Fallo ovárico prematuro en supervivientes a un tumor sólido: puntos clave de manejo / Primary ovarian insufficiency in cancer survivors: Keys to optimal management

Introducción: La insuficiencia ovárica prematura (POI) conlleva importante morbilidad, causando infertilidad, disfunción sexual, disminución de la densidad ósea, riesgo cardiovascular, alteraciones emocionales y mortalidad precoz. Objetivo: Conocer la incidencia y el manejo actual de la POI en supervivientes a un tumor sólido en la infancia y/o adolescencia en nuestro medio. Material y métodos: Estudio observacional multicéntrico. Mujeres entre 12 y 18 años con diagnóstico de tumor sólido y criterios clínicos y/o analíticos de POI. El riesgo se estima según los criterios «The Pediatric Initiative Network of the Oncofertility Consortium». Resultados: Incidencia de 1,5 (30 casos de POI). Edad media 14±2,09. Los tumores sólidos que más se asociaron a la POI fueron: sarcoma de Ewing, tumores cerebrales y germinales. El 83% de los casos no realizó preservación previa al tratamiento. Un 63% no referían menarquia al diagnóstico de la POI. El 97% cumplían criterios de alto riesgo de toxicidad gonadal, a pesar de ello el 47% no realizó ninguna vigilancia previa al diagnóstico. La mediana de tiempo tras el diagnóstico y la aparición del evento es de 43,5 y 29,5 meses tras finalizar tratamiento. Las curvas de Kaplan-Meier, muestran que al 30% de los casos, aparecen en los 2 años tras el diagnóstico y las mujeres con estadio puberal 1 desarrollan insuficiencia más tardíamente que aquellas con estadio 5. Conclusiones: El seguimiento de mujeres en riesgo de la POI, es susceptible de mejora. Las herramientas actuales facilitan conocer el riesgo al planificar los tratamientos del tumor y realizar vigilancia, educación, diagnóstico precoz, preservación e instauración de tratamiento sustitutivo. Todo ello, supondría importantes mejoras en salud.(AU)

Introduction: Primary ovarian insufficiency (POI) carries significant morbidity, causing infertility, sexual disfunction, decreased bone density, cardiovascular risk, emotional distress and early mortality. Objective: To know the incidence and current management of POI in childhood/adolescent solid tumour survivors. Material and methods: We conducted a multicentre observational study. It included female patients aged 12 to 18 years with a diagnosis of solid tumour and meeting clinical or biochemical criteria for POI. The risk was estimated based on the criteria of the Pediatric Initiative Network of the Oncofertility Consortium. Results: We found an incidence of 1.5 (30 cases of POI): The median age at the time of the event was 14 years (standard deviation, 2.09). The solid tumours associated most frequently with POI were Ewing sarcoma and brain and germ cell tumours. Eighty-three percent of patients did not undergo fertility preservation. Sixty-three percent reported not having undergone menarche at the time of ovarian failure. Ninety-seven percent were at high risk of gonadal toxicity, yet 47% were not monitored before the diagnosis. The median time elapsed to the occurrence of the event was 43.5 months after diagnosis and 29.5 months after completing treatment. The Kaplan-Meier curves showed that approximately 30% of POI cases developed within 2 years of diagnosis and that women at Tanner stage 1 developed insufficiency later than women at Tanner stage 5. Conclusions: There is room for improvement in the followup of women at risk of POI in Spain. The tools currently available facilitate risk assessment at the time of treatment planning and allow the implementation of monitoring, education, early diagnosis, fertility preservation, and replacement therapy as needed. All of this would achieve significant improvement in health outcomes.(AU)

Primary ovarian insufficiency in cancer survivors: Keys to optimal management.

INTRODUCTION: Primary ovarian insufficiency (POI) carries significant morbidity, causing infertility, sexual disfunction, decreased bone density, cardiovascular risk, emotional distress and early mortality. OBJECTIVE: To know the incidence and current management of POI in childhood/adolescent solid tumour survivors. MATERIAL AND METHODS: We conducted a multicentre observational study. It included female patients aged 12-18 years with a diagnosis of solid tumour and meeting clinical or biochemical criteria for POI. The risk was estimated based on the criteria of the Pediatric Initiative Network of the Oncofertility Consortium. RESULTS: We found an incidence of 1.5 (30 cases of POI): The median age at the time of the event was 14 years (standard deviation, 2.09). The solid tumours associated most frequently with POI were Ewing sarcoma and brain and germ cell tumours. Eighty-three percent of patients did not undergo fertility preservation. Sixty-three percent reported not having undergone menarche at the time of ovarian failure. Ninety-seven percent were at high risk of gonadal toxicity, yet 47% were not monitored before the diagnosis. The median time elapsed to the occurrence of the event was 43.5 months after diagnosis and 29.5 months after completing treatment. The Kaplan-Meier curves showed that approximately 30% of POI cases developed within 2 years of diagnosis and that women at Tanner stage 1 developed insufficiency later than women at Tanner stage 5. CONCLUSIONS: There is room for improvement in the follow-up of women at risk of POI in Spain. The tools currently available facilitate risk assessment at the time of treatment planning and allow the implementation of monitoring, education, early diagnosis, fertility preservation, and replacement therapy as needed. All of this would achieve significant improvement in health outcomes.

Atención al adolescente con cáncer. ¿Qué ha cambiado en nuestro país en una década? / Adolescent cancer care: What has changed in Spain in the past decade?

Introducción y objetivo: En el año 2012 el Grupo de trabajo de adolescentes con cáncer publicó los resultados de una encuesta sobre la asistencia a adolescentes en España como punto de partida para futuras actuaciones. Nuestro objetivo fue evaluar si las líneas de trabajo han supuesto cambios asistenciales en la última década. Material y métodos: Encuesta que consta de las mismas preguntas analizadas y publicadas en el año 2012. La encuesta se divide en: epidemiología, atención psicosocial, infraestructuras, tratamiento y seguimiento de los adolescentes con cáncer. Se envió a todos los hospitales con unidades de hematología y oncología pediátrica. Se realizó un análisis estadístico descriptivo de los resultados. Resultados: El porcentaje de pacientes hasta los 18 años tratados en unidades pediátricas ha aumentado del 35,9% al 77,5%. Las hemopatías malignas tratadas en unidades pediátricas se incrementan del 31% al 52% y los tumores sólidos del 49% al 85%. En 2012 30 (39) unidades referían que los casos nuevos de adolescentes representaban un 10%. Actualmente 14 (40) mantienen este porcentaje. Hace una década no existían unidades específicas para adolescentes con cáncer en España. Actualmente, 7 (40) centros disponen de unidades. La atención psicológica para adolescentes apenas ha variado. El seguimiento de supervivientes se realiza por especialistas pediátricos en el 82,5% de los centros. Conclusiones: El trabajo para centralizar los cuidados de adolescentes con cáncer en unidades específicas multidisciplinarias, o en su defecto pediátricas, se ve reflejado en los cambios en la atención sanitaria en nuestro país en la última década. Aún queda un largo recorrido en pilares fundamentales en el abordaje de esta población. (AU)

Introduction and objective: In 2012, the Adolescents with Cancer working group published the results of a survey on care delivery for the adolescent population in Spain as a starting point for future intervention. The aim of this nationwide survey was to outline the current situation and assess whether the implemented strategies have resulted in changes in care delivery. Material and methods: Survey consisting of the same items analysed and published in 2012. The questionnaire was structured into sections devoted to epidemiology, psychosocial care, infrastructure, treatment and follow-up of adolescents with cancer. It was submitted to all hospitals in Spain with a paediatric haematology and oncology unit. We conducted a descriptive analysis of the results. Results: The percentage of patients aged up to 18 years managed in paediatric units has increased from 35.9% to 77.5% in the past decade. The proportion of malignant blood tumours treated in paediatric units increased from 31% to 52%, and the proportion of solid tumours from 49% to 85%. In 2012, 30 units (out of 39) reported that new cases in adolescents amounted to up to 10% of the total. At present, only 14 (out of 40) continue to report this percentage. A decade ago, there were no specific adolescent cancer units in Spain. Now, 7 centres (out of 40) have specific multidisciplinary units. There has been little change in psychological support services for adolescents. The follow-up of survivors is carried out by paediatric specialists in 82.5% of the hospitals. Conclusions: The efforts made to centralise the care of adolescents with cancer in specific multidisciplinary adolescent units or, failing that, paediatric units, is reflected in the changes in care delivery in Spain in the past decade. Much remains to be done in key components of the management of adolescents with cancer. (AU)

Adolescent cancer care: What has changed in Spain in the past decade?

INTRODUCTION AND OBJECTIVES: In 2012, the Adolescents with Cancer Working Group published the results of a survey on care delivery for the adolescent population in Spain as a starting point for future intervention. The aim of this nationwide survey was to outline the current situation and assess whether the implemented strategies have resulted in changes in care delivery. MATERIAL AND METHODS: Survey consisting of the same items analysed and published in 2012. The questionnaire was structured into sections devoted to epidemiology, psychosocial care, infrastructure, treatment and follow-up of adolescents with cancer. It was submitted to all hospitals in Spain with a paediatric haematology and oncology unit. We conducted a descriptive analysis of the results. RESULTS: The percentage of patients aged up to 18 years managed in paediatric units has increased from 35.9% to 77.5% in the past decade. The proportion of malignant blood tumours treated in paediatric units increased from 31% to 52%, and the proportion of solid tumours from 49% to 85%. In 2012, 30 units (out of 39) reported that new cases in adolescents amounted to up to 10% of the total. At present, only 14 (out of 40) continue to report this percentage. A decade ago, there were no specific adolescent cancer units in Spain. Now, 7 centres (out of 40) have specific multidisciplinary units. There has been little change in psychological support services for adolescents. The follow-up of survivors is carried out by paediatric specialists in 82.5% of the hospitals. CONCLUSIONS: The efforts made to centralise the care of adolescents with cancer in specific multidisciplinary adolescent units or, failing that, paediatric units, is reflected in the changes in care delivery in Spain in the past decade. Much remains to be done in key components of the management of adolescents with cancer.

Subcutaneous protein C concentrate in the management of severe protein C deficiency--experience from 12 centres.

Since the first description of subcutaneous protein C concentrate as treatment for severe protein C deficiency in 1996, further cases have been reported but there is no uniform approach to this form of treatment. In order to assess the safety and effectiveness of subcutaneous protein C concentrate and suggest recommendations for future use, patients who had received subcutaneous protein C concentrate were identified from the literature, by contacting the manufacturers and by personal communication. Treatment details were available from 14 cases. Apart from one case where the infusion interval was inadvertently increased, no thrombotic events occurred even when doses were subsequently reduced. Initially, a trough protein C level of >0·25 iu/ml should be aimed for. Subsequently, a smaller dose of subcutaneous protein C concentrate, especially if taken with an oral anticoagulant, may be protective maintenance treatment. The treatment was well tolerated with few side effects. Subcutaneous protein C concentrate on its own or combined with an oral anticoagulant appears to be safe and effective as maintenance treatment of severe protein C deficiency. A major advantage is the avoidance of central venous access devices. The incidence of neurodevelopmental handicap was high with blindness affecting the majority of patients.