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This study assessed the psychometric properties of the Impact of Event Scale (IES) in a Spanish sample at increased risk of hereditary cancer and the contribution of socio-demographic and clinical characteristics to predict cognitive intrusions and avoidance. A total of 766 patients participated in this cross-sectional study. Psychometric analyses of the IES were performed using exploratory and confirmatory factor analyses. The influences of socio-demographic and clinical characteristics were determined using multiple linear regression analyses. The exploratory analysis supported the original two-factor solution of the IES, and the confirmatory analysis added the cross-factor loading for item 12, characteristic for this population. Related to the socio-demographic and clinical variables, patient affected by cancer (β = -.19), sex (β = .15), previous psychiatric treatment (β = -.10), and age (β = -.08), were significant predictors of the intrusion subscale. Patient affected by cancer (β = -.19), sex (β = .13), and previous psychiatric treatment (β = -.14) were significant predictors of the avoidance subscale. Keywords: Cognitive symptoms; psychometrics; genetic counseling; hereditary cancer; emotional distress. Escala del Impacto del Estresor (EIE): Propiedades psicométricas en una muestra española con riesgode cáncer hereditario Resumen: Este estudio evaluó las propiedades psicométricas de la Escala de Impacto del Estresor (EIE) en una muestra española con alto riesgo de cáncer hereditario, y la contribución de las características sociodemográficas y clínicas para predecir cogniciones de intrusión y evitación. Un total de 766 pacientes participaron en un diseño transversal. Los análisis psicométricos se realizaron a través de análisis factoriales exploratorios y confirmatorios. La influencia de las características sociodemográficas y clínicas se determinó llevando a cabo regresiones múltiples. El análisis exploratorio confirmó la solución original bifactorial del EIE, y el análisis confirmatorio añadió la carga factorial cruzada del ítem 12, que caracteriza a esta población. En las características sociodemográficas y clínicas, paciente afecto de cáncer (β = -.19), sexo (β = .15), antecedentes de tratamiento psiquiátrico (β = -.10), y edad (β = -.08), fueron predictores significativos de la subescala de intrusión. Paciente afecto de cáncer (β = -.19), sexo (β = .13), y antecedentes de tratamiento psiquiátrico (β = -.14) fueron predictores significativos de la subescala de evitación. Palabras clave: síntomas cognitivos; psicometría; consejo genético; cáncer hereditario; malestar emocional.

Neuroendocrine neoplasms (NENs) are a heterogeneous family of tumors of challenging diagnosis and clinical management. Their incidence and prevalence continue to rise mainly due to an improvement on diagnostic techniques and awareness. Earlier detection, along with steadfast improvements in therapy, has led to better prognosis over time for advanced gastrointestinal and pancreatic neuroendocrine tumors. The aim of this guideline is to update evidence-based recommendations for the diagnosis and treatment of gastroenteropancreatic and lung NENs. Diagnostic procedures, histological classification, and therapeutic options, including surgery, liver-directed therapy, peptide receptor radionuclide therapy, and systemic hormonal, cytotoxic or targeted therapy, are reviewed and discussed, and treatment algorithms to guide therapeutic decisions are provided.

Although future treatments may speciically target the tumour phenotype, other factors should be included to confirm the efficacy of treatment and prevention strategies. The objective of this study was to compare sociodemographic characteristics and psychological distress for breast, ovarian and colorectal cancer predisposition syndrome in a sample at high risk of hereditary cancer.A cross-sectional study was designed with 799 patients. The nonparametric test, with Kruskal-Wallis test, was used to compare three genetic cancer syndromes, with significant differences in sample size.There were no differences in cancer hereditary syndromes related to sociodemographic characteristics except sex, as breast/ovarian cancer mainly affects women. No group differences were observed for cancer worry (P = 0.17). Breast/ovarian cancer syndrome showed significantly higher scores in cognitive distress compared to colorectal cancer (P = 0.01).The differences in the distribution of sociodemographic characteristics in these hereditary cancer syndromes can help to better plan resources for patient care in genetic counselling units.

Introduction: To date, there are no guidelines for a rational and more favourable sequence of treatment after docetaxel. Two drugs (cabazitaxel and abiraterone) have recently been approved as second-line treatment after docetaxel failure in metastatic castration-resistant prostate cancer (mCRPC), but there are no studies comparing abiraterone versus cabazitaxel. The most suitable drug is chosen based on the physician's opinion and the patient's characteristics. In patients with a good performance status who are able to receive either treatment, it would be convenient to begin with cabazitaxel and to reserve abiraterone in case there is a worsening of the general status, in consideration of abiraterone's more favourable toxicity profile. Case Report: We describe the case of a 74-year-old male with mCRPC who presented with an interesting and uncommon tumour dissemination (pleuropulmonary) occurring after the first standard treatment with docetaxel. Intravenous treatment with cabazitaxel 25 mg/m2 and oral prednisone 10 mg continuously was initiated. The patient received a total of 8 cycles of chemotherapy. A reduction of mediastinal adenopathies and infrarenal para-aortic stable bone involvement and an absence of pleural effusion were observed. No relevant toxicity was noted. Since February 2012, a progressive PSA increase without clinical deterioration has been noted. Conclusions: The selection criteria for second- and third-line systemic treatment and the excellent response obtained with cabazitaxel in an unusual disease setting are described. The results confirm the long duration and quality of response of cabazitaxel treatment. Further therapeutic options in this group of patients are suggested.

En los últimos 50 años la medicina ha evolucionado más que en los dos siglos anteriores. Sin embargo, existen en la actualidad multitud de patologías que la medicina contemporánea no ha sido capaz de solventar. Hasta hace tan solo unas décadas, los niños diagnosticados de cáncer fallecían en poco tiempo. Así pues, esto ha ido modificándose y más de la mitad llegan a ser supervivientes a largo plazo. Los cuidados paliativos como especialidad se han desarrollado de forma marcada en los últimos años. Sin embargo, destaca una parcela de ella que todavía se encuentra muy lejos de estar establecida, los cuidados paliativos pediátricos (CPP). Los niños presentan necesidades diferentes que es necesario atender. Se pretende revisar el concepto de CPP, haciendo hincapié en las diferencias que presentan los niños con respecto a los adultos y en su problemática específica. In the last 50 years medicine has evolved more than in the previous two centuries but there are currently many pathologies that modern medicine has not been able to cure. Until a mere few decades ago, children diagnosed with cancer died within a short time. However, this has gradually changed and, nowadays, over half of them become long-term survivors. Palliative care as a medical specialization has markedly developed in recent years. Nevertheless, there is a specific part that is still far from being established, pediatric palliative care (PPC). Children have different needs to be attended. PPC should be reviewed with a special focus on the precise differences present in children and their specific problems.

Los carcinomas microciticos extrapulmonares (CMEP) se han reconocido como entidad diferenciada del carcinoma microcitico broncopulmonar (CMBP). Se consideran una enfermedad sistemica desde el inicio y esto hace que su abordaje sea similar al de los CMBP debiendo considerarse siempre el empleo de quimioterapia tanto en enfermedad localizada como diseminada. Presentamos un estudio retrospectivo sobre los CMEP y los comparamos con los CMBP atendidos en nuestro centro. Usando la base de datos del servicio de Oncologia Medica del Hospital Universitario La Fe se localizan 24 CMEP atendidos entre los anos 1987 y 2004. Se realiza una comparacion indirecta entre estos y una serie publicada de nuestro servicio de 341 pacientes afectos de CMBP, durante los anos 1981 a 1992 De los 24 pacientes diagnosticados de CMEP 19 son hombres y 5 mujeres, con una media de edad de 58 anos (extremos 23 a 85). Predomina la localizacion mediastinica. Un 58% presenta enfermedad diseminada. Todos los pacientes menos uno recibieron algun tipo de tratamiento. El abordaje terapeutico fue local y sistemico en 11 pacientes, solo sistemico en 6, y solo local en 4. Predomina el uso de quimioterapia basada en platino y etoposido. La mediana de seguimiento potencial de la serie es de 53 meses (extremos 4 y 211), la mediana de supervivencia es de 18,9 meses; 30,8 meses en el estadio localizado y 8 en el estadio diseminado. En cuanto a la serie de CMBP, existe un claro predominio de hombres (336) sobre mujeres (5). Un 62% presenta enfermedad diseminada al diagnostico. La supervivencia global de la serie es de 10 meses, 12 en enfermedad limitada y 8 en enfermedad extendida. La supervivencia global de la serie de CMEP es superior a la de CMBP. Los CMEP obtienen mejor supervivencia en enfermedad localizada. La quimioterapia es la piedra angular del tratamiento. En determinadas situaciones puede ser suficiente un tratamiento exclusivamente local.

Introduction. Despite significant advances in the palliative care of cancer patients with terminal disease, a considerable proportion of them still die at hospital. The causes of this fact are multiple including sociocultural, familial, and sanitary reasons.

El tratamiento del dolor es una de las prioridades de la Organizacion Mundial de la Salud (OMS) en la lucha contral el cancer. La OMS aboga por su escala analgesica contresniveles. Los opioides mayores son el tratamiento farmacologico fundamental del dolor oncologico cronico. La morfina oral de liberacion inmediata tiene una duracion de accion de 4 horas. La morfina oral de liberacion retardada tiene 8–12 horas de duracion. El fentanilo transdermico es un sistema disenado para liberar el farmaco durante 72 horas. Hay sistemas disenados para liberar 25, 50, 75 y 100 μg/hora, segun la superficie del parche. La buprenorfina transdermica es el ultimo medicamento comercializado para el tratamiento del dolor cronico. Existen tres presentaciones, cada una de ellas libera de forma respectiva 35, 52,5 y 70 μg/hora. El parche debe cambiarse cada 72 horas. La terapeutica fundamental en el manejo del dolor incidental es el uso de dosis suplementarias de opioides de liberacion inmediata. La dosis oscila entre el 10%–25% de la dosis opioide total diaria.

Material y metodos Estudiamos pacientes con infeccion por el virus de la inmunodeficiencia humana (VIH) que desarrollaron un linfoma no Hodgkin desde enero de 1985 a octubre de 2001. Resultados Cuarenta y cuatro pacientes (36 varones y 8 mujeres; mediana de edad de 34 anos). Linfoma de Burkitt el 34% de los casos, linfoma difuso de celula grande B el 29,5%. Diagnostico de sida previo en 20 casos (45%). Indice pronostico internacional (IPI) 0-1 en 19 pacientes (43%), IPI 2 en 12 (27%) y superior a 3 en 13 (30%). Recibieron quimioterapia el 64% de los pacientes, radioterapia el 2% y ambas el 11%. Alcanzaron criterios de remision completa 13 pacientes (29%), hubo respuesta parcial en dos (4%) y estabilizacion en uno (2%). Nueve (20%) pacientes siguen vivos (5 sin enfermedad), 22 (50%) fallecieron por el linfoma no Hodgkin, 5 (11%) fallecieron por toxicidad del tratamiento y 8 por otras causas. La mediana de supervivencia fue de tres meses, proyectada al ano del 24% y a los dos anos del 14%. En el analisis univariante de factores pronostico resultaron significativos el IPI 0-1 frente al 2-5 (p = 0,000), el performance status igual o inferior a 2 (p = 0,021) y la ausencia de sintomas B (p = 0,012). En el analisis multivariante mantiene la significacion el IPI 0-1 (p = 0,000). Conclusiones La poblacion VIH con linfoma no Hodgkin tiene multiples factores de mal pronostico. La supervivencia es escasa y la toxicidad de la quimioterapia elevada. Los pacientes con IPI bajo definen un subgrupo de mejor pronostico.

Background Oxaliplatin has been introduced to the clinical setting in combination with 5-fluorouracil/folinic acid (5-FU/FA) in an attempt to improve the response rate against colorectal cancer. It is unclear the optimum schedule of combination. In order to evaluate the response rate and toxicity profile of the combination of oxaliplatin with 5-FU/FA, according to the Mayo Clinic schedule, we evaluated our patients in a retrospective analysis.

Los pacientes que presentan inmunodeficiencias de cualquier tipo, congenitas, adquiridas o iatrogenicas, tienen un mayor riesgo de padecer neoplasias, y los linfomas no hodgkinianos (LNH) estan entre las mas frecuentes. Los LNH asociados a inmunodeficiencias congenitas son muy poco frecuentes y se diagnostican en la edad pediatrica. En los adultos, los dos grupos mas importantes son los LNH asociados a la infeccion por el virus de la inmunodeficiencia humana (VIH) y los sindromes linfoproliferativos postrasplante 1 . En los ultimos 30 anos, el desarrollo de nuevos tratamientos inmunodepresores ha permitido la realizacion del trasplante alogenico de organos, lo que ha supuesto un gran avance terapeutico para algunas enfermedades. El numero de trasplantes realizados ha experimentado un crecimiento constante, por lo que se ha observado un incremento de los tumores observados en esta poblacion. Desde el principio se reconocio que los pacientes sometidos a inmunodepresion iatrogenica tenian un riesgo elevado de sufrir neoplasias; las mas frecuentes son los sindromes linfoproliferativos (21%) seguidos de los tumores epiteliales cutaneos (8%) y el sarcoma de Kaposi (6%) 2

Background Nowadays, 65-80% of pheochromocytoma and paraganglioma (PPGL) cases are explained by germline or somatic mutations in one of 22 genes. Several genetic testing algorithms have been proposed, but they usually exclude sporadic-PPGLs (S-PPGLs) and none include somatic testing. We aimed to genetically characterise S-PPGL cases and propose an evidence-based algorithm for genetic testing, prioritising DNA source. Methods The study included 329 probands fitting three criteria: single PPGL, no syndromic and no PPGL family history. Germline DNA was tested for point mutations in RET and for both point mutation and gross deletions in VHL, the SDH genes, TMEM127, MAX and FH. 99 tumours from patients negative for germline screening were available and tested for RET, VHL, HRAS, EPAS1, MAX and SDHB. Results Germline mutations were found in 46 (14.0%) patients, being more prevalent in paragangliomas (PGLs) (28.7%) than in pheochromocytomas (PCCs) (4.5%) (p= 6.62x10(-10)). Somatic mutations were found in 43% of those tested, being more prevalent in PCCs (48.5%) than in PGLs (32.3%) (p= 0.13). A quarter of S-PPGLs had a somatic mutation, regardless of age at presentation. Head and neck PGLs (HN-PGLs) and thoracic-PGLs (T-PGLs) more commonly had germline mutations (p= 2.0x10(-4) and p= 0.027, respectively). Five of the 29 metastatic cases harboured a somatic mutation, one in HRAS. Conclusions We recommend prioritising testing for germline mutations in patients with HN-PGLs and TPGLs, and for somatic mutations in those with PCC. Biochemical secretion and SDHB-immunohistochemistry should guide genetic screening in abdominal-PGLs. Paediatric and metastatic cases should not be excluded from somatic screening.

The current situation of palliative care. Review of a non-standardized situation En los ultimos 50 anos la medicina ha evolucionado mas que en los dos siglos anteriores. Sin embargo, existen en la actualidad multitud de patologias que la medicina contemporanea no ha sido capaz de solventar. Hasta hace tan solo unas decadas, los ninos diagnosticados de cancer fallecian en poco tiempo. Asi pues, esto ha ido modificandose y mas de la mitad llegan a ser supervivientes a largo plazo. Los cuidados paliativos como especialidad se han desarrollado de forma marcada en los ultimos anos. Sin embargo, destaca una parcela de ella que todavia se encuentra muy lejos de estar establecida, los cuidados paliativos pediatricos (CPP). Los ninos presentan necesidades diferentes que es necesario atender. Se pretende revisar el concepto de CPP, haciendo hincapie en las diferencias que presentan los ninos con respecto a los adultos y en su problematica especifica. In the last 50 years medicine has evolved more than in the previous two centuries but there are currently many pathologies that modern medicine has not been able to cure. Until a mere few decades ago, children diagnosed with cancer died within a short time. However, this has gradually changed and, nowadays, over half of them become long-term survivors. Palliative care as a medical specialization has markedly developed in recent years. Nevertheless, there is a specific part that is still far from being established, pediatric palliative care (PPC). Children have different needs to be attended. PPC should be reviewed with a special focus on the precise differences present in children and their specific problems.

A retrospective study analyzing non-Hodgkin's lym-phoma (NHL) diagnosed in patients in our center above 65 years of age between the years 1977–1991 is reported. Histological classification has been completed following the criteria of the Working Formulation. Of 521 patients, 427 were candidates for evaluation. Those above 65 years of age comprised the subject of our study, with a total of 95 cases.

BACKGROUND:Metastatic renal cell carcinoma (MRCC) has been characteristically unresponsive to chemotherapy. In lieu of an effective regimen, interleukin-2 (IL-2) and interferon alfa are considered drugs of choice to treat this cancer. Subcutaneous IL-2 is safe and well tolerated, with a mortality rate OBJECTIVE:To report a case of cutaneous and hematologic toxicity in a patient treated with IL-2.CASE SUMMARY:A 67-year-old woman received radiotherapy and immunotherapy for cancer that had metastasized to the bone and lungs. IL-2 was part of the regimen. After 5 days of treatment with IL-2, the patient developed a hemorrhagic lesion that progressed to toxic epidermal necrolysis, as well as grade 4 pancytopenia. She died 10 days after treatment was begun. At the time of death, leukocytes were 0.3 × 103/mm3, platelets 10 × 103/mm3, and hemoglobin 6.8 mg/dL.DISCUSSION:Cutaneous IL-2 adverse effects are frequent, but generally mild and reversible. The adverse hematologic effects are usually transitory and pancytopenia is not frequent. The severity of cutaneous and hematologic toxicity experienced by our patient has rarely been reported.CONCLUSIONS:The use of IL-2 in bedridden patients with performance status >2 must be given on an individualized basis. If radiotherapy over extensive areas of the body is needed, the use of IL-2 must be postponed until radiotherapy is completed.

Sorafenib is the standard treatment of patients with advanced hepatocellular carcinoma (HCC), with demonstrated outcome benefits in randomized clinical trials. We present a single-center experience with sorafenib with the aim to establish its efficacy and safety in daily clinical practice. A total of 62 patients were treated with sorafenib 400 mg/12 h until disease progression or unacceptable toxicity. Response rates, incidence of adverse events, actuarial disease-free survival, and overall survival (OS) were estimated. Univariate and multivariate analyses of prognostic factors for survival were also performed. Median treatment duration was 92 days. A 43% disease control rate was achieved (partial response, 15% and disease stabilization, 28%). After a median follow-up of 24.1 months, the median progression-free survival and OS for the overall population were 5.8 and 6.7 months, respectively, with survival rates of 27% at 1 year and 17 % at 2 years. The most common grade 3-4 adverse events were fatigue (19%), hand-foot syndrome (8%), hypertension (5%), and diarrhea (3%). The univariate analysis showed that patient performance status (PS), use of previous treatments, and albumin >3.5 g/dL were significant prognostic factors for survival. In the multivariate study, only PS, alcoholic etiology and albumin >3.5 g/dL remained as independent predictors of survival. Sorafenib is a safe and moderately effective drug in HCC, although patients must be properly selected before starting therapy. Baseline PS, Barcelona Clinic Liver Cancer staging, and liver function should be taken into account as prognostic factors. Results in daily practice are somewhat inferior than observed in clinical trials.

The rhabdomyosarcoma (RMS) is a tumour derived from the mesenchymal cells that give origin to striate muscle. It appears most frequently at the pediatric age, although some appear in adolescence and in young adulthood. It is a high grade malignancy that metastatisis frequently (most importantly to the lung). There are two principal types: alveolar RMS and embrionary RMS. It can appear in any localization, being the most frequent the periorbitary area and the paranasal sinuses, and the second in frequency the genitourinary system (bladder, prostate and vagina). The therapeutic management is multidisciplinary and local therapies must always be accompanied with polychemotherapy. With combined treatments a curation rate of almost 90% can be reached in good prognosis cases, although it drops to 20% in those with disfavourable prognostic factors. The protocols with higher chemotherapy doses and subsequent rescue with blood-borne progenitor cells have not yet proved their efficiency. We present the case of a fifteen-year old patient with an embrionary rhabdomyosarcoma of the prostate, we analyze the clinical evolution and we revise the present therapeutic management of these neoplasies.

Oligodendrogliomas are primary brain tumours derived from oligodendroglial cells, or precursors, and represent 2%–5% of brain tumours. This type of glioma has a favourable prognosis compared to other brain tumours. The treatment is multidisciplinary and is based on three therapeutic arms: surgery, radiotherapy and chemotherapy. We present a patient who had received treatment previously for a lowgrade glioma and who subsequently developed an anaplastic oligoastrocytoma in the same zone together with skull and extra-cranial involvement in the disease progression.

Pseudomyxoma peritonei is an unusual condition that has caused much confusion regarding its aetiology, clinical manifestations, treatment and prognosis. It is characterised by mucinous ascites and diffuse mucinous invasions of the peritoneum. Three histological subtypes have been defined: a) disseminated peritoneal adenomucinosis (peritoneal lesions composed of abundant extra-cellular mucin containing scant simple-to-focally-proliferating mucinous epithelium with little cytological atypia or mitotic activity); b) peritoneal mucinous carcinomatosis (peritoneal lesions composed of more abundant mucinous epithelium with the architectural and cytological features of carcinoma); and c) an intermediate group. The different histological subtypes have different prognoses.

La aparicion de linfomas no Hogdkin es una complicacion frecuente en los pacientes con infeccion por virus de la inmunodeficiencia humana (VIH). Sin embargo la afectacion primaria pancreatica por linfoma es un evento raro y solo constituye el 0,16% del total de neoplasias pancreaticas. Presentamos el caso de una paciente de 24 anos infectada por VIH desde 1996 tras trasmision heterosexual, que en el momento del diagnostico no cumplia criterios de sida. En septiembre de 2000 la paciente ingreso para estudio de una lumbalgia de un mes de evolucion. Durante su ingreso la paciente desarrollo ictericia conjuntival, dolor abdominal, nauseas, vomitos, acolia y coluria. La bioquimica mostro un patron de colostasis y en la ecografia se aprecio dilatacion de la via biliar intra y extrahepatica con imagenes nodulares y distorsion en la celda pancreatica. Se realizo laparotomia terapeutica y diagnostica. Las biopsias pancreaticas mostraron infiltracion por celulas linfoides sugestivas de linfoma de celulas B, las celulas eran CD-20 +. No fue posible realizar mas tecnicas inmunohistoquimicas. Recibio tratamiento con quimioterapia seguida de radioterapia locorregional. Veintiseis meses despues del diagnostico la paciente permanece libre de enfermedad.