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E. Ruffini

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    MS05 - Modern Management of Neuroendocrine Tumours (ID 22)

    • Event: WCLC 2013
    • Type: Mini Symposia
    • Track: Surgery
    • Presentations: 1
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      MS05.3 - Mediastinal Neuroendocrine Tumours (ID 478)

      14:00 - 15:30  |  Author(s): E. Ruffini

      • Abstract
      • Presentation
      • Slides

      INTRODUCTION Mediastinal Neuroendocrine Tumors occur most frequently in the thymus. Primary Thymic Neuroendocrine Tumors (NETs) are rare and highly aggressive neoplasms; a little more than 350 cases have been described in the literature, many of which are single case reports. We collected one of the largest series ever reported through a multicenter International study, with the aim to evaluate factors influencing survival and recurrence development in patients with Thymic NETs. MATERIAL AND METHODS A multicenter retrospective study of patients operated for NETs between 1989 and 2012 in 9 high-volume International Thoracic Surgery Institutions, was conducted. According to the International Thymic Malignancy Interest Group (ITMIG) outcome measures, primary and secondary outcome were Cause Specific Survival (CSS) and Disease Free Survival (DFS). Competing-risks regression models (Fine and Gray method), taking into account death by any causes as competing event, were used to identify the association between individual factors and tumor related death. Cox proportional hazards regression models were used to define association between individual factors and DFS, considering R0 cases only. Univariate and multivariate analyses were also performed. RESULTS There were 52 patients (41 males –79%-, median age 49 years). The tumor was asymptomatic in 22 cases (42%). Endocrine paraneoplastic syndromes were observed in 23 cases (44%): 13 Cushing’s syndrome and 10 MEN-1 syndrome. Well differentiated neuroendocrine carcinoma (Typical and Atypical Carcinoid) was the commonest histological subtype (30 cases –58%-). Eight patients (15%) received induction therapy (3 chemotherapy, 2 chemo+radiotherapy, 2 biological therapy and 1 chemo+radio+biological therapy), because of their radiological invasiveness. Median sternotomy was the commonest surgical approach (29 cases). The median tumor size was 8 cm (range 1 – 31 cm); a complete resection (R0) was achieved in 48 cases (92%). Advanced Masaoka-Koga stage (III-IV) was observed in 35 patients (67%). Postoperative treatment was offered to 26 (50%): radiotherapy in 17, chemotherapy in 1, chemo+radiotherapy in 5 and chemo+radio+biological therapy in 3 patient, respectively. Three, 5 and 10-year survival rates were 89%, 76% and 51% (Figure 1). Recurrences were observed in 32 cases (62%): 11 local, 10 intrathoracic and 11 distant. Cumulative incidence of recurrence was 41% at 2 years and 70% at 3 years (Figure 2). Variables influencing survival were: tumor size (p< 0.00) and recurrences (p=0.01). Independents DFS predictors were: age > 50 (p= 0.02), paraneoplastic syndromes (p=0.02), symptoms at presentation (p= 0.01) and poor differentiated histology (p= 0.04). CONCLUSIONS We have confirmed that Thymic NETs are rare mediastinal tumors presenting with an aggressive biological behavior; surgery remains the mainstay of treatment and it should be proposed whenever possible, even in case of advanced diseases. Recurrences are frequent, especially in the first years after operation. Survival is statistically related to the tumor size and to the presence of recurrences, whereas, surprisingly, it is not influenced by induction/adjuvant treatment. A global International effort is needed to collect larger series and to confirm these conclusions. Figure 1: Thymic NETs overall survival curveFigure 1Figure 2: Thymic NETs: cumulative incidence of tumor recurrencesFigure 2

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    P3.14 - Poster Session 3 - Mesothelioma (ID 197)

    • Event: WCLC 2013
    • Type: Poster Session
    • Track: Mesothelioma
    • Presentations: 1
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      P3.14-009 - High resolution study on survival of pleural mesothelioma from the records of the Piedmont Registry of Malignant Mesotheliomas - report on incident cases 2008-2009 (ID 2302)

      09:30 - 16:30  |  Author(s): E. Ruffini

      • Abstract

      The legacy of occupational and environmental asbestos exposure in Piedmont, Italy, is a continuing epidemic of pleural malignant mesothelioma (MPM). MPM still entails a poor prognosis, but progresses in its medical and surgical treatment have occurred and guidelines started to appear. We aimed to assess: (i) the appropriateness of treatment for MPM cases recently diagnosed in the Piedmont population, taking into account patients’ general conditions and disease stage at diagnosis, (ii) the end results of treatment.

      We exploited the Registry of Malignant Mesotheliomas (RMM) records to identify incident cases from 2008 to 2011. Patients diagnosed/treated in hospitals including a thoracic surgery unit and in the Casale Monferrato hospital were known to represent about 70% of all MPM incident cases and were included in this study, as for them not only clinical records on diagnostic procedures, but also on treatment and follow-up were completely retrieved by RMM. Vital status at 31/12/2012 was ascertained for all MPM cases. Current analyses were limited to incident cases 2008-2009, followed at least up to 3 years. Multinomial logistic regression was used to estimate the odds ratio (OR) of receiving a specific treatment (categories: cytoreductive surgery, CRS, chemo/immunotherapy, CIT, best supportive care, BSC), conditional on individual characteristics. Survival was assessed with univariate (Kaplan-Meier) and multivariate (Cox) methods.

      There were 297 MPM cases. Taking CIT as reference, the OR of receiving CRS was decreased by older age and low performance status (Table 1). That of receiving BSC alone was increased by older age and low performance status as well, and by non-epithelial histotype. Median survival was 13.6 months for patients receiving CIT and 18.3 for those undergoing CRS Figure 1

      In participating centres, MPM are currently treated in agreement with available guidelines, and treatment outcomes are consistent with expectations.