Multidisciplinary consensus on the criteria for fertility preservation in cancer patients

Infertility is one of the main sequelae of cancer and its treatment in both children and adults of reproductive age. It is, therefore, essential that oncologists and haematologists provide adequate information about the risk of infertility and the possibilities for its preservation before starting treatment. Although many international clinical guidelines address this issue, this document is the first Spanish multidisciplinary guideline in paediatric and adult oncological patients. Experts from the Spanish Society of Medical Oncology, the Spanish Fertility Society, the Spanish Society of Haematology and Haemotherapy, the Spanish Society of Paediatric Haematology and Oncology and the Spanish Society of Radiation Oncology have collaborated to develop a multidisciplinary consensus.

Multidisciplinary consensus on the criteria for fertility preservation in cancer patients.

Infertility is one of the main sequelae of cancer and its treatment in both children and adults of reproductive age. It is, therefore, essential that oncologists and haematologists provide adequate information about the risk of infertility and the possibilities for its preservation before starting treatment. Although many international clinical guidelines address this issue, this document is the first Spanish multidisciplinary guideline in paediatric and adult oncological patients. Experts from the Spanish Society of Medical Oncology, the Spanish Fertility Society, the Spanish Society of Haematology and Haemotherapy, the Spanish Society of Paediatric Haematology and Oncology and the Spanish Society of Radiation Oncology have collaborated to develop a multidisciplinary consensus.

Paediatric Horner syndrome. A case series of 14 patients in a tertiary hospital.

BACKGROUND: Horner syndrome (HS) is characterised by the triad of upper eyelid ptosis, miosis, and facial anhidrosis. Due to its wide variety of causes, it can occur at any age, and is uncommon in paediatrics. The aetiology and diagnostic approach of paediatric HS (PHS) is controversial. OBJECTIVE: The purpose of this study is to describe the clinical characteristics of a 14 case series, focusing on the aetiology of HS and the clinical evolution the patients presented. METHODS: A retrospective observational study was conducted on patients under 14 years-old (enrolled between 1st January 2009 and 30th April 2020). Depending on the age at diagnosis (before or after the first 5 months of life), the study cases were divided into two groups: congenital or acquired. RESULTS: Fourteen patients, with a mean age of 8.5 months, were enrolled. The most frequent cause of PHS were tumours (6/14), with the most representative neoplasm being neuroblastoma (4/14). Of the acquired cases (8/14), the most frequent cause was iatrogenic (5/8), mainly secondary to cervical or thoracic surgery. The main origin of congenital HS (6/14) was neuroblastoma (4/6), being the first manifestation of the disease in 50% of patients (2/4). CONCLUSION: HS may be the first sign of a major underlying disease, such as neuroblastoma. For this reason, children presenting with HS of unknown origin require imaging studies to exclude a life threatening disease. A thorough examination is essential for early diagnosis of these patients.

Síndrome de Horner pediátrico. A propósito de una serie de 14 casos en un hospital terciario / Paediatric Horner syndrome. A case series of 14 patients in a tertiary hospital

Antecedentes El síndrome de Horner (SH) se caracteriza por la triada de ptosis palpebral, miosis y anhidrosis facial. Debido a su amplia variedad de causas puede ocurrir en cualquier edad, siendo infrecuente en pediatría. La etiología y estudio diagnóstico del SH pediátrico (SHP) es motivo de controversia. ObjetivoDescribir las características clínicas de una serie de 14 niños diagnosticados de SH, incidiendo en la etiología del SH y en la evolución clínica que presentaron. Métodos Estudio observacional retrospectivo de pacientes menores de 14 años diagnosticados de SHP en nuestro centro entre el 01 de enero del 2009 y el 30 de abril del 2020. En función de la edad al diagnóstico, los casos se dividieron en congénitos (antes de los cinco meses) y adquiridos. Resultados Se reclutaron 14 pacientes, con una mediana de edad al diagnóstico de 8,5 meses. La causa más frecuente de SHP fue tumoral (6/14), siendo la neoplasia más representativa el neuroblastoma (4/14). De los casos adquiridos (8/14), la causa más frecuente fue iatrogénica (5/8), secundario a cirugía cérvico-torácica. La etiología principal del SH congénito (6/14) fue el neuroblastoma (4/6), siendo la primera manifestación clínica de la enfermedad en el 50% de los pacientes (2/4). Conclusiones El SH puede ser el primer signo de una enfermedad subyacente grave, como es el neuroblastoma. Por este motivo, es necesario realizar un adecuado estudio de extensión en todos los pacientes pediátricos diagnosticados de SH sin una causa clara atribuible. Es fundamental un examen riguroso para un diagnóstico precoz de estos pacientes (AU)

Background Horner syndrome (HS) is characterised by the triad of upper eyelid ptosis, miosis, and facial anhidrosis. Due to its wide variety of causes, it can occur at any age, and is uncommon in paediatrics. The aetiology and diagnostic approach of paediatric HS (PHS) is controversial. ObjectiveThe purpose of this study is to describe the clinical characteristics of a 14 case series, focusing on the aetiology of HS and the clinical evolution the patients presented. Methods A retrospective observational study was conducted on patients under 14 years-old (enrolled between 1 st January 2009 and 30th April 2020). Depending on the age at diagnosis (before or after the first 5 months of life), the study cases were divided into two groups: congenital or acquired. Results Fourteen patients, with a mean age of 8.5 months, were enrolled. The most frequent cause of PHS were tumours (6/14), with the most representative neoplasm being neuroblastoma (4/14). Of the acquired cases (8/14), the most frequent cause was iatrogenic (5/8), mainly secondary to cervical or thoracic surgery. The main origin of congenital HS (6/14) was neuroblastoma (4/6), being the first manifestation of the disease in 50% of patients (2/4). Conclusion HS may be the first sign of a major underlying disease, such as neuroblastoma. For this reason, children presenting with HS of unknown origin require imaging studies to exclude a life threatening disease. A thorough examination is essential for early diagnosis of these patients. (AU)

Prognostic factors for overall survival in paediatric patients with Ewing sarcoma of bone treated according to multidisciplinary protocol.

BACKGROUND: The purpose of this study is to assess the outcome of patients with Ewing sarcoma (EWS) of the bone and to identify prognostic factors. MATERIALS AND METHODS: Seventy-seven patients younger than 18 years old, diagnosed with EWS of the bone between 1979 and 2009, were analysed retrospectively. Four different protocols of chemotherapy were used successively. Local treatment consisted of surgery (N=32), radiotherapy (N=20) and a combination of both (N=19). RESULTS: The median age at diagnosis was 10 years old (range, 2-17) and the median follow-up for survivors 8.6 years (range, 1-18.8). Thirty-two relapses occurred (21 distant, 5 local and 6 both). The 2- and 5-year overall survival rates were 70% and 51%, respectively. Multivariate analysis showed four significant independent predictors for death: age ≥14 years old (HR: 5.06; p=0.019), lack of complete response (HR: 8.04; p<0.001), tumour volume ≥150 ml (HR: 2.21; p=0.045) and distant recurrences (HR: 1.45; p=0.001). CONCLUSIONS: Outcome of EWS of bone is influenced by many clinical and treatment-correlated variables. Criteria to stratify patients should include all the variables that have shown prognostic significance. The development of novel therapies should target these high-risk groups.

Prognostic factors for overall survival in paediatric patients with Ewing sarcoma of bone treated according to multidisciplinary protocol

BACKGROUND: The purpose of this study is to assess the outcome of patients with Ewing sarcoma (EWS) of the bone and to identify prognostic factors. MATERIALS AND METHODS: Seventy-seven patients younger than 18 years old, diagnosed with EWS of the bone between 1979 and 2009, were analysed retrospectively. Four different protocols of chemotherapy were used successively. Local treatment consisted of surgery (N=32), radiotherapy (N=20) and a combination of both (N=19). RESULTS: The median age at diagnosis was 10 years old (range, 2-17) and the median follow-up for survivors 8.6 years (range, 1-18.8). Thirty-two relapses occurred (21 distant, 5 local and 6 both). The 2- and 5-year overall survival rates were 70% and 51%, respectively. Multivariate analysis showed four significant independent predictors for death: age ≥14 years old (HR: 5.06; p=0.019), lack of complete response (HR: 8.04; p<0.001), tumour volume ≥150 ml (HR: 2.21; p=0.045) and distant recurrences (HR: 1.45; p=0.001). CONCLUSIONS: Outcome of EWS of bone is influenced by many clinical and treatment-correlated variables. Criteria to stratify patients should include all the variables that have shown prognostic significance. The development of novel therapies should target these high-risk groups (AU)

Calidad de vida y funcionalidad en pacientes pediátricos intervenidos de tumores óseos en miembros inferiores: cirugía reconstructiva versus amputación / Quality of life and functionality in pediatric patients operated on for lower limb bone tumors: limb salvage surgery versus amputation

Objetivo. Analizar las diferencias en cuanto a funcionalidad y calidad de vida relacionadas con la salud en pacientes intervenidos de un tumor óseo maligno en edad infantil según el tipo de cirugía practicada. Material y método. Estudio descriptivo transversal de pacientes mayores de 14 años tratados por osteosarcoma o sarcoma de Ewing en miembro inferior y que recibieron tratamiento quirúrgico en edad infantil tras una evolución mínima de 2 años. Se realizó una evaluación de funcionalidad y calidad de vida utilizando las escalas de Enneking y Medical Outcomes Study Short-Form 36. Comparamos los resultados funcionales según la técnica quirúrgica. Resultados. Nuestra muestra quedó formada por 17 pacientes (8 hombres y 9 mujeres) con una edad media actual de 22 años. El tumor más frecuente fue el osteosarcoma, y la localización, el tercio distal del fémur y proximal de la tibia. En relación a la técnica quirúrgica, en 8 pacientes se colocó endoprótesis, 4 se trataron mediante injerto y/u osteosíntesis y 5 fueron amputados en cirugía inicial. Cinco pacientes tuvieron que ser amputados en un segundo momento debido a complicaciones. La puntuación expresada en mediana y percentiles obtenidos en la escala de Enneking sobre la totalidad de la muestra fue del 83,33% (66,66-88,33%). En pacientes no amputados fue del 83,33% (73,33-86,66%), frente al 90% (80-93,33%) en pacientes amputados inicialmente. En los que se realizó amputación posterior tras complicación, la puntuación fue de 56,66% (51,66-73,33%). Conclusiones. En nuestra muestra no encontramos diferencias significativas en cuanto a funcionalidad y calidad de vida de los pacientes tratados mediante cirugía reconstructiva en comparación con amputación inicial, aunque sí peores resultados en los pacientes que sufren una amputación tardía debido a una complicación tras cirugía de salvamento inicial (AU)

Objective. To analyze the differences in functionality and quality of life related with outcome of a malignant bone tumor intervention in childhood age according to type of surgery performed. Material and methods. A descriptive, cross-sectional study of patients over 14 years treated for osteosarcoma or Ewing's sarcoma in lower limb during childhood after a minimum of two years evolution. Functionality and quality of life were evaluated with the Enneking and Medical Outcomes Study Short-Form 36 scales. The functional results were compared according to surgical technique. Results. Our sample consisted of 8 men and 9 women, with a current average age of 22 years. The most common type of tumor was osteosarcoma and most common location was distal third of the femur and proximal tibia. In relation to the surgical technique, stent was placed in 8 patients, 4 were treated by graft and/or osteosynthesis and 5 patients underwent amputation as initial surgery. Five more patients had to under amputation at a second time due to complications. The score, expressed as median and percentiles obtained in the scale of Enneking on the entire sample, was 83.33% (66.66%-88.33%). In non-amputees, it was 83.33% (73.33%-86.66%), compared with 90% (80%-93.33%) in patients who were initially amputees. In those in whom the amputation was performed later after a complication, the median Enneking score was 56.66% (51.66%-73.33%). Conclusions. In our sample, we did not find significant differences in terms of functionality and quality of life of patients treated by reconstructive surgery compared with initial amputation although there were worse results in those patients who underwent a later amputation due to a complication after the initial life-saving surgery (AU)

Health related quality of life and late side effects of long-term survivors of Ewing's sarcoma of bone.

PURPOSE: The data examining the Health Related Quality of Life (HRQOL) after definitive treatment for Ewing's sarcoma (EWS) is sparse. The objective of this study was to assess the HRQOL and late side effects in EWS of bone survivors treated in the past 2 decades. METHODS: Seventeen long-term (≥ 5 years) EWS survivors (age range, 11-27 years) treated from 1990 to 2004 completed the EORTC-QLQ-C30. We compared the HRQOL of our data set with a reference group of cancer survivors and also with the general population. Musculoskeletal late treatment toxicity was also evaluated. The Mann-Whitney and Wilcoxon tests were used for analyses. RESULTS: EWS survivors of our series reported significantly better global, physical, role and cognitive HRQOL and less symptoms of fatigue, pain and insomnia than the normative sample of cancer survivors. Moreover, the HRQOL was equivalent to the general population, with the exception of social functioning. Additionally, patients with tumor location in the extremities did not report significant HRQOL differences compared with those with tumor at other locations. Common chronic grade 2 side effects were generalized muscle weakness (23%) and decreased joint range of motion (23%). Only one patient experienced musculoskeletal chronic grade 3 toxicity. CONCLUSION: Our findings suggest that EWS survivors treated in the modern era do not experience lower HRQOL than other cancer survivors. Rather, the HRQOL appears to be equivalent to the general population. Further and larger studies are needed to confirm these results.

Rabdomiosarcoma de vías biliares como causa de ictericia obstructiva / Rhabdomyosarcoma of the bile ducts as a cause of obstructive jaundice

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