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Item Extraskeletal Ewing Sarcoma of the Gastrointestinal and Hepatobiliary Tract: Deceptive Immunophenotype Commonly Leads to Misdiagnosis(Wolters Kluwer, 2024) Shiyanbola, Oyewale; Nigdelioglu, Recep; Dhall, Deepti; González, Iván A.; Warmke, Laura M.; Schechter, Shula; Choi, Won-Tak; Hu, Shaomin; Voltaggio, Lysandra; Zhang, Yujie; Liang, Tom Z.; Ko, Huaibin M.; Charville, Greg W.; Longacre, Teri A.; Pathology and Laboratory Medicine, School of MedicineEwing sarcoma (ES) is an uncommon mesenchymal neoplasm that typically develops as a bone mass, although up to 30% arise in extraskeletal sites. ES of the gastrointestinal (GI) and hepatobiliary tract is rare and may be misdiagnosed as other, more common neoplasms that occur in these sites. However, the correct classification of extraskeletal ES is important for timely clinical management and prognostication. We reviewed our experience of ES in the GI and hepatobiliary tract in order to further highlight the clinicopathologic features of these neoplasms and document the potential for misdiagnosis in this setting. The archives and consultation files of 6 academic institutions were retrospectively queried for cases of ES occurring in the GI and hepatobiliary tract. The histologic slides and ancillary studies were reviewed and clinical data were retrieved for each case through the electronic medical records, when available. Twenty-three patients with ES in the GI and/or hepatobiliary tract were identified from 2000 to 2022. Of these, 11 were women and 12 were men with a median age of 38 years (range, 2 to 64). Tumor locations included the pancreas (n=5), liver (n=2), stomach (n=3), colorectum (n=3), and small intestine (n=5), as well as tumors involving multiple organs, pelvis and retroperitoneum (n=5). Tumor size varied between 2 cm and 18 cm. Twenty were primary and 3 were metastases. Of the 23 cases, only 17% were initially diagnosed as ES. The most common misdiagnoses involved various forms of neuroendocrine neoplasia due to expression of synaptophysin and other neuroendocrine markers (22%). A wide variety of diagnoses including GI stromal tumor was considered due to aberrant CD117 expression (4%). The diagnosis of ES was ultimately confirmed by detection of the EWSR1 rearrangement in 22 cases. The remaining case was diagnosed using traditional immunohistochemistry. Follow-up information was available in 20 cases, with follow-up time varying between 2 and 256 months. Six patients with follow-up died of disease between 6 and 60 months following initial presentation. Our data indicate ES in the GI and hepatobiliary tract is commonly misdiagnosed leading to a delay in therapy. In light of the attendant therapeutic and prognostic implications, ES should be considered in the differential diagnosis of any GI or hepatobiliary tumor with epithelioid and/or small round cell morphology.Item Highly variable biodistribution of 68Ga labeled somatostatin analogues 68Ga-DOTA-NOC and 68Ga-DOTA-TATE in neuroendocrine tumors: clinical implications for somatostatin receptor directed PET/CT(AME, 2022) Cheng, Monica; Tann, Mark; Radiology and Imaging Sciences, School of MedicineBackground: Somatostatin receptor (SSTR)-targeted positron emission tomography/computed tomography (PET/CT) imaging has risen to the forefront for neuroendocrine tumor (NET) detection and management, yet the variability of significant uptake variability (SUV) as a semiquantitative measure of disease detection and tumor response to treatment has not been fully explored. Methods: We assess the reproducibility and interscan variability of SUV metrics of normal tissue and NET in serial 68Ga-DOTA-NOC and 68Ga-DOTA-TATE PET imaging to clinically monitor disease state. Eighty-one patients were enrolled in this retrospective study. Results: Both primary and metastatic hepatic lesions demonstrated SUV (SUVmean 16.5±8.0). The median SUVmean was 16 for the spleen, 9.7 for the pituitary, 12.6 for the adrenal glands, and 4.8 for the liver. The normal pituitary gland demonstrates focal homogenous uptake with SUVmax range of 4.5-23. The adrenal gland showed uptake with SUVmax range of 4.1-29.4, which is more than two times greater than liver uptake (SUVmean range, 2.3-12.4). Highest physiological uptake seen in the spleen (average SUVmean of 17.3, range of 5.4-34.4). Conclusions: The highly variable nature of regional SUVmean and SUVmax in both physiologic tissue and lesions suggests the need for incorporation of more reliable quantitative measures for clinical decision making.Item Phosphate Binders and Non-Phosphate Effects in the Gastrointestinal Tract(Elsevier, 2020-01) Biruete, Annabel; Hill Gallant, Kathleen M.; Lindemann, Stephen R.; Wiese, Gretchen; Chen, Neal; Moe, Sharon; Medicine, School of MedicinePhosphate binders are commonly prescribed in patients with end-stage kidney disease to prevent and treat hyperphosphatemia. These binders are usually associated with gastrointestinal distress, may bind molecules other than phosphate, and may alter the gut microbiota, altogether having systemic effects unrelated to phosphate control. Sevelamer is the most studied of the available binders for nonphosphate-related effects including binding to bile acids, endotoxins, gut microbiota-derived metabolites, and advanced glycation end products. Other binders (calcium- and noncalcium-based binders) may bind vitamins, such as vitamin K and folic acid. Moreover, the relatively new iron-based phosphate binders may alter the gut microbiota, as some of the iron or organic ligands may be used by the gastrointestinal bacteria. The objective of this narrative review is to provide the current evidence for the nonphosphate effects of phosphate binders on gastrointestinal function, nutrient and molecule binding, and the gut microbiome.Item Reimagining Gastroenterology Fellowship Training – The Third Year of the Future(Elsevier, 2023) Bhavsar-Burke, Indira; Shah, Brijen J.; Carethers, John M.; Whitson, Matthew J.; Kaul, Vivek; David, Yakira; Dilly, Christen K.; Medicine, School of MedicineItem The Clinical Significance of Incidental GIT Uptake on PET/CT: Radiologic, Endoscopic, and Pathologic Correlation(MDPI, 2023-03-30) Hosni, Mohammad N.; Kassas, Mutaz; Itani, Mohamad I.; Rahal, Mahmoud A.; Al-Zakleet, Safaa; El-Jebai, Malak; Abi-Ghanem, Alain S.; Moukaddam, Hicham; Haidar, Mohamad; Vinjamuri, Sobhan; Shaib, Yasser H.; Medicine, School of MedicineIncidental gastrointestinal tract (GIT) [18F]-Fluorodeoxyglucose (FDG) uptake in positron emission technology/computed tomography (PET/CT) is an unexpected and often complicated finding for clinicians. This retrospective study reviewed 8991 charts of patients who underwent PET/CT: 440 patients had incidental GIT uptake, of which 80 underwent endoscopy. Patient characteristics, imaging parameters, and endoscopic findings were studied. Of the 80 patients, 31 had cancer/pre-cancer lesions (16 carcinomas; 15 pre-malignant polyps). Compared to patients with benign/absent lesions, patients with cancer/pre-cancer lesions were significantly older (p = 0.01), underwent PET/CT for primary evaluation/staging of cancer (p = 0.03), had focal GIT uptake (p = 0.04), and had lower GIT uptake (p = 0.004). Among patients with focal uptake, an SUVmax of 9.2 had the highest sensitivity (0.76) and specificity (0.885) in detecting cancer/pre-cancerous lesions. Lower GIT uptake was most common in the sigmoid colon, and upper GIT uptake was most frequent in the stomach. In a bivariate analysis, predictors of cancer/pre-cancer were older age, PET/CT indicated for primary evaluation, focal uptake, uptake in the lower GIT, and higher SUVmax. Further endoscopic investigation is warranted for patients with incidental GIT uptake, especially in the elderly or those presenting for primary evaluation with PET/CT, with the following findings on imaging: lower GIT uptake, focal uptake, or high SUVmax.Item What is the Clinical Impact of a Multiplex Gastrointestinal Panel in Children?(Oxford University Press, 2022) Liepmann, Claire; Hecht, Shaina; Pediatrics, School of Medicine