0048). There was no correlation between tumor size and ulcer number. The MIB-1 index was not related to ulceration, nor were EUS findings. The number of mitotic cells tended to be higher in the ulceration group than in the non-ulceration group (p=0.05988). Intraluminal growth pattern was strongly associated with ulceration (p=0.00019). After a multivariate analysis, the growth pattern was the only factor associated with ulceration of gastric GIST. Conclusion Although formation of surface ulceration in gastric GIST was partially associated with the degree of malignancy, the growth pattern was the most important factor associated with ulceration in gastric GIST.