El discurso político-institucional sobre las enfermedades poco frecuentes: las agrupaciones valorativas en las iniciativas parlamentarias

The 135 parliamentary initiatives (IP) on Rare Diseases (EPF) represent partially the political discourse say and attitude towards these pathologies, which according to the Government and the associations affect approximately three million citizens. The Critical Discourse Analysis applied of these initiatives and their corresponding sub-typologies (Control Function, Political Orientation Function and Institutional Declaration) throughout 7 legislatures allows us to observe the following: the political appropriation of the Federación Española de Enfermedades Raras (FEDER)’s message concerning rare diseases and the use of a series of valuation groups that express vagueness, a tenuous vindication and a lack of parliamentary responsibility. Also, these valuation groups show an apparent involvement that masks a distancing towards the disease, the patients and their families.Las 135 Iniciativas parlamentarias (IP) sobre las Enfermedades Poco Frecuentes (EPF) son parte de la representación del hacer y del decir del discurso político sobre estas patologías, que para el Gobierno y el movimiento asociativo afectan aproximadamente a tres millones de ciudadanos. El Análisis Crítico del Discurso aplicado a estas iniciativas y a sus correspondientes tipologías (función de control, función de orientación política y declaración institucional) a lo largo de siete legislaturas nos permite observar la apropiación política de la voz de la Federación Española de Enfermedades Raras (FEDER) sobre las EPF y el empleo de una serie de agrupaciones valorativas que expresan vaguedad, una tenue reivindicación y una falta de responsabilidad parlamentaria. Estas, además, demuestran una aparente implicación a la vez que un distanciamiento hacia la enfermedad, los pacientes y sus familiares

El discurso político-institucional sobre las enfermedades poco frecuentes: las agrupaciones valorativas en las iniciativas parlamentarias

The 135 parliamentary initiatives (IP) on Rare Diseases (EPF) represent partially the political discourse say and attitude towards these pathologies, which according to the Government and the associations affect approximately three million citizens. The Critical Discourse Analysis applied of these initiatives and their corresponding sub-typologies (Control Function, Political Orientation Function and Institutional Declaration) throughout 7 legislatures allows us to observe the following: the political appropriation of the Federación Española de Enfermedades Raras (FEDER)’s message concerning rare diseases and the use of a series of valuation groups that express vagueness, a tenuous vindication and a lack of parliamentary responsibility. Also, these valuation groups show an apparent involvement that masks a distancing towards the disease, the patients and their families.Las 135 Iniciativas parlamentarias (IP) sobre las Enfermedades Poco Frecuentes (EPF) son parte de la representación del hacer y del decir del discurso político sobre estas patologías, que para el Gobierno y el movimiento asociativo afectan aproximadamente a tres millones de ciudadanos. El Análisis Crítico del Discurso aplicado a estas iniciativas y a sus correspondientes tipologías (función de control, función de orientación política y declaración institucional) a lo largo de siete legislaturas nos permite observar la apropiación política de la voz de la Federación Española de Enfermedades Raras (FEDER) sobre las EPF y el empleo de una serie de agrupaciones valorativas que expresan vaguedad, una tenue reivindicación y una falta de responsabilidad parlamentaria. Estas, además, demuestran una aparente implicación a la vez que un distanciamiento hacia la enfermedad, los pacientes y sus familiares

The sacral chordoma margin

Objective: Aim of the manuscript is to discuss how to improve margins in sacral chordoma. Background: Chordoma is a rare neoplasm, arising in half cases from the sacrum, with reported local failure in >50% after surgery. Methods: A multidisciplinary meeting of the \u201cChordoma Global Consensus Group\u201d was held in Milan in 2017, focusing on challenges in defining and achieving optimal margins in chordoma with respect to surgery, definitive particle radiation therapy (RT) and medical therapies. This review aims to report on the outcome of the consensus meeting and to provide a summary of the most recent evidence in this field. Possible new ways forward, including on-going international clinical studies, are discussed. Results: En-bloc tumor-sacrum resection is the cornerstone of treatment of primary sacral chordoma, aiming to achieve negative microscopic margins. Radical definitive particle therapy seems to offer a similar outcome compared to surgery, although confirmation in comparative trials is lacking; besides there is still a certain degree of technical variability across institutions, corresponding to different fields of treatment and different tumor coverage. To address some of these questions, a prospective, randomized international study comparing surgery versus definitive high-dose RT is ongoing. Available data do not support the routine use of any medical therapy as (neo)adjuvant/cytoreductive treatment. Conclusion: Given the significant influence of margins status on local control in patients with primary localized sacral chordoma, the clear definition of adequate margins and a standard local approach across institutions for both surgery and particle RT is vital for improving the management of these patients

The sacral chordoma margin

Objective Aim of the manuscript is to discuss how to improve margins in sacral chordoma. Background Chordoma is a rare neoplasm, arising in half cases from the sacrum, with reported local failure in >50% after surgery. Methods A multidisciplinary meeting of the “Chordoma Global Consensus Group” was held in Milan in 2017, focusing on challenges in defining and achieving optimal margins in chordoma with respect to surgery, definitive particle radiation therapy (RT) and medical therapies. This review aims to report on the outcome of the consensus meeting and to provide a summary of the most recent evidence in this field. Possible new ways forward, including on-going international clinical studies, are discussed. Results En-bloc tumor-sacrum resection is the cornerstone of treatment of primary sacral chordoma, aiming to achieve negative microscopic margins. Radical definitive particle therapy seems to offer a similar outcome compared to surgery, although confirmation in comparative trials is lacking; besides there is still a certain degree of technical variability across institutions, corresponding to different fields of treatment and different tumor coverage. To address some of these questions, a prospective, randomized international study comparing surgery versus definitive high-dose RT is ongoing. Available data do not support the routine use of any medical therapy as (neo)adjuvant/cytoreductive treatment. Conclusion Given the significant influence of margins status on local control in patients with primary localized sacral chordoma, the clear definition of adequate margins and a standard local approach across institutions for both surgery and particle RT is vital for improving the management of these patients