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.