Miracle-dz/UniFAR-FTUs huggingface dataset:
Incarcerated umbilical hernia of unexpected origin: a primitive neuroectodermal tumor with early recurrence.

Introduction Primitive neuroectodermal tumor (PNET)/Ewing sarcoma (ES) is the second most common sarcoma of bone and soft tissue in children. PNET/ES usually occurs in adolescence or in early adulthood, with the age at diagnosis ranging from 14 to 22 years. An association between ES and hernias has been demonstrated, especially in children and young adults. However, the ES found in these patients were not in the umbilical or inguinal hernia region but in various parts of the body. Other risk factors such as smoking during pregnancy or farming could also be identified. Most patients with extraskeletal PNET/ES present with a nonpainful mass in the thoracic or paraspinal soft tissues, with the extremities and head and neck as less common sites of involvement. Apart from these sites, there have been isolated reports of peripheral PNET/ES arising from unusual locations in the body, including the lung, uterus, ovary, testis, pancreas, urinary bladder, parotid gland, skin, and subcutaneous tissue.
Role of reduced-intensity conditioning allogeneic hematopoietic stem-cell transplantation in older patients with de novo myelodysplastic syndromes: an international collaborative decision analysis.

PURPOSE Myelodysplastic syndromes (MDS) are clonal hematopoietic disorders that are more common in patients aged ≥ 60 years and are incurable with conventional therapies. Reduced-intensity conditioning (RIC) allogeneic hematopoietic stem-cell transplantation is potentially curative but has additional mortality risk. We evaluated RIC transplantation versus nontransplantation therapies in older patients with MDS stratified by International Prognostic Scoring System (IPSS) risk. PATIENTS AND METHODS A Markov decision model with quality-of-life utility estimates for different MDS and transplantation states was assessed. Outcomes were life expectancy (LE) and quality-adjusted life expectancy (QALE). A total of 514 patients with de novo MDS aged 60 to 70 years were evaluated. Chronic myelomonocytic leukemia, isolated 5q- syndrome, unclassifiable, and therapy-related MDS were excluded. Transplantation using T-cell depletion or HLA-mismatched or umbilical cord donors was also excluded. RIC transplantation (n = 132) stratified by IPSS risk was compared with best supportive care for patients with nonanemic low/intermediate-1 IPSS (n = 123), hematopoietic growth factors for patients with anemic low/intermediate-1 IPSS (n = 94), and hypomethylating agents for patients with intermediate-2/high IPSS (n = 165). RESULTS For patients with low/intermediate-1 IPSS MDS, RIC transplantation LE was 38 months versus 77 months with nontransplantation approaches. QALE and sensitivity analysis did not favor RIC transplantation across plausible utility estimates. For intermediate-2/high IPSS MDS, RIC transplantation LE was 36 months versus 28 months for nontransplantation therapies. QALE and sensitivity analysis favored RIC transplantation across plausible utility estimates. CONCLUSION For patients with de novo MDS aged 60 to 70 years, favored treatments vary with IPSS risk. For low/intermediate-1 IPSS, nontransplantation approaches are preferred. For intermediate-2/high IPSS, RIC transplantation offers overall and quality-adjusted survival benefit.
PARAMOUNT: Final overall survival results of the phase III study of maintenance pemetrexed versus placebo immediately after induction treatment with pemetrexed plus cisplatin for advanced nonsquamous non-small-cell lung cancer.

PURPOSE In the phase III PARAMOUNT trial, pemetrexed continuation maintenance therapy reduced the risk of disease progression versus placebo (hazard ratio [HR], 0.62; 95% CI, 0.49 to 0.79; P < .001). Here we report final overall survival (OS) and updated safety data. PATIENTS AND METHODS In all, 939 patients with advanced nonsquamous non-small-cell lung cancer (NSCLC) received four cycles of pemetrexed-cisplatin induction therapy; then, 539 patients with no disease progression and Eastern Cooperative Oncology Group performance status 0 or 1 were randomly assigned (2:1) to maintenance pemetrexed (500 mg/m(2) on day 1 of 21-day cycles; n = 359) or placebo (n = 180). Log-rank test compared OS between arms as measured from random assignment (α = .0498). RESULTS The mean number of maintenance cycles was 7.9 (range, one to 44) for pemetrexed and 5.0 (range, one to 38) for placebo. After 397 deaths (pemetrexed, 71%; placebo, 78%) and a median follow-up of 24.3 months for alive patients (95% CI, 23.2 to 25.1 months), pemetrexed therapy resulted in a statistically significant 22% reduction in the risk of death (HR, 0.78; 95% CI, 0.64 to 0.96; P = .0195; median OS: pemetrexed, 13.9 months; placebo, 11.0 months). Survival on pemetrexed was consistently improved for all patient subgroups, including induction response: complete/partial responders (n = 234) OS HR, 0.81; 95% CI, 0.59 to 1.11 and stable disease (n = 285) OS HR, 0.76; 95% CI, 0.57 to 1.01). Postdiscontinuation therapy use was similar: pemetrexed, 64%; placebo, 72%. No new safety findings emerged. Drug-related grade 3 to 4 anemia, fatigue, and neutropenia were significantly higher in pemetrexed-treated patients. CONCLUSION Pemetrexed continuation maintenance therapy is well-tolerated and offers superior OS compared with placebo, further demonstrating that it is an efficacious treatment strategy for patients with advanced nonsquamous NSCLC and good performance status who did not progress during pemetrexed-cisplatin induction therapy.
ATLAS: randomized, double-blind, placebo-controlled, phase IIIB trial comparing bevacizumab therapy with or without erlotinib, after completion of chemotherapy, with bevacizumab for first-line treatment of advanced non-small-cell lung cancer.

PURPOSE This phase III trial was performed to assess the potential benefit of adding maintenance erlotinib to bevacizumab after a first-line chemotherapy regimen with bevacizumab for advanced non-small-cell lung cancer (NSCLC). PATIENTS AND METHODS One thousand one hundred forty-five patients with histologically or cytologically confirmed NSCLC (stage IIIB with malignant pleural effusion, stage IV, or recurrent) received four cycles of chemotherapy plus bevacizumab. Seven hundred forty-three patients without disease progression or significant toxicity were then randomly assigned (1:1) to bevacizumab (15 mg/kg, day 1, 21-day cycle) plus either placebo or erlotinib (150 mg per day). The primary end point was progression-free survival (PFS). RESULTS Median PFS from time of random assignment was 3.7 months with bevacizumab/placebo and 4.8 months with bevacizumab/erlotinib (hazard ratio [HR], 0.71; 95% CI, 0.58 to 0.86; P < .001). Median overall survival (OS) times from random assignment were 13.3 and 14.4 months with bevacizumab/placebo and bevacizumab/erlotinib, respectively (HR, 0.92; 95% CI, 0.70 to 1.21; P = .5341). During the postchemotherapy phase, there were more adverse events (AEs) overall, more grade 3 and 4 AEs (mainly rash and diarrhea), more serious AEs, and more AEs leading to erlotinib/placebo discontinuation in the bevacizumab/erlotinib arm versus the bevacizumab/placebo arm. The incidence of AEs leading to bevacizumab discontinuation was similar in both treatment arms. CONCLUSION The addition of erlotinib to bevacizumab significantly improved PFS but not OS. Although generally well tolerated, the modest impact on survival and increased toxicity associated with the addition of erlotinib to bevacizumab maintenance mean that this two-drug maintenance regimen will not lead to a new postchemotherapy standard of care.
Hairy-cell leukemia presenting as lytic bone lesions.

Case Report A 56-year-old man with chronic low back and intermittent hip pain presented to our center with worsening symptoms. He reported no constitutional symptoms. Physical examination revealed tenderness on percussion of the lower dorsal and lumbar spine. His CBC included hemoglobin, 143 g/L; WBC, 6.5 10/L with a normal differential; and platelets, 112 10/L. Serum lactate dehydrogenase, calcium, PO4, albumin, uric acid, 2-microglobulin, and paraprotein levels were normal. Plain radiographs of the dorsal/lumbar spine, pelvis, and femora were unremarkable except for mild wedge compression of the D9 vertebral body. A magnetic resonance imaging (MRI) scan of the spine showed abnormal signal intensity in the vertebral bodies and posterior elements of vertebrae D3 through D6, D7, and D9 through L1, with an altered intramedullary signal in vertebrae L1, L2, L5, S2, and S3. An MRI scan of the pelvis showed increased T2 signal intensity that was widely distributed throughout the pelvis and sacrum, including both iliac crests, the posterior left ilium, both supra-acetabular areas, and within the femoral head and neck bilaterally. The marrow T1 signal was decreased. A computed tomography scan showed lytic areas within the vertebral bodies of D7 and D9 and a small lytic lesion within the right ilium that suggested metastases. Bone scintigraphy showed some uptake at D9 but no other changes to correlate with the computed tomography scan findings. Abdominal ultrasound revealed hepatic steatosis but no splenomegaly. A blood film and bone marrow aspirate with flow cytometric immunophenotyping were interpreted as essentially normal. Retrospective review confirmed the absence of hairy cells on the blood film, with only rare cells suggestive of hairy cell leukemia (HCL) in the bone marrow aspirate and touch imprints. The hematoxylin and eosin slides of the bone marrow biopsy appeared free of disease (Fig 1A), but subsequent CD20 immunohistochemistry revealed a sparse interstitial infiltrate that was suggestive of HCL (Fig 1B). Hematoxylin and eosin staining of an open biopsy of the left iliac crest revealed a diffuse infiltrate of lymphocytes with cleaved nuclei and abundant cytoplasm (Fig 1C), and immunohistochemistry was positive for CD20, CD10, BCL2, and cyclin D1, and negative for CD3, CD5, and CD138. Flow cytometry revealed coexpression of CD19, CD20, CD10, CD11c, CD25, and CD103, which established a diagnosis of hairy cell leukemia. Enzyme cytochemistry confirmed tartrateresistant acid phosphatase positivity, special stains confirmed reticulin fibrosis, and polymerase chain reaction confirmed a clonal immunoglobulin heavy chain gene rearrangement. Fluorescent in situ hybridization was negative for translocations t(11;14) and t(14;18). Bone biopsy was referred in consultation to P. Kurtin at the Mayo Clinic, who confirmed the diagnosis of HCL and kindly performed and reported annexin A1 immunocytochemistry, which was positive and consistent with HCL. Analysis of the BRAF gene by shifted termination assay and capillary electrophoresis (Applied Biosystems BRAF Mutation A
Cricket: Injuries, Rehabilitation, and Training

Cricket is a popular team sport played throughout the world. The 3 unique aspects of the game—bowling, batting, and fielding—can each be associated with injury. Specific injuries to the shoulder are rarer than would be expected from a throwing sport because of the nature of the bowling technique. It is more common for an injury to be caused by throwing and then to be aggravated by bowling. Good surveillance and concentration on technique by coaches have highlighted the etiology of many of the injury patterns seen. It is uncommon for an injury from cricket to require surgery. The implementation of rule changes together with better coaching, early accurate diagnosis, and appropriate rehabilitation have helped players avoid and, if they occur, properly manage their injuries.
Deficits in physical function among young childhood cancer survivors.

PURPOSE Childhood cancer survivors (CCSs) are at risk for physical disability. The aim of this investigation was to characterize and compare physical performance among CCSs and a group of siblings age < 18 years and determine if diagnosis, treatment, and physical activity levels were associated with lower performance scores. METHODS CCSs ≥ 5 years from diagnosis and a sibling comparison group were recruited and evaluated for strength, mobility, and fitness. Physical performance measures were compared in regression models between survivors and siblings by diagnosis and among survivors by treatment exposures and physical activity levels. RESULTS CCSs (n = 183; mean age ± standard deviation [SD], 13.5 ± 2.5 years; 53% male) scored lower than siblings (n = 147; mean age ± SD, 13.4 ± 2.4 years; 50% male) on lower-extremity strength testing, the timed up-and-go (TUG) test, and the 6-minute walk (6MW) test, despite reporting similar levels and types of habitual physical activity. The lowest scores were prevalent among survivors of CNS tumors and bone and soft tissue sarcomas on strength testing (score ± SD: CNS tumors, 76.5 ± 4.7; sarcoma 67.1 ± 7.2 v siblings, 87.3 ± 2.4 Newton-meters quadricep strength at 90° per second; P = .04 and .01, respectively) and among CNS tumor survivors on the TUG (score ± SD: 5.1 ± 0.1 v siblings, 4.4 ± 0.1 seconds; P < .001) and 6MW tests (score ± SD: 533.3 ± 15.6 v siblings, 594.1 ± 8.3 m; P < .001). CONCLUSION CCSs may have underlying physiologic deficits that interfere with function that cannot be completely overcome by participation in regular physical activity. These survivors may need referral for specialized exercise interventions in addition to usual counseling to remain physically active.
Subgroup-specific prognostic implications of TP53 mutation in medulloblastoma.

PURPOSE Reports detailing the prognostic impact of TP53 mutations in medulloblastoma offer conflicting conclusions. We resolve this issue through the inclusion of molecular subgroup profiles. PATIENTS AND METHODS We determined subgroup affiliation, TP53 mutation status, and clinical outcome in a discovery cohort of 397 medulloblastomas. We subsequently validated our results on an independent cohort of 156 medulloblastomas. RESULTS TP53 mutations are enriched in wingless (WNT; 16%) and sonic hedgehog (SHH; 21%) medulloblastomas and are virtually absent in subgroups 3 and 4 tumors (P < .001). Patients with SHH/TP53 mutant tumors are almost exclusively between ages 5 and 18 years, dramatically different from the general SHH distribution (P < .001). Children with SHH/TP53 mutant tumors harbor 56% germline TP53 mutations, which are not observed in children with WNT/TP53 mutant tumors. Five-year overall survival (OS; ± SE) was 41% ± 9% and 81% ± 5% for patients with SHH medulloblastomas with and without TP53 mutations, respectively (P < .001). Furthermore, TP53 mutations accounted for 72% of deaths in children older than 5 years with SHH medulloblastomas. In contrast, 5-year OS rates were 90% ± 9% and 97% ± 3% for patients with WNT tumors with and without TP53 mutations (P = .21). Multivariate analysis revealed that TP53 status was the most important risk factor for SHH medulloblastoma. Survival rates in the validation cohort mimicked the discovery results, revealing that poor survival of TP53 mutations is restricted to patients with SHH medulloblastomas (P = .012) and not WNT tumors. CONCLUSION Subgroup-specific analysis reconciles prior conflicting publications and confirms that TP53 mutations are enriched among SHH medulloblastomas, in which they portend poor outcome and account for a large proportion of treatment failures in these patients.
Influence of Therapy Ball Seats on Attentional Ability in Children with Attention Deficit/Hyperactivity Disorder

[Purpose] The aim of this study was to investigate the P300 in electroencephalography (EEG) and the reaction time in children with attention deficit hyperactivity disorder (ADHD) during an auditory oddball task when sitting on a classroom chair or therapy ball. [Subjects] Fifteen ADHD children with a mean age of 8.6 ± 2.1 years and 14 healthy children with a mean age of 8.7 ± 2.0 years were used as subjects in this study. [Methods] All subjects were asked to sit on a chair or therapy ball and perform simultaneously the auditory oddball task. A portable 40-channel EEG system and a sound operating system were employed to record and analyze the EEG and button reaction time signals. [Results] When seated on the chair, the ADHD group had a significantly longer reaction time than the control group. ADHD children seated on a therapy ball showed a significant improvement in reaction time compared with when seated on the chair. In the parietal lobe, the ADHD group had a significantly delayed P300 latency during chair seating compared with the control group. The ADHD group showed a significantly shorter P300 latency time when seated on a therapy ball. [Conclusion] The therapy ball has a significant advantage for enhancing the attentional ability in children with ADHD.
Trastuzumab-related cardiotoxicity among older patients with breast cancer.

PURPOSE The use of trastuzumab in the adjuvant setting improves outcomes but is associated with cardiotoxicity manifested as congestive heart failure (CHF). The rates and risk factors associated with trastuzumab-related CHF among older patients are unknown. PATIENTS AND METHODS Breast cancer patients at least 66 years old with full Medicare coverage, diagnosed with stage I-III breast cancer between 2005 and 2009, and treated with chemotherapy were identified in the SEER-Medicare and in the Texas Cancer Registry-Medicare databases. The rates and risk factors associated with CHF were evaluated. Chemotherapy, trastuzumab use, comorbidities, and CHF were identified using International Classification of Diseases, version 9, and Healthcare Common Procedure Coding System codes. Analyses included descriptive statistics and Cox proportional hazards models. RESULTS In total, 9,535 patients were included, of whom 2,203 (23.1%) received trastuzumab. Median age of the entire cohort was 71 years old. Among trastuzumab users, the rate of CHF was 29.4% compared with 18.9% in nontrastuzumab users (P < .001). Trastuzumab users were more likely to develop CHF than nontrastuzumab users (hazard ratio [HR], 1.95; 95% CI, 1.75 to 2.17). Among trastuzumab-treated patients, older age (age > 80 years; HR, 1.53; 95% CI, 1.16 to 2.10), coronary artery disease (HR, 1.82; 95% CI, 1.34 to 2.48), hypertension (HR, 1.24; 95% CI, 1.02 to 1.50), and weekly trastuzumab administration (HR, 1.33; 95% CI, 1.05 to 1.68) increased the risk of CHF. CONCLUSION In this large cohort of older breast cancer patients, the rates of trastuzumb-related CHF are higher than those reported in clinical trials. Among patients treated with trastuzumab, those with cardiac comorbidities and older age may be at higher risk. Further studies need to confirm the role that the frequency of administration plays in the development of trastuzumab-related CHF.
Phase I study of quizartinib administered daily to patients with relapsed or refractory acute myeloid leukemia irrespective of FMS-like tyrosine kinase 3-internal tandem duplication status.

PURPOSE FMS-like tyrosine kinase 3-internal tandem duplication (FLT3-ITD) mutations in acute myeloid leukemia (AML) are associated with early relapse and poor survival. Quizartinib potently and selectively inhibits FLT3 kinase activity in preclinical AML models. PATIENTS AND METHODS Quizartinib was administered orally at escalating doses of 12 to 450 mg/day to 76 patients (median age, 60 years; range, 23 to 86 years; with a median of three prior therapies [range, 0 to 12 therapies]), enrolled irrespective of FLT3-ITD mutation status in a phase I, first-in-human study in relapsed or refractory AML. RESULTS Responses occurred in 23 (30%) of 76 patients, including 10 (13%) complete remissions (CR) of any type (two CRs, three CRs with incomplete platelet recovery [CRp], five CRs with incomplete hematologic recovery [CRi]) and 13 (17%) with partial remissions (PRs). Of 17 FLT3-ITD-positive patients, nine responded (53%; one CR, one CRp, two CRis, five PRs); of 37 FLT3-ITD-negative patients, five responded (14%; two CRps, three PRs); of 22 with FLT3-ITD-indeterminate/not tested status, nine responded (41%; one CR, three CRis, five PRs). Median duration of response was 13.3 weeks; median survival was 14.0 weeks. The most common drug-related adverse events (> 10% incidence) were nausea (16%), prolonged QT interval (12%), vomiting (11%), and dysgeusia (11%); most were ≤ grade 2. The maximum-tolerated dose was 200 mg/day, and the dose-limiting toxicity was grade 3 QT prolongation. FLT3-ITD phosphorylation was completely inhibited in an in vitro plasma inhibitory assay. CONCLUSION Quizartinib has clinical activity in patients with relapsed/refractory AML, particularly those with FLT3-ITD, and is associated with an acceptable toxicity profile.
Modifiable risk factors and major cardiac events among adult survivors of childhood cancer.

PURPOSE To evaluate the relative contribution of modifiable cardiovascular risk factors on the development of major cardiac events in aging adult survivors of childhood cancer. PATIENTS AND METHODS Among 10,724 5-year survivors (median age, 33.7 years) and 3,159 siblings in the Childhood Cancer Survivor Study, the prevalence of hypertension, diabetes mellitus, dyslipidemia, and obesity was determined, along with the incidence and severity of major cardiac events such as coronary artery disease, heart failure, valvular disease, and arrhythmia. On longitudinal follow-up, rate ratios (RRs) of subsequent cardiac events associated with cardiovascular risk factors and cardiotoxic therapy were assessed in multivariable Poisson regression models. RESULTS Among survivors, the cumulative incidence of coronary artery disease, heart failure, valvular disease, and arrhythmia by 45 years of age was 5.3%, 4.8%, 1.5%, and 1.3%, respectively. Two or more cardiovascular risk factors were reported by 10.3% of survivors and 7.9% of siblings. The risk for each cardiac event increased with increasing number of cardiovascular risk factors (all P(trend) < . 001). Hypertension significantly increased risk for coronary artery disease (RR, 6.1), heart failure (RR, 19.4), valvular disease (RR, 13.6), and arrhythmia (RR, 6.0; all P values < .01). The combined effect of chest-directed radiotherapy plus hypertension resulted in potentiation of risk for each of the major cardiac events beyond that anticipated on the basis of an additive expectation. Hypertension was independently associated with risk of cardiac death (RR, 5.6; 95% CI, 3.2 to 9.7). CONCLUSION Modifiable cardiovascular risk factors, particularly hypertension, potentiate therapy-associated risk for major cardiac events in this population and should be the focus of future interventional studies.
Invited. Ultrafast MR imaging of the abdomen: Echo planar imaging and diffusion‐weighted imaging

Ultrafast MRI technique has become available with the introduction of new generation MR scanners for abdominal imaging. However, there is no consensus about the optimal imaging acquisition at the present time. Because single shot echo planar imaging (EPI) technique is based on high technology and had just applied in clinical imaging, further clinical investigation will be needed. Currently, the hypersensitivity to magnetic inhomogeneity and local magnetic susceptibility and the low spatial resolution may limit the widespread application of EPI technique. In addition to providing information for morphologic diagnosis, EPI will be more widely used for functional and qualitative diagnosis. Diffusion‐weighted imaging can be used for differentiation of solid tumors according to their different cellular construction, evaluation of cystic lesions based on the different viscosity of their contents, and assessment of diffused pathologic changes in the parenchyma of solid organs. In addition to the previous parameters such as proton density and T1 and T2 values, diffusion factors may provide important information for the qualitative and dynamic evaluation of abdominal pathologic changes. Even though there are many difficulties that must be solved for diffusion‐weighted imaging, a more wide application of this technique is expected through technologic improvement.