Individual risk evaluation for local recurrence and distant metastasis in Ewing sarcoma: A multistate model: A multistate model for Ewing sarcoma.

BACKGROUND: We investigated the effects of surgical margins, histological response, and radiotherapy on local recurrence (LR), distant metastasis (DM), and survival in Ewing sarcoma. PROCEDURE: Disease evolution was retrospectively studied in 982 patients with Ewing sarcoma undergoing surgery after chemotherapy using a multistate model with initial state surgery, intermediate states LR, pulmonary metastasis (DMpulm), other DM ± LR (DMother), and final state death. Effect of risk factors was estimated using Cox proportional hazard models. RESULTS: The median follow-up was 7.6 years (95% CI, 7.2-8.0). Risk factors for LR are pelvic location, HR 2.04 (1.10-3.80), marginal/intralesional resection, HR 2.28 (1.25-4.16), and radiotherapy, HR 0.52 (0.28-0.95); for DMpulm the risk factors are <90% necrosis, HR 2.13 (1.13-4.00), and previous pulmonary metastasis, HR 4.90 (2.28-8.52); for DMother are 90% to 99% necrosis, HR 1.56 (1.09-2.23), <90% necrosis, HR 2.66 (1.87-3.79), previous bone/other metastasis, HR 3.08 (2.03-4.70); and risk factors for death without LR/DM are pulmonary metastasis, HR 8.08 (4.01-16.29), bone/other metastasis, HR 10.23 (4.90-21.36), and <90% necrosis, HR 6.35 (3.18-12.69). Early LR (0-24 months) negatively influences survival, HR 3.79 (1.34-10.76). Once DMpulm/DMother arise only previous bone/other metastasis remain prognostic for death, HR 1.74 (1.10-2.75). CONCLUSION: Disease extent and histological response are risk factors for progression to DM or death. Tumor site and surgical margins are risk factors for LR. If disease progression occurs, previous risk factors lose their relevance. In case of isolated LR, time to recurrence is important for decision-making. Radiotherapy seems protective for LR especially in pelvic/axial. Low percentages of LR in extremity tumors and associated toxicity question the need for radiotherapy in extremity Ewing sarcoma.

Easy-to-use clinical tool for survival estimation in Ewing sarcoma at diagnosis and after surgery.

Accurate survival estimations in Ewing sarcoma are necessary to develop risk- and response adaptive treatment strategies allowing for early decision-making. We aim to develop an easy-to-use survival estimation tool from diagnosis and surgery. A retrospective study of 1314 Ewing sarcoma patients was performed. Associations between prognostic variables at diagnosis/surgery and overall survival (OS), were investigated using Kaplan-Meier and multivariate Cox models. Predictive accuracy was evaluated by cross-validation and Harrell C-statistics. Median follow-up was 7.9 years (95%CI 7.6-8.3). Independent prognostic factors at diagnosis were age, volume, primary tumor localization and disease extent. 5 risk categories (A-E) were identified with 5-year OS of 88% (86-94), 69% (64-74), 57% (50-64), 51% (42-60) and 28% (22-34) respectively. Harrell C-statistic was 0.70. Independent prognostic factors from surgery were age, volume, disease extent and histological response. In categories A-B, 5y OS increased to 92% (87-97) and 79% (71-87) respectively for 100% necrosis and decreased to 76% (67-85) and 62% (55-69) respectively for <100% necrosis. In categories C-E, 5y OS increased to 65% (55-75), 65% (52-78) and 52% (38-66) respectively for ≥90% necrosis and decreased to 38% (22-54), 11% (0-26) and 7% (0-19) respectively for <90% necrosis. We present an easy-to-use survival estimation tool from diagnosis in Ewing sarcoma based on age, volume, primary tumor localization and disease extent. Histological response is a strong additional prognostic factor for OS.

Strain bidimensional longitudinal na cardiomiopatia hipertrófica com fraçao de ejeçao preservada / Two-dimensional longitudinal strain in hypertrophic cardiomyopathy with preserved ejection fraction

Introdução: A nova tecnologia do strain bidimensional (st2d) permite uma análise mais precisa da função global e segmentar do ventrículo esquerdo (VE), incluindo sua porção apical, por ser ângulo-independente. Objetivo: Avaliar a função sistólica global do VE com o st-2d em pacientes (pcs) com Cardiomiopatia Hipertrófica (CMH). Métodos: Foram incluídos 21 pcs com CMH e fração de ejeção do VE preservada (G-CMH), e 21 pcs sem CMH, compondo o grupo controle (G-nl). Foi mensurado o st-2d longitudinal endocárdico global (st2D-L), pela técnica optical flow, dos 18 segmentos do VE, a partir de 3 cortes apicais. Empregou-se o teste t de Student para análise de variáveis contínuas, e o Qui-quadrado (Pearson) para variáveis não contínuas, considerando-se um nível de significância de 0,05. Resultados: Não foi observada diferença entre os grupos quanto à idade, sexo e pressão arterial sistólica e diastólica e frequência cardíaca. Dezesseis pcs do G-CMH apresentavam a forma assimétrica septal da hipertrofia, com envolvimento adicional ou não de paredes adjacentes (G-CMHs), e cinco apresentavam a forma apical da CMH (G-CMHap). Apesar da fração de ejeção do VE ser preservada nos 2 grupos, o st2D-L mostrou-se globalmente reduzido no G-CMH (14,6 + 4,3 vs 18,6 + 2,6% no G-C;p=0,009). No G-CMHs, notou-se valor significativamente menor do st2D-L em relação ao G-nl (13,6 + 3,9 versus 18,5 + 2,5%; p< 0,0002), não havendo diferença significativa entre o G-nl e o G-CMHap. Conclusão: O st2D-L mostrou-se significativamente reduzido na CMH em relação ao G-nl, indicando alteração precoce da função sistólica do VE na CMH, apesar de uma fração de ejeção preservada.

Introduction: The recent developed technology of two-dimensional strain (st2D) allows a more precise analysis of global and segmental function of the left ventricle (LV), including the apical region, due to its angle-independence. Objective: The aim of the study was to evaluate global LV systolic function with st2D in patients (pts) with Hypertrophic Cardiomyopathy (HCM). Methods: We included 21 pts with HCM and preserved LV ejection fraction (G-HCM), and 21 control pcs (G-nl). The global endocardial longitudinal st2D (st2D-L) of 18 myocardial LV segments was measured, by using “optical flow” technology, from the 3 apical views. The Student “t” test was used for analysis of the continuous variables, and the “Chi-square” (Pearson) for the non-continuous variables, considering a significance level of 0,05. Results: No significant difference was observed between the groups regarding age, sex, systolic and diastolic blood pressure, and heart rate. Sixteen pts from the G-HCM presented septal asymmetric hypertrophy, with additional involvement of adjacent walls or not (G-HCMs), and five showed the apical form of HCM (G-HCMap). Although LV ejection fraction was preserved in both groups, the st2D-L was significantly reduced in the G-HCM (14,.1 + 4,3 vs 18,6 + 2,6% in G-nl, p = 0.009). In the G-HCMs the st2D-L was lower compared to G-nl (13,6 + 3.9 vs 18,5 + 2.5%, p <0.0002), with no significant difference between G-nl and G-HCMap. Conclusion: The st2D-L was significantly reduced in pcs with HCM compared to G-nl, indicating early change of LV systolic function in HCM despite a preserved ejection fraction.

Per-rectal portal scintigraphy with technetium-99m pertechnetate for the early diagnosis of cirrhosis in patients with chronic hepatitis.

We evaluated the role of per-rectal portal scintigraphy with 99m-technetium pertechnetate (99m-Tc test) for early diagnosis of cirrhosis. Forty patients with biochemical evidence of chronic liver disease were studied. Laparobiopsy documented chronic active hepatitis (CAH) without cirrhosis in 22 of the patients and CAH with cirrhosis (CAHc) in 18 patients. Clinical or laboratory findings could not differentiate between CAH and CAHc. Twelve healthy volunteers served as controls. The results, expressed as shunt index (SI), i.e., the ratio between heart radioactivity and the sum of heart and liver radioactivity in the first 30 s of observation, were: controls 5.66 +/- 1.66, CAH 15.27 +/- 2.83 and CAHc 24.88 +/- 3.95. A significant difference between the mean SI values in the three groups studied (F = 142.71, p less than 0.0001) was observed. At values less than 17, our test showed a predictivity of 100% for cirrhosis exclusion, while at values higher than 19 the predictive positive value for a diagnosis of cirrhosis was 100%. Invasive diagnostic procedures should be performed only in patients with SI values between 17-19.

5-Aminosalicylic acid suppositories in the maintenance of remission in idiopathic proctitis or proctosigmoiditis: a double-blind placebo-controlled clinical trial.

Thirty patients with distal ulcerative colitis in remission (17 proctitis, 13 proctosigmoiditis) were randomly given either 5-aminosalicylic acid (5-ASA) or placebo suppositories, 400 mg bid. During the 1-yr follow-up, patients were assessed clinically every month, and flexible sigmoidoscopy with a rectal pinch biopsy specimen and laboratory data were carried out every 3 months. Two patients in the 5-ASA group chose to withdraw from the study, one relapsed, and 12 remained in remission. In the placebo group, one patient chose to withdraw, 11 relapsed, and three remained in remission. The cumulative remission rate at the 12th month was 92% in the 5-ASA group and 21% in the placebo group. Log rank test showed a significant difference in the relapse rate between the two groups (chi 2 = 14.26, p less than 0.001). No side effects were observed. We conclude that 5-ASA in suppository form (800 mg/day), administered for 1 yr, is safe and effective in maintaining remission of distal ulcerative colitis.