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PURPOSE: Localized gastrointestinal tract amyloidosis is uncommon and little is known regarding this entity. There is no current standard of care for the management of localized amyloidosis. The objective of this study was to evaluate the characteristics, available treatments, outcomes and surveillance of these patients. METHODS: We conducted a systematic review of cases reported in the literature from 1962 to 2021. Patients with gastrointestinal amyloidosis reported in English literature were included in the analysis. We described and summarized the patient's characteristics, treatments, clinical presentations, outcomes and surveillance. RESULTS: The systematic review of reported clinical cases included 62 patients. In these patients, the most common site of amyloid deposition was the stomach (42%). The median age of diagnosis is 64.4 years old; there is a 2:1 prevalence among males (63%) to females (37%); abdominal pain is the most common type of presentation (41%), although patients could also be asymptomatic. There is a high curative rate (100%) with resection alone. Among patients treated with a type of systemic therapy, 80% achieved a complete response. The minority of cases reported a type of surveillance post treatment, and among those 62% pursued serial clinical evaluations alone. CONCLUSION: To our knowledge, this is the first and largest systematic review of the literature in gastrointestinal tract amyloidosis. This is more common among males and seems to have an excellent curative rate (100%) with surgery alone. Systemic therapy is an option for those with non-resectable amyloidomas. Serial clinical evaluations should be part of the standard surveillance care in these patients.

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OBJECTIVES: Functional neuroendocrine tumors (FNETs) are characterized by excess secretion of disease-specific hormones. In this study, we attempted to define survival trends in patients with some of these uncommon tumors. METHODS: Using the Surveillance, Epidemiology, and End Results database, 529 patients with FNETs (gastrinoma, insulinoma, glucagonoma, VIPoma, and somatostatinoma) were identified. We analyzed patient and tumor characteristics, overall survival, and cancer-specific survival. RESULTS: Functional neuroendocrine tumors were found to be more predominant in White patients older than 50 years. Most common FNETs were gastrinoma (56.3%) and insulinoma (23.8%). Most FNETs were found in the pancreas, with the second most common location being the small bowel. Surgery was the primary modality of treatment, used in 55.8% of the cases. Median overall survival was 9.8 years (95% confidence interval [CI], 7.9-11.8) with a median cancer-specific survival of 18.5 years (95% CI, 12.8-24.2). In multivariate analysis, age >50 years (hazard ratio [HR], 2.7; 95% CI, 2.02-3.64), no surgical resection (HR, 1.88; 95% CI, 1.43-2.46), metastasis (HR, 3.0; 95% CI, 2.0-4.5), and poor differentiation were associated with poor survival. Site and histology did not have a significant impact on survival (P = 0.82 and 0.57 respectively). CONCLUSIONS: Our study highlights the most important prognostic factors for gastrointestinal FNETs.

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OBJECTIVE: National Institute of Health recommends that patient education material should be written at no greater than 6th-grade level. Cancer survivorship and fertility preservation discussion with patients is recommended by many national societies and being done more frequently. We sought to analyze online patient information on cancer survivorship and fertility preservation to see if they meet the criteria set by national guidelines. METHODS: Online patient information on cancer survivorship and fertility preservation was collected and analyzed by six of the most common readability tests. Only websites in English and free to access were used. RESULTS: A total of 15 separate websites for cancer survivorship and fertility preservation was used. All websites failed to meet national guidelines. Cancer survivorship information was written at a high school senior and a 12th-grade level. Fertility preservation information was written at a high school-senior and junior college level. CONCLUSION: Online patient information on cancer survivorship and fertility preservation did not meet national guidelines. Testing across six of the most used readability indexes showed that information is challenging to understand for the general patient population. POLICY STATEMENT: This article shares an insight into the complex and growing fields of cancer survivorship and fertility preservation. Educating patients about their condition is critical and improves outcomes and participation in shared decision making. Healthcare policy should focus on implementing a system that will provide culturally and linguistically appropriate information in the community for patients about their disease.

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BACKGROUND: Mogamulizumab (Moga) is a C-C chemokine receptor-4 antibody approved in the United States for relapsed /refractory mycosis fungoides and Sézary syndrome. Few cases reported an increased risk of hepatitis B reactivation and cytomegalovirus (CMV) related infection post-Moga. However, literature is limited to mainly case reports and series, while no study has used the Food and Drug Administration adverse events reporting system (FARES) database to investigate the relationship. METHODS: Using United States Food and Drug Administration adverse events reporting system database, we collected all cases of hepatitis B reactivation and CMV related infection between January 1, 2011, and December 31, 2019, for Moga and other drugs. The reporting odds ratio (ROR) was calculated, which was considered significant when the lower limit of 95% confidence interval (CI) >1. FINDINGS: Three hundred and thirty-eight total adverse cases were reported for Moga during the study period, with 261 cases reported indication for use, including cutaneous T cell lymphoma (47.04%), and adult T cell leukemia/lymphoma (30.18%). Eight cases were reported for hepatitis B reactivation with Moga use, compared to 2290 cases with other medications. The ROR is 143.67 (p<0.001, 95% CI, 71.17-290.04). CMV related infection was noted in 17 cases using Moga, while 12,849 cases with others. The ROR is 55.89 (p<0.001, 95% CI, 34.31-91.06). In the Moga group, five deaths occurred in hepatitis B reactivation patients and nine deaths with CMV cases. INTERPRETATION: A signal has been identified between Moga exposure and hepatitis B reactivation as well as CMV related infection. A consideration in future studies should be placed on determining the relationship and investigating the need for pre-treatment screening, close monitoring, and utilization of prophylaxis in this population-based on pre-treatment risks. FUNDING: None.

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The kidney is one of the major organs affected in preeclampsia. There is evidence suggesting a role for excessive complement activation in the pathogenesis of preeclampsia. We describe a case of preeclampsia with severe features, including HELLP syndrome (hemolysis, elevated liver enzymes, low platelets) and acute kidney injury (AKI) that developed following caesarian section. The patient required renal replacement therapy. A trial of daily plasma exchange was not effective. The patient received a single dose of eculizumab, a humanised monoclonal IgG antibody that binds to complement protein C5. One week post administration of eculizumab, there was significant improvement in haematologic, hepatic and renal function. Blood pressure had normalised and renal replacement therapy was discontinued. The use of eculizumab may have contributed to recovery of kidney function further supporting the role of complement activation in the pathogenesis of preeclampsia and associated AKI.

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