Purpose: 57 year old man was seen for screening colonoscopy. He reported no symptoms, specifically no weight loss, no changes to his bowel habits and no abdominal pain. He had no chronic medical problems, no family history of colorectal, breast or thyroid cancers, no polyposis syndromes and no abnormal skin lesions. His physical exam and laboratory values were unremarkable. During colonoscopy, a single 5mm sessile polyp located 10 cm distal from the ileocecal valve was cold snared. Microscopic examination of the polyp revealed expansion of the lamina propria by compact interlacing bundles of Schwann cells lying in a fibrillary matrix displacing the colonic glands. Interspersed among them were ganglion cells, scattered singly or in clusters within the mucosa. Immunohistochemistry demonstrated intense nuclear and cytoplasmic staining of Schwann cells with S-100 protein. Ganglion cells were intensely immunopositive for GFAP and synaptophysin. These findings were consistent with a ganglioneuroma. Ganglioneuroma of the gastrointestinal tract is a rare tumor composed of ganglion cells. It occurs as three well recognized entities: isolated, polyposis or diffuse. Isolated ganglioneuroma resembles a hyperplastic polyp endoscopically and does not appear to have a risk of malignancy. Complications arise from its size and anatomical location leading to obstruction. Ganglioneuromatous Polyposis is characterized by a larger number of polyps (usually more than 20) and occurs in conjunction with other syndromes that have malignancy potential such as von Recklinghausen's, multiple endocrine neoplasia 2b, Cowden disease and Adenomatous Polyposis syndromes. Diffuse ganglioneuromatosis presents endoscopically as irregular nodular mucosa in areas of stricture or thickening associated with bowel obstruction. Conclusion: Our patient presented with isolated ganglioneuroma incidentally found on screening colonoscopy. His family history, past medical history, physical exam and endoscopic exam did not support ganglioneuromatous polyposis or diffuse ganglioneuromatosis. Reassurance was provided and an average risk surveillance colonoscopy schedule recommended.Figure: Intense staining of ganglion cells by synaptophysin (immunoperoxidase diaminobenzidine and hematoxylin counterstain, original magnification X 200).
OBJECTIVES/GOALS: The objective of this project was to evaluate the factors that contribute to LGBTQIA2+ patient comfortability. This information was then used to understand how best to create a comfortable space for LGBTQIA2+ patients. METHODS/STUDY POPULATION: This survey was focused on underinsured and uninsured patients seen at the Rainbow Clinic - a free student-run LGBTQIA2+ clinic. Surveys were distributed by undergraduate volunteers on tablets as a qualtrics survey. Surveys collected demographic information in addition to 5 questions that assessed patient comfortability. These questions included evaluating the patient’s comfort with sharing information with the provider and the patient’s comfort of coming into clinical spaces. These surveys were distributed before and after clinic appointments to capture any changes in comfortability that could have occurred as a result of the appointment. RESULTS/ANTICIPATED RESULTS: Up to May of 2023, 49 patients were seen in Rainbow Clinic. 33 patients filled out the intake survey and 31 patients filled out the check-out survey resulting in a 67% and 63% response rate respectively. Questions were asked on a likert scale (1-5) from Strongly Disagree to Strongly Agree. Questions evaluating patient comfort in sharing information with their provider yielded an average score that was statistically significant, suggesting patients felt comfortable at the Rainbow Clinic. Additionally, patients indicated that the LGBTQIA2+ specific labeling of the Rainbow Clinic made them significantly more comfortable coming into the clinic. DISCUSSION/SIGNIFICANCE: This project suggests that patient comfortability can be improved by training and intentional LGBTQIA2+ labeling. Considering the hesitancy of this community towards healthcare, improving comfortability not only benefits clinical care and outcomes but can also bolster the body of research on this community.
Angiomatoid fibrous histiocytoma (AFH) is a rare and slow-growing soft tissue lesion that typically arises in the extremities of young patients. Microscopically, AFH is characterized by pseudovascular, blood-filled spaces that are surrounded by a multinodular proliferation of spindle and/or round cells and lymphoid cuffs. However, there is a wide morphological spectrum, including a myxoid variant. Examples with a prominent myxoid matrix are rare and may pose great diagnostic difficulty. Specific gene fusions have been found to play a significant role in AFH tumorigenesis. Gene fusions of Ewing sarcoma breakpoint region 1 (EWSR1) with members of the cAMP response element-binding protein family (CREB) of transcription factors (CREB1, activating transcription factor 1 (ATF1), and cAMP response element modulator (CREM)) have been described in histopathologically diverse mesenchymal neoplasms such as AFH, hyalinising clear cell carcinomas of salivary glands, primary pulmonary myxoid sarcoma, and clear cell sarcoma. Classically, EWSR1-CREB is known to be the prominent gene fusion in AFH. Recently, a small series of intracranial mesenchymal tumors with EWSR1-CREB family gene fusions has been reported. These tumors seem to show histologic, immunophenotypic, and cytogenic features similar to those observed in the myxoid variant of AFH; therefore, there is a debate on whether these tumors merely represent a variant of AFH or a novel entity. This case report is of a 58-year-old woman presenting with the first episode of generalized seizure due to an extra-axial lesion with homogenous contrast enhancement in the right parietal lobe, which was initially diagnosed as a World Health Organization (WHO) grade I meningioma. Following a series of pathological investigations, the diagnosis of an intracranial myxoid variant of AFH was made. This case report illustrates the need to consider the myxoid variant of intracranial AFH in the differential diagnosis of meningioma-like tumors. A high index of suspicion is required if the meningioma behaves abnormally with a much higher recurrence rate.
Acute illness causes physical function decline and mortality in older adults (age ≥ 65 years).1, 2 Older adults admitted or discharged from the emergency department (ED)1, 2 are particularly at risk for functional decline and some never return to their pre-illness baseline.2 Functional status determines whether a person can manage their new illness or injury at home and therefore impacts disposition decisions. The Geriatric ED Guidelines,3 Geriatric ED Accreditation, and growth of geriatric EDs has placed a new focus on identifying older adults at risk for functional decline during ED visits. Although a recent umbrella review demonstrated low evidence of benefit of ED interventions to prevent or reduce functional decline in older adults, these previous efforts focus on the long-term trajectory of functional status after acute illness, trauma, or heterogenous patient groups prior to Geriatric ED Accreditation. There is limited understanding of acute functional impairment due to medical illness in older adults at the point of ED presentation since the advent of Geriatric ED Guidelines. The purpose of this study was to describe the prevalence of acute decline in ability to perform activities of daily living (ADL) in older adults presenting to the ED with suspected pneumonia. We conducted a preplanned secondary descriptive analysis of patients in a prospective, observational cohort study of older adult ED patients presenting to two EDs in one health system with methodology previously reported.4 Study was approved by the institution's institutional review board. The Older American Resources and Services (OARS)5 ADL questionnaire was used to measure change in function at ED presentation compared to the pre-morbid period. We report the total OARS score change observed in ability to perform ADL at ED presentation compared to 1 week before based on patient/proxy report. The change in total OARS score was categorized as improved/unchanged; decrease of 1, 2, and ≥3. We chose these due to the varied OARS decrease identified as clinically important.6-8 We also report any decrease in ≥1 individual physical or instrumental ADLs and in ≥1 in both categories. Data are presented as frequency and percent in all and discharged patients. The parent study enrolled 135 patients from October 2019 to March 2022 with a pause due to the COVID-19 pandemic. Five patients were enrolled by proxy without enough information to complete the OARs, therefore, 130 patients are included. Demographics are in Table 1. Compared to a week before the ED visit, 60.8% had worsening total OARS score and 39.2% had no worsening; 6.9% had a decrease of 1; 6.9% of 2; and 46.9% of ≥3. Ten percent had only instrumental ADL worsening (decrease of ≥1); 5.4% only physical ADL worsening (decrease of ≥1) and 46.2% had both (decrease of ≥2 in total with at least one in each) (Figure 1). Among the discharged patients (n = 21), 33.3% had worsening total OARS score and 66.7% had no worsening; 4.8% had a decrease of 1; 9.5% of 2; and 19.1% of ≥3; 14.3% had only instrumental; 4.8% had only physical; and 14.3% had both. Using the most restrictive definition (≥3), almost one in two older adult ED patients with suspected pneumonia experienced functional decline including one in five discharged patients. This presents a unique perspective—that when patients arrive to the ED, they have already suffered an acute decline in their functional ability prior to that well-described associated with hospitalization. For discharged patients, the ED visit may be the only opportunity for intervention on acute functional decline and many EDs have community resources that should be leveraged (PT consults, meal delivery, etc.). For admitted patients, early identification of functional decline could expedite inpatient intervention. Due to this, universal screening is recommended by the Geriatric ED Guidelines,3 but this has proven challenging.9, 10 Appropriately, identifying which older adults are most likely to benefit from the screening is a research topic of growing importance. This study has limitations. Most importantly, that the included population was ill with a high admission rate; thus, the generalizability to all ED older adults may be limited. And, the OARS score is aggregated, which introduces complexity as determining the impact of the decline requires consideration of individual components. Older adults with suspected pneumonia have significant rates of acute functional decline at ED presentation. Emergency physicians should evaluate for acute decline during their treatment and disposition decisions. KMH, ALS, JAS, TRG, LCM, and JMC obtained funding for this work and conceived the idea for this manuscript; KMH, ALS, ME, CH, BCL, LTS, EWB, TRG, LCM, MH, CBC, GL, and JMC were critical in the conduct of the clinical study; KMH and JMC drafted the manuscript, performed revisions, and contributed their expertise in the area; ALS, ME, CH, BCL, LTS, JAS, EWB, TRG, LCM, MH, CBC, and GL were responsible for critical revision of the manuscript for important intellectual content and their content expertise. Dr. Hunold is supported by NIA R03AG064379. Dr. Caterino is supported by NIA R01AG050801. The authors have no conflicts to report. The funders had no role in the design, methods, subject recruitment, data collections, analysis, or preparation of this manuscript.
Once again, it is that time of the year! This time, not only is the New Year upon us, it is also the dawn of a new millennium. Because we all made it and the purported Y2K bug has not caused the bank to deposit a few million dollars extra into our bank accounts by mistake, maybe we should use this time to really take stock of our lives, start anew and recommit to working on all those New Year’s resolutions. Inevitably, ‘taking stock’ includes the subject of money, and someone is bound to say,
To evaluate the long-term outcomes in patients diagnosed with symptomatic optic pathway gliomas (OPGs). Retrospective review of all patients with symptomatic OPG treated in a single center from 1984 to 2016. Thirty-seven patients were identified with a median follow-up of 13.74 years (range 1.0-29.6). Eleven (29.7%) of them had NF1. The mean age was 5.8 years and female: male ratio was 1.8:1. Decreased visual acuity was the commonest symptom (75.7%) at presentation. Six cases underwent surgical debulking, 13 were biopsied, and 2 had cosmetic orbital decompression. Histopathological subtypes were pilocytic astrocytoma (n=18) and ganglioglioma (n=1). Seventeen patients received chemotherapy and 18 had radiotherapy. The progression-free survival (PFS) was 53 ± 8.3% at 5 years and 49.7 ± 8.4% at 10, 20 and 25 years. The overall survival (OS) was 97.2 ± 2.7% at 5 and 10 years, 86.1 ± 7.8% at 20 and 77.5 ± 10.8% at 25 years. Twenty-two (59.5%) patients without initial evidence of endocrinopathy developed hormonal defects following therapy. Vision remained unchanged in 15 (40.5%) patients, 14 (37.8%) had visual deterioration at least in one eye, whilst improvement of vision and normal vision were seen in 5 (13.5%) and 3 (8.1%) cases respectively. Second brain tumour was observed in 3 patients at 20.6, 25.3 and 28.7 years post-diagnosis. All 3 had received prior radiotherapy. Whilst the PFS remained static after 10 years, the OS dropped after 20 years, due to the occurrence of second malignant brain tumours in patients who received radiotherapy. Significant long-term morbidities were observed in the majority of survivors.
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