Implementing an osteoarthritis management program to deliver guideline-driven care for knee and hip osteoarthritis in a U.S. academic health system.

Objective: Assess implementation feasibility and outcomes for an Osteoarthritis Management Program (OAMP) at an academic center. Design: This open study assessed an OAMP designed to deliver care in 1-5 individual or group visits across ≤12 months. Eligibility included adults with knee or hip osteoarthritis with ≥1 visit from 7/1/2017-1/15/2021. A multidisciplinary care team provided: education on osteoarthritis, self-management, exercise, weight loss; pharmacologic management; assessments of mood, sleep, quality of life, and diet. Clinic utilization and growth are reported through 2022. Patient outcomes of body mass index (BMI), pain, and function were analyzed using multivariable general linear models. OAMP outcomes were feasibility and sustainability. Results: Most patients were locally referred by primary care. 953 patients attended 2531 visits (average visits 2.16, treatment duration 187.9 days). Most were female (72.6%), older (62.1), white (91.1%), and had medical insurance (95.4%). Obesity was prevalent (84.7% BMI ≥30, average BMI 40.9), mean Charlson Comorbidity Index was 1.89, and functional testing was below average. Longitudinal modeling revealed statistically but not clinically significant pain reduction (4.4-3.9 on 0-10 scale, p â€‹= â€‹0.002). BMI did not significantly change (p â€‹= â€‹0.87). Higher baseline pain and BMI correlated with greater reductions in each posttreatment. Uninsured patients had shorter treatment duration. Increasing clinic hours (4-24 â€‹h weekly) and serving 953 patients over four years demonstrated OAMP sustainability. Conclusions: OAMP implementation was feasible and sustainable. Patients with high baseline pain and BMI were more likely to improve. Noninsurance was a barrier. These results contribute to understanding OAMP outcomes in U.S. healthcare.

Next-Generation Sequencing in the Diagnosis of Rare Pediatric Sinonasal Tumors.

The diagnosis of desmoid fibromatosis or other spindle cell tumors in the sinonasal region is very rare in children and needs to be thoroughly confirmed with immunohistochemical and/or molecular tests. We report 2 patients with such rare tumors and describe the use of next-generation sequencing in their evaluation. A 3-year-old female had a 4.4-cm midline nasal cavity mass involving the bony septum and extending into the base of the skull bilaterally. The moderate cellular fibroblastic proliferation revealed areas of thick keloid-like collagen bands and other areas with myxoid edematous stroma. Deep targeted sequencing identified a novel G34V mutation in the CTNNB1 gene consistent with desmoid fibromatosis. An 11-month-old male infant presented with a right nasal mass that extended through the cribriform plate into the anterior cranial fossa and involved the right ethmoid sinus and adjacent right orbit. Histology revealed an infiltrative atypical fibrous proliferation with focal calcifications that was negative for CTNNB1 and GNAS mutations. A novel RET E511K variant was identified in the tumor and later was also found in the germline and hence rendered of unknown significance. Both cases highlight the utility of next-generation sequencing in the evaluation of pediatric sinonasal spindle cell tumors that may have overlapping pathologic features. Reporting of rare or novel variants in tumor-only sequencing should be cautiously evaluated in children and pairing with germline sequencing may be needed to avoid the pitfall of assigning uncommon variants.

Colonic Adenocarcinoma at Advanced Stage in Adolescence: Report of 2 Cases.

BACKGROUND: Carcinoma of colon is rare in children and adolescents. The staging criteria of the carcinoma is the same as those for adults. However, the pathogenetic background in pediatric cases is different from adults and usually involves mismatch repair gene mutations or familial polyposis syndromes. Case report. We describe two adolescents diagnosed with advanced stage colon carcinoma and discuss the histological appearance, testing for mismatch repair genes and contrast- it with carcinoma occurring in the setting of familial polyposis syndrome. CONCLUSION: Colonic carcinoma occurring in pediatric patients should prompt a work-up for mismatch repair gene mutation status. Despite higher stage of presentation, some of the pediatric patients may respond favorably to chemotherapy and surgical resection.

Precision Medicine in Pediatric Cancer: Current Applications and Future Prospects.

Precision oncologic medicine is an emerging approach for cancer treatment that has recently taken giant steps in solid clinical practice. Recent advances in molecular diagnostics that can analyze the individual tumor's variability in genes have provided greater understanding and additional strategies to treat cancers. Although tumors can be tested by several molecular methods, the use of next-generation sequencing (NGS) has greatly facilitated our understanding of pediatric cancer and identified additional therapeutic opportunities. Pediatric tumors have a different genetic make-up, with a fewer number of actionable targets than adult tumors. Nevertheless, precision oncology in the pediatric population has greatly improved the survival of patients with leukemia and solid tumors. This review discusses the current status of pediatric precision oncology and the different clinical scenarios in which it can be effectively applied.

Prolonged Response in Patient With Multiply Relapsed B-cell Acute Lymphoblastic Leukemia and Monosomy-7 to Bortezomib, Lenalidomide, and Dexamethasone.

Isolated monosomy-7, a rare cytogenetic abnormality in patients with pediatric acute lymphoblastic leukemia (ALL), portends a worse prognosis. Despite improvements in treatment, outcomes for patients with relapsed ALL remain poor. Novel treatments adopted from the B-cell malignancy multiple myeloma may have a role in treatment of ALL. Bortezomib is one such agent currently in phase III trials for B and T ALL. This study presents a patient with B-cell ALL and monosomy-7 who relapsed off therapy. The combination of bortezomib, lenalidomide, and dexamethasone was used to attain remission before bone marrow transplant after conventional relapse therapy failed. A recurrence after bone marrow transplant was controlled for a prolonged period with the same therapy. The case supports the hypothesis that bortezomib, lenalidomide, and dexamethasone should be further explored in the treatment of B-cell ALL with monosomy-7.