RESUMO
The recurrence of Wilms tumor after a 5-year disease-free interval is rare. We present two patients who had recurrent disease after a disease-free interval of greater than 7 years. Three additional patients, registered with the National Wilms Tumor Study who had a recurrence after 5 years, are also described. Of these three patients, two had nephroblastomatosis. Because more patients are achieving long-term survival, careful surveillance after apparent "cure" is recommended, particularly if nephroblastomatosis is identified in the original nephrectomy specimen.
Assuntos
Neoplasias Renais/patologia , Tumor de Wilms/patologia , Pré-Escolar , Terapia Combinada , Feminino , Humanos , Lactente , Neoplasias Renais/cirurgia , Neoplasias Pulmonares/secundário , Masculino , Recidiva Local de Neoplasia , Nefrectomia , Tumor de Wilms/secundário , Tumor de Wilms/cirurgiaRESUMO
Major advances have been made against Wilms' tumor as a result of treatment methods developed by single institutions that then have been confirmed and extended by national cooperating groups. Better survival rates have been achieved, and therapy has been refined so that treatment can be reduced in early stage disease without jeopardizing tumor control. This results in fewer short- and long-term complications, an especially important consideration in children. Their organs and tissues are vulnerable to anti-mitotic treatments such as chemo- and radiotherapy, that can produce disabling if not lethal dysfunctions. This progress has been the result of the cooperative efforts by multiple specialists, and provides evidence of the value of such integrated studies. They have changed the outlook from a 90% death rate in the early years of this century to the 90% survival rate now possible with modern management.
RESUMO
Clinical and autopsy data on 25 patients with DiGeorge syndrome and its variants are presented. Congenital heart disease was the most common presenting complaint; 15 patients came to medical attention in the first 48 hours of life because of cyanosis, cardiac murmurs, or tachycardia and tachypnea. Two unusual anomalies, interrupted aortic arch or truncus arteriosus, were seen in 17 patients. Clinically documented hypocalcemia associated with seizures was seen in ten patients, with a median age at onset of eight days. Fifteen of our 25 patients died at less than one month of age. Most of the patients surviving the first month of life developed purulent rhinitis, maculopapular rashes, failure to thrive, and developmental delay. Sixteen patients had major congenital anomalies not localized to the anterior neck and thorax; these anomalies included arhinencephaly, cleft lip, palate, or uvula, diaphragmatic abnormalities, hydronephrosis, malrotation of the gut and imperforate anus. The 24 autopsied cases constitute 0.7% of the 3,469 sequential postmortem studies done in the period 1950--1975 at The Children's Orthopedic Hospital and Medical Center.