44 research outputs found

    Systemic therapies in advanced epithelioid haemangioendothelioma: A retrospective international case series from the World Sarcoma Network and a review of literature

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    [Background] This observational, retrospective effort across Europe, US, Australia, and Asia aimed to assess the activity of systemic therapies in EHE, an ultra-rare sarcoma, marked by WWTR1-CAMTA1 or YAP1-TFE3 fusions.[Methods] Twenty sarcoma reference centres contributed data. Patients with advanced EHE diagnosed from 2000 onwards and treated with systemic therapies, were selected. Local pathologic review and molecular confirmation were required. Radiological response was retrospectively assessed by local investigators according to RECIST. Progression free survival (PFS) and overall survival (OS) were estimated by Kaplan-Meier method.[Results] Overall, 73 patients were included; 21 had more than one treatment. Thirty-three patients received anthracyclines regimens, achieving 1 (3%) partial response (PR), 25 (76%) stable disease (SD), 7 (21%) progressive disease (PD). The median (m-) PFS and m-OS were 5.5 and 14.3 months respectively. Eleven patients received paclitaxel, achieving 1 (9%) PR, 6 (55%) SD, 4 (36%) PD. The m-PFS and m-OS were 2.9 and 18.6 months, respectively. Twelve patients received pazopanib, achieving 3 (25%) SD, 9 (75%) PD. The m-PFS and m-OS were.2.9 and 8.5 months, respectively. Fifteen patients received INF-α 2b, achieving 1 (7%) PR, 11 (73%) SD, 3 (20%) PD. The m-PFS and m-OS were 8.9 months and 64.3, respectively. Among 27 patients treated with other regimens, 1 PR (ifosfamide) and 9 SD (5 gemcitabine +docetaxel, 2 oral cyclophosphamide, 2 others) were reported.[Conclusion] Systemic therapies available for advanced sarcomas have limited activity in EHE. The identification of new active compounds, especially for rapidly progressive cases, is acutely needed.Peer reviewe

    Pulmonary nodules: volume repeatability at multidetector CT lung cancer screening.

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    To assess in vivo volumetric repeatability of an automated software algorithm in pulmonary nodules detected during a lung cancer screening trial.This study was approved by an institutional review board. Written informed consent was obtained from all participants. Data were collected from the Multicentric Italian Lung Detection project, a randomized controlled lung cancer screening trial. The first 1236 consecutive baseline computed tomographic (CT) studies performed at the Istituto Nazionale Tumori of Milan were evaluated. Among the enrolled participants, those who underwent repeat low-dose CT after 3 months and had at least one indeterminate nodule with a volume of more than 60 mm(3) (diameter of 4.8 mm or greater) were considered. Nonsolid, part-solid, and pleural-based nodules were excluded from this study. A descriptive analysis was performed by calculating means and standard deviations of nodule volumes at three assessment times (at baseline and 3 and 12 months later). The volume measurement repeatability was determined by using the approach described by Bland and Altman.One hundred one subjects (70 men, 31 women; mean age, 58 years) with 233 eligible nodules (mean volume, 98.3 mm(3); range, 5-869 mm(3)) were identified. The 95\% confidence interval for difference in measured volumes was in the range of +/-27\%. About 70\% of measurements had a relative difference in nodule volume of less than 10\%. No malignant lesions were registered during the follow-up of these subjects.Semiautomatic volumetry is sufficiently accurate and repeatable and may be useful in assisting with lung nodule management in a lung cancer screening program

    Prognostic nomogram in patients with metastatic adenoid cystic carcinoma of the salivary glands

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    BACKGROUND: Distant metastases in adenoid cystic carcinoma (ACC) are common. There is no consensus on the management of metastatic disease because no therapeutic approach has demonstrated improvement in overall survival (OS) and because of prolonged life expectancy. The aim of this study is to build and validate a prognostic nomogram for metastatic ACC patients. METHODS: The study end-point was OS, measured from the date of first metastatic presentation to death/last follow-up. A retrospective analysis including metastatic ACC patients was performed to build the prognostic nomogram at the INT (Milan, Italy). The model was validated on an independent cohort of patients with similar characteristics treated at Leuven (Belgium). Outcome data and covariates were modelled by resorting to a random forest method. This machine-learning approach was used to guide and benchmark the subsequent use of more conventional modelling methods. Cox model performance was assessed in terms of discrimination (Harrell's c-index). RESULTS: Two hundred ninety-eight patients with metastatic ACC (testing set 259 INT, validation set 39 Leuven) were studied. Akaike Information Criterion-based backward selection yielded a 5-factor model showing a bias-corrected c-index of 0.730. Five independent prognostic factors were found: gender, disease-free interval and presence of lung, liver or bone metastases. Nomogram discrimination in the validation series was c = 0.701. CONCLUSION: This retrospective analysis allowed the building of an externally validated prognostic nomogram. This tool might help clinicians to discriminate patients requiring prompt management from who can benefit from a 'watchful waiting'. In addition, the nomogram might be useful to stratify patients in clinical trials.status: publishe

    Hope in cancer patients: the relational domain as a crucial factor

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    15Aims and background: Hope is crucial for patients with cancer. We explored the determinants of hope in patients with cancer using a questionnaire administered over the course of 1 day to an unselected sample of patients at an Italian cancer center. Methods: A team of oncologists, statisticians, and chaplains developed a questionnaire with medical, psychological, spiritual, and religious content. A cross-sectional study was conducted on 320 patients who answered the questionnaire. Results: In the group of participants, 92.8% had a religious belief. Women, patients with limited formal education, and believers were more hopeful. Patients placed trust in God, their partners and children, scientific research, and doctors. On univariate and multivariate analysis, hope was found sensitive to patients' sharing their experiences with others (including family and friends), their positive perception of the people around them, and their relationship with doctors and nurses. Conclusions: If validated in further studies, these results support the notion that a patient with cancer's sense of hope is sensitive to the quality of relationships with caregivers. This may be important to health care organization and resource allocation.nonenoneProserpio T; Ferrari A; Lo Vullo S; Massimino M; Clerici CA; Veneroni L; Bresciani C; Casali PG; Ferrari M; Bossi P; Galmozzi G; Pierantozzi A; Licitra L; Marceglia S; Mariani LProserpio, T; Ferrari, A; Lo Vullo, S; Massimino, M; Clerici, Ca; Veneroni, L; Bresciani, C; Casali, Pg; Ferrari, M; Bossi, P; Galmozzi, G; Pierantozzi, A; Licitra, L; Marceglia, S; Mariani,
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