Citogenética de los tumores derivados del neuroectodermo / Cytogenetics of neuroectodermal tumors

Las aplicaciones de los métodos modernos de citogenética permiten identificar los sitios de alteración cromosómica donde se han generado rupturas y rearreglos por translocación. Tales áreas pueden ser además tipificadas mediante el uso de sondas para ADN denominadas FISH. Presentamos dos ejemplos de alteraciones genéticas en un Sarcoma de Ewing Paravertebral y en un ependimoma supratentorial recidivante.

Colon carcinoma in children and adolescents: prognostic factors and outcome-a review of 11 cases.

BACKGROUND: Carcinoma of the colon and rectum is rare in the pediatric age group, and usually presents with an advanced stage disease bearing a poor prognosis. Colorectal carcinoma should be considered in children with signs of intestinal obstruction, alteration in bowel habits, gastrointestinal bleeding and chronic abdominal pain. We performed a retrospective study to evaluate the clinical characteristics, and prognosis of these patients. METHODS: Between 1974 and 2007, 11 patients were identified and treated for colorectal carcinoma at the Oncology Unit. The medical records were studied to analyze the age, sex, clinical presentation, diagnostic procedures, extent of disease (Dukes staging), treatment, histological types, and outcome. RESULTS: There were seven boys and four girls ranging from 7 to 17 of age. Predisposing diseases and syndromes were encountered in three children, (1 with Turner's syndrome and two with adenomatous familial polyposis). Abdominal pain, acute intestinal obstruction, rectal bleeding and weight loss were the commonest symptoms. Surgical procedures were done in 11 patients (incomplete resection with segmental resection in 4 patients, complete resection in the other 4, and biopsy alone in 3 patients).The predominant histological type was mucinous carcinoma. Seven patients received adjuvant chemotherapy, all of whom did not survive. Two patients died shortly after initial surgery, and two patients are alive, and well. CONCLUSIONS: Colorectal carcinoma in children is very uncommon and could be easily misdiagnosed, resulting in advanced stage disease at diagnosis. Because radical surgery which is the mainstay of treatment is possible only in patients with early stage disease, a high level of awareness and early diagnosis are critical.

Diagnosis of anterior mediastinal mass lesions using the Chamberlain procedure in children.

The mediastinum is the most common site for primary intra-thoracic pathology in childhood. This paper describes the versatility of the Chamberlain operation in establishing tissue diagnosis and guiding definitive treatment in children with mediastinal mass lesions. During 1999-2006, 28 consecutive patients were referred to a National Oncology Center with anterior mediastinal pathology. Eleven underwent the Chamberlain procedure. Demographic data, diagnostic sensitivity, operating time and morbidity were recorded. All patients had preoperative chest X-ray and computed tomography scans. The Chamberlain operation was deployed in only those children without other ways of accurately establishing their diagnosis. Others with mediastinal pathology had a diagnosis established by lymph node biopsy, thoracentesis or other method(s). Eleven patients (nine male, two female; age range 2-13 years) underwent the Chamberlain procedure. In these children, there was no pre-existent diagnosis and this was the primary procedure employed. Diagnostic accuracy was 100%. Three patients had pleural disruption and chest tubes were placed at the time of surgery. No patient required a thoracotomy. Average operating time was 1.3 h. Five patients were diagnosed with Hodgkin's lymphoma, four had non-Hodgkin's lymphoma and two children thymic hyperplasia. The Chamberlain operation provides excellent access to the antero-superior mediastinum for biopsy of obscure mediastinal mass lesions in childhood. Complications from this procedure are very rare.

Evaluación de la superficie corporal quemada en pacientes del Hospital Universitario San Vicente de Paúl, Medellín, 2004 / Valoration of burned body surface area in patients of San Vicente de Paúl University Hospital, Medellín 2004

INTRODUCCIÓN: la evaluación de la superficie corporal quemada tiene gran importancia para el tratamiento inicial y el pronóstico del paciente quemado. Se revisan los diferentes métodos para evaluar la superficie corporal quemada y algunos conceptos básicos de reanimación.MATERIALES Y MÉTODOS: se realizó un estudio descriptivo, retrospectivo y transversal a partir de las historias clínicas de los pacientes quemados hospitalizadosen el Hospital Universitario San Vicente de Paúl (HUSVP) de Medellín durante el año 2004. Se compararon los diagnósticos de extensión quemada emitidos porel médico remitente, el médico del servicio de urgencias pediátricas o de adultos del HUSVP en el momento del ingreso del paciente a esta institución, y el cirujanoplástico de la unidad de quemados. Los datos obtenidos fueron procesados con el paquete EpiInfo 6,04 y se consideró como estadísticamente significativo un valor de p<0,05. Las variables se presentan como valores absolutos y sus respectivos porcentajes.RESULTADOS: fueron atendidos 329 pacientes. El 60 por ciento de los diagnósticos de remisión eran incompletos o errados, comparado con 39,2 por ciento de incompletos o incorrectos en el momento del ingreso a los servicios de urgencias del HUSVP; fueron más frecuentes los errores por exceso en la evaluación de la extensión de la superficie quemada, que por defecto. En la mayoría de los casos la magnitud del error fue suficiente para modificar la categoría de la quemadura (leve, moderada o severa). CONCLUSIONES: es necesario reforzar el conocimiento básico sobre la evaluación y tratamiento de las quemaduras en los médicos generales o especialistas que atienden servicios de urgencias.

Phase I pharmacokinetic and pharmacodynamic study of weekly 1-hour and 24-hour infusion BMS-214662, a farnesyltransferase inhibitor, in patients with advanced solid tumors.

PURPOSE: BMS-214662 is a potent, nonpeptide, small molecule inhibitor of human farnesyltransferase (FT). We have conducted a phase I pharmacokinetic (PK) and pharmacodynamic study of BMS-214662 administered intravenously weekly with 1- and 24-hour infusions. The objectives were to determine the dose-limiting toxicities and the recommended dose (RD), to describe PKs, and to evaluate the relationships between BMS-214662 exposure, FT inhibition, downstream signaling, and induction of apoptosis in tumor samples. PATIENTS AND METHODS: Patients with advanced solid tumors and adequate organ function were eligible. The dose was escalated according to a modified Fibonacci schedule. RESULTS: high (> 80%) but short-lived (< or = 6 hours) in the 1-hour infusion and moderate (> 40%) but long-lived (24 hours) in the 24-hour infusion. BMS-214662 induced apoptosis in tumors but did not inhibit MAPK signaling. CONCLUSION: BMS-214662 can be safely delivered in both the 1-hour and 24-hour infusions at biologically active doses, with the preclinical, PK, and pharmacodynamic profiles favoring the 24-hour schedule.

Management of adverse events and other practical considerations in patients receiving capecitabine (Xeloda).

As capecitabine (Xeloda) is converted to 5-FU within tumours it can produce 5-FU-like side effects. However, diarrhoea, stomatitis, nausea, alopecia and neutropenia are significantly less frequent than with i.v. 5-FU. Hand-foot syndrome (HFS) is the only clinical adverse event occurring more often during capecitabine treatment. These findings in MCRC have also been confirmed in a large phase III trial in early stage colon cancer (X-Act adjuvant study) and phase II clinical trials in metastatic breast cancer. Because capecitabine is taken in the outpatient setting, the nurse and/or supervising clinician are responsible for educating patients how to use it correctly and on the nature/recognition/severity of adverse events. Patients need to be aware that temporary interruptions/dose modifications do not reduce the overall efficacy of capecitabine and will most likely lead to a resolution of side effects. Consequently, oncology nurses will be assuming a more significant and pivotal role in the efficient education and support of patients during home-based therapy with capecitabine.