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Background: Primary sclerosing cholangitis (PSC) associated inflammatory bowel disease (IBD) is a unique form of IBD (PSC-IBD) with distinct clinical and histologic features from ulcerative colitis (UC) and Crohn disease (CD). In patients with PSC and IBD, the severity of the two disease processes may depend on each other., Aim: To study the histologic and clinical features of PSC patients with and without IBD., Methods: We assessed specimens from patients with UC ( n = 28), CD ( n = 10), PSC and UC (PSC-UC; n = 26); PSC and CD (PSC-CD; n = 6); and PSC and no IBD (PSC-no IBD; n = 4) between years 1999-2013. PSC-IBD patients were matched to IBD patients without PSC by age and colitis duration. Clinical data including age, gender, age at IBD and PSC diagnoses, IBD duration, treatment, follow-up, orthotopic liver transplantation (OLT) were noted., Results: PSC-UC patients had more isolated right-sided disease ( P = 0.03), and less active inflammation in left colon, rectum ( P = 0.03 and P = 0.0006), and overall ( P = 0.0005) compared to UC. They required less steroids ( P = 0.01) and fewer colectomies ( P = 0.03) than UC patients. The PSC-CD patients had more ileitis and less rectal involvement compared to PSC-UC and CD. No PSC-CD patients required OLT compared to 38% of PSC-UC ( P = 0.1). PSC-IBD (PSC-UC and PSC-CD) patients with OLT had severe disease in the left colon and rectum ( P = 0.04)., Conclusion: PSC-UC represents a distinct form of IBD. The different disease phenotype in PSC-IBD patients with OLT may support liver-gut axis interaction, however warrants clinical attention and further research., Competing Interests: Conflict-of-interest statement: We have no financial relationships to disclose., (©The Author(s) 2020. Published by Baishideng Publishing Group Inc. All rights reserved.)

Objectives/hypothesis: To evaluate outcomes for patients with pathological N3 (pN3) neck disease from human papillomavirus (HPV)-related oropharyngeal squamous cell carcinoma (OPSCC) and determine variables predictive of survival., Study Design: Retrospective case series with chart review., Methods: This study was conducted between 1998 and 2013 and included patients with HPV-related OPSCC treated with surgery with or without adjuvant therapy and who had pN3 nodal disease. The primary outcome was disease-specific survival (DSS). Secondary outcomes included overall survival (OS), disease-free survival (DFS), adverse events, and gastrostomy tube rates., Results: Thirty-nine patients were included, of whom 36 (90%) underwent adjuvant therapy. Median follow-up was 39 months (range, 2-147 months). Mean age was 56 years, and 87% were male. Seventeen patients (44%) underwent selective neck dissection, whereas six (15%) underwent radical (n = 2) or extended radical (n = 4) neck dissection. Ninety-two percent had extracapsular extension. Five-year Kaplan-Meier estimated DSS, OS, and DFS were 89% (95% confidence interval [CI]: 79%-99%), 87% (95% CI: 75%-99%), and 84% (95% CI: 72%-96%), respectively. The disease recurrence rate was 10% (5% regional, 5% distant metastasis). Patients with less than 5 pathologically positive lymph nodes (P = .041) had improved DFS., Conclusions: Patients with HPV-related OPSCC and pN3 nodal disease treated with surgery and adjuvant therapy have very favorable long-term survival and regional control. Patients with five or more pathologically positive lymph nodes may be at higher risk for recurrence., Level of Evidence: 4. Laryngoscope, 127:2033-2037, 2017., (© 2016 The American Laryngological, Rhinological and Otological Society, Inc.)

Pediatric fibroblastic and myofibroblastic tumors are a relatively common group of soft-tissue proliferations that are associated with a wide spectrum of clinical behavior. These tumors have been divided into the following categories on the basis of their biologic behavior: benign (eg, myositis ossificans, myofibroma, fibromatosis colli), intermediate-locally aggressive (eg, lipofibromatosis, desmoid fibroma), intermediate-rarely metastasizing (eg, inflammatory myofibroblastic tumors, infantile fibrosarcoma, low-grade myofibroblastic sarcoma), and malignant (eg, fibromyxoid sarcoma, adult fibrosarcoma). Imaging has a key role in the evaluation of lesion origin, extent, and involvement with adjacent structures, and in the treatment management and postresection surveillance of these tumors. The imaging findings of these tumors are often nonspecific. However, certain imaging features, such as low or intermediate signal intensity on T2-weighted magnetic resonance images and extension along fascial planes, support the diagnosis of a fibroblastic or myofibroblastic tumor. In addition, certain tumors have characteristic imaging findings (eg, multiple subcutaneous or intramuscular lesions in infantile myofibromatosis, plaquelike growth pattern of Gardner fibroma, presence of adipose tissue in lipofibromatosis) or characteristic clinical manifestations (eg, great toe malformations in fibrodysplasia ossificans fibroma, neonatal torticollis in fibromatosis colli) that suggest the correct diagnosis. Knowledge of the syndrome associations of some of these tumors-for example, the association between familial adenomatous polyposis syndrome and both Gardner fibroma and desmoid fibromatosis, and that between nevoid basal cell carcinoma syndrome and cardiac fibroma-further facilitate a diagnosis. The recognition of key imaging findings can help guide treatment management and help avoid unnecessary intervention in cases of benign lesions such as myositis ossificans and fibromatosis colli. In this article, we describe the various types of fibroblastic and myofibroblastic tumors in children and the characteristic clinical manifestations, imaging features, and growth patterns of these neoplasms-all of which aid in the appropriate radiologic assessment and management of these lesions. (©)RSNA, 2016.

Gestational trophoblastic tumors can be difficult to distinguish from nongestational neoplasms. Somatic and germ cell tumors can mimic gestational choriocarcinoma, and epithelioid trophoblastic tumor (ETT) is known for its histologic, and sometimes clinical, resemblance to squamous cell carcinoma. Short tandem repeat (STR) analysis can separate gestational from nongestational neoplasms and can provide useful information about the type of causative conceptus. We present a series of cases which demonstrate the utility of STR analysis in the evaluation of gestational choriocarcinoma, epithelioid trophoblastic tumor, and their mimics. Samples from normal tissue and tumor were microdissected. DNA was extracted, and STR analysis was performed. Five cases were identified in which there was clinical and/or histologic concern for a gestational trophoblastic neoplasm. Case 1 is a choriocarcinoma presenting concurrently with a 16-week gestation. STR testing on the tumor, mother, and fetus showed that the tumor arose from a previous occult complete hydatidiform mole. Case 2 is an ETT presenting as multiple masses in bilateral kidneys, initially diagnosed as urothelial carcinoma. However, because of an elevated human chorionic gonadotropin, additional workup was performed which showed that the tumor was most likely an ETT. STR analysis showed that the tumor arose from a nonmolar pregnancy. Cases 3-5 illustrate somatic carcinomas mimicking gestational neoplasia. In those cases, STR confirmed a somatic origin. STR can be useful in distinguishing gestational from nongestational neoplasms, particularly in unusual settings. Also, STR analysis can add clinically useful information that is not available from clinical or histologic evaluation., (Copyright © 2016 Elsevier Inc. All rights reserved.)

Objective: The objective of our study was to compare the cost-effectiveness of two approaches to using MRI for the evaluation of patients with hearing loss., Materials and Methods: We developed a decision tree to compare the cost-effectiveness of conventional MRI with that of a proposed two-tiered model in which an initial 3D T2-weighted imaging examination was used to determine the need for comprehensive MR scanning. Three radiologists independently and blindly reviewed the 3D T2-weighted images acquired as part of the comprehensive MR examinations of 256 patients with hearing loss to assess the diagnostic efficacy of the two-tiered approach. Costs were defined in terms of both the scanner utilization time for the imaging facility and the dollar amount for payers. Effectiveness was defined in terms of the ability to correctly detect the presence or absence of disease., Results: The conventional approach was less cost-effective, with a baseline incremental cost-effectiveness ratio (ICER) of 27,299 minutes of scanner utilization per unit increase in effectiveness. Assuming a 50% reduction in the reimbursement of the technical component from the current level by the Centers for Medicare & Medicaid Services, this result reflected an ICER of $258,664 per unit increase in effectiveness. The results of a sensitivity analysis showed the robustness of the cost-effectiveness of the two-tiered imaging approach in a variety of scenarios that reflect differences in scanning practices and possible differences in recall rates. The conventional imaging approach was absolutely dominated by the two-tiered approach in the scenarios created to reflect the expected range of prevalence of disease., Conclusion: A two-tiered approach to MRI provides a more cost-effective alternative to the current approach of using a comprehensive MRI examination without and with contrast material to evaluate patients with hearing loss.

Objective: The purpose of this study was to compare the diagnostic efficacy and cost implications of a proposed two-tiered approach to MRI in patients with headache., Materials and Methods: We identified 245 consecutive patients with headache using MRI studies performed at a tertiary care facility between October 2009 and July 2011. Three radiologists prospectively used FLAIR sequences from these MR studies to diagnose underlying abnormality or to identify the need for a comprehensive MRI study. We compared the diagnostic efficacy and the cost implications of such a two-tiered approach with those of conventional MRI from the perspectives of the payer, the patient, and the imaging facility., Results: The sensitivity and specificity for two-tiered (83.3% and 100%, respectively) and conventional (91% and 97.8%, respectively) MRI approaches were not significantly different. Assuming a 50% reduction in the payment for the initial limited MRI performed as a first step of the two-tiered approach, this approach would have resulted in 44.8% savings to the payer. A substantial reduction in the scanner utilization time from 4168 minutes to 1249 minutes for the two-tiered approach would have enabled increased throughput at the imaging facility. Although 27 (11%) patients would have been recalled for a comprehensive MRI study in the two-tiered approach, the average time spent in the scanner by each patient would have been less for the two-tiered approach (5.1 minutes vs 17.0 minutes)., Conclusion: A two-tiered approach to MRI can serve as a viable cost-effective alternative to the conventional approach.