RESUMEN
The incidence, survival patterns, and presenting symptoms of children with medulloblastoma were studied. Data were ascertained from the Manchester Tumour Registry which is population-based and has collected data on all childhood malignancies in northwest England since 1954. Incidence rates standardized to the European standard population were calculated and Poisson regression models were used to examine temporal changes in the incidence rates during the period 1954 to 1997. Kaplan-Meier survival curves were derived and used to study changes in survival patterns. World-standardized incidence rates were 5.5 per million child years in males and 3.4 per million child years in females. Incidence rates increased from the 1950s to the 1980s but have declined recently. The 5-year survival rate has improved from 29 to 58% with similar rates for males and females. The 1-year survival rate has also improved, but females had worse survival at this point (58%) than males (75%). The type of symptom or sign at presentation is strongly affected by age, with 10 of the 22 recorded symptoms or signs showing significant age differences. The older the child is, the more likely is the presentation to show pressure features of headache, vomiting, and ophthalmic signs. Younger children present with non-specific features such as lethargy, behavioural disturbance, or increasing head size. Ataxia is seen in about 75% of children across the age range.
Asunto(s)
Neoplasias Cerebelosas/epidemiología , Meduloblastoma/epidemiología , Adolescente , Distribución por Edad , Ataxia/etiología , Neoplasias Cerebelosas/complicaciones , Neoplasias Cerebelosas/diagnóstico , Neoplasias Cerebelosas/terapia , Niño , Trastornos de la Conducta Infantil/etiología , Preescolar , Inglaterra/epidemiología , Femenino , Cefalea/etiología , Humanos , Incidencia , Lactante , Mortalidad Infantil , Recién Nacido , Masculino , Meduloblastoma/complicaciones , Meduloblastoma/diagnóstico , Meduloblastoma/terapia , Vigilancia de la Población , Modelos de Riesgos Proporcionales , Sistema de Registros , Análisis de Regresión , Distribución por Sexo , Tasa de Supervivencia , Vómitos/etiologíaRESUMEN
Anaplastic choroid plexus carcinoma is a tumour with a predilection for the posterior fossa of infants and can be difficult to distinguish histologically from medulloblastoma without the aid of immunocytochemistry using a panel of antibodies. Of a series of 17 choroid plexus carcinomas (five of which were classed as moderately differentiated and 12 as anaplastic) 17 expressed antigens to transthyretin, transferrin and cathepsin and 16 expressed carbonic anhydrase II. Eleven expressed at least one epithelial marker (cytokeratin or epithelial membrane antigen). In contrast, none of six medulloblastomas expressed epithelial markers, transrythetin, carbonic anhydrase II or transferrin, though three were positive with antibodies to cathepsin.
Asunto(s)
Carcinoma/química , Carcinoma/patología , Neoplasias del Plexo Coroideo/química , Neoplasias del Plexo Coroideo/patología , Anhidrasas Carbónicas/análisis , Carcinoma/enzimología , Catepsina D/análisis , Niño , Preescolar , Neoplasias del Plexo Coroideo/enzimología , Femenino , Humanos , Técnicas para Inmunoenzimas , Lactante , Masculino , Fosfopiruvato Hidratasa/análisis , Prealbúmina/análisis , Transferrina/análisisRESUMEN
OBJECTIVE: To assess the value of the autopsy findings on a series of infants dying with features of the oligohydramnios sequence, with particular reference to anomalies of the renal tract. DESIGN: Retrospective review. SETTING: Pathology departments serving three maternity units in Manchester. SUBJECTS: Eighty-nine infants having an autopsy examination between 1976 and 1990. RESULTS: Thirty-two (34%) infants had bilateral renal agenesis, 30 (34%) had bilateral cystic dysplasia, eight (9%) had unilateral agenesis with unilateral cystic dysplasia, four (4%) had renal histology characteristic of a recessively inherited disorder (two cases of renal tubular dysgenesis and two cases of autosomal recessive (infantile) polycystic disease), nine (10%) had minor urinary tract anomalies, and three (3%) had morphologically normal renal tracts. Forty-eight (54%) infants had congenital abnormalities other than those resulting from oligohydramnios sequence; most commonly, these were anomalies of the sporadic VATER association, but in four infants the extra renal anomalies present allowed recognition of a recessively inherited syndrome (Meckel's in three cases, Smith-Lemli-Opitz in one). CONCLUSIONS: A detailed autopsy is vital in assessment of infants with oligohydramnios sequence resulting from a congenital abnormality of the kidneys or urinary tract. This applies equally to second trimester fetuses following miscarriage or therapeutic abortion, to stillborn infants, or to neonatal deaths.
Asunto(s)
Riñón/anomalías , Oligohidramnios/patología , Anomalías Múltiples , Humanos , Recién Nacido , Enfermedades Renales Quísticas/patología , Oligohidramnios/complicaciones , Estudios Retrospectivos , Trisomía , Uréter/anomalías , Vejiga Urinaria/anomalíasRESUMEN
A case of clear cell sarcoma of the kidney is described in a 4-month-old male infant. The tumor had a typical histological pattern, consisting of sheets of tumor cells possessing "empty" nuclei set within a delicate capillary network. There was extensive necrosis and tumor cell cytoplasm contained vimentin. This tumor occurs only rarely in infants less than six months of age. The different renal neoplasms of childhood have diverse prognoses and demand specific therapy so that accurate diagnosis is mandatory. The present case illustrates that clear cell sarcoma, a highly malignant neoplasm, must be considered in the differential diagnosis of a renal mass occurring in this age group.
Asunto(s)
Neoplasias Renales/patología , Sarcoma/patología , Preescolar , Humanos , MasculinoRESUMEN
A patient receiving haemodialysis for 15 years developed systemic amyloidosis of beta 2 microglobulin type. Noticeable deposits of amyloid were present in the myocardium, intervertebral discs, joint cartilages and tendons. Less conspicuous amounts were present in blood vessel walls in the lungs, liver, adrenal glands and brain, and within the stroma of the prostate, testis and kidney, often with foci of calcification.
Asunto(s)
Amiloidosis/patología , Microglobulina beta-2/análisis , Vasos Sanguíneos/metabolismo , Huesos/metabolismo , Cartílago Articular/metabolismo , Humanos , Técnicas para Inmunoenzimas , Disco Intervertebral/metabolismo , Fallo Renal Crónico/complicaciones , Fallo Renal Crónico/terapia , Masculino , Persona de Mediana Edad , Miocardio/metabolismo , Diálisis RenalRESUMEN
Two sisters developed a bullous skin disease in early childhood. The disease had features of junctional epidermolysis bullosa but differed clinically from previously recorded variants.
Asunto(s)
Epidermólisis Ampollosa de la Unión/patología , Piel/ultraestructura , Adulto , Epidermólisis Ampollosa de la Unión/etnología , Familia , Femenino , Humanos , Microscopía ElectrónicaRESUMEN
A 35-year-old man who had been treated by hemodialysis for 15 years suffered from systemic amyloidosis. It was identified as a beta 2-microglobulin type according to immunohistochemistry. Extensive amyloid deposits were found in myocardium, cartilages of intervertebral discs and in carpal tunnel ligament. Less conspicuous deposits comprised blood vessels of lungs, liver, suprarenal glands, brain, and stroma of prostate gland, testicles, kidney, often combined with calcified foci.
Asunto(s)
Amiloidosis/patología , Microglobulina beta-2/análisis , Adulto , Amiloidosis/metabolismo , Humanos , MasculinoRESUMEN
Histological examination of a tumor centered in the body of the pancreas of a 65-year-old Iranian man revealed it to have a substantial component in which osteoclast-like giant cells were set within a stroma of pleomorphic mononuclear cells though other areas were composed of conventional adenocarcinoma. Immunocytochemistry was used to investigate the differentiation of the various component cells of the tumor. The carcinoma cells of the usual type expressed epithelial antigens (EMA and cytokeratin). The giant cells expressed vimentin and showed membrane staining with anti-LCA, in common with examples of cells originating from the mononuclear phagocytic system, including normal osteoclasts. The accompanying stromal cells expressed vimentin only. This implies that the giant cells are likely to have their origin in the bone marrow, whereas the mononuclear stromal cells that separate them may represent tumor cells that have lost their epithelial phenotype. The giant cells are therefore an unusual tissue response to the presence of the carcinoma.
Asunto(s)
Adenocarcinoma/patología , Células Gigantes/patología , Osteoclastos/patología , Neoplasias Pancreáticas/patología , Adenocarcinoma/química , Anciano , Membrana Celular/química , Antígenos HLA/análisis , Humanos , Técnicas para Inmunoenzimas , Irán/etnología , Masculino , Glicoproteínas de Membrana/análisis , Mucina-1 , Neoplasias Pancreáticas/química , Vimentina/análisisRESUMEN
A 3 1/2-year-old boy presented with a palpable hepatic tumor thought on clinical and radiological grounds to be a metastasis but which was found to be an inflammatory pseudotumor on histological examination. Eighteen months previously he had received chemotherapy and radiotherapy for a stage IV Wilms' tumor, which had been surgically excised 4 months after commencing treatment. This case illustrates the importance of obtaining a histological diagnosis in the management of patients with malignant tumors.
Asunto(s)
Granuloma de Células Plasmáticas/patología , Neoplasias Renales/patología , Hepatopatías/patología , Tumor de Wilms/patología , Preescolar , Diagnóstico Diferencial , Humanos , Neoplasias Pulmonares/secundario , Masculino , Tumor de Wilms/secundarioRESUMEN
The hepatocellular carcinomas that develop in patients treated with androgens have previously been associated with a benign clinical outcome. We describe a man who developed a hepatocellular carcinoma after 24 years of androgen treatment, whose tumour initially showed partial regression after withdrawal of androgens but subsequently pursued an aggressive and fatal course.
Asunto(s)
Carcinoma Hepatocelular/inducido químicamente , Neoplasias Hepáticas/inducido químicamente , Metiltestosterona/efectos adversos , Carcinoma Hepatocelular/patología , Humanos , Hipogonadismo/tratamiento farmacológico , Neoplasias Hepáticas/patología , Cuidados a Largo Plazo , Masculino , Persona de Mediana EdadRESUMEN
Endocardial fibroelastosis, defined as an endocardium in excess of 30 microns thick, was found in 10 out of 34 cases of hydrops fetalis in a review of 1589 perinatal necropsies carried out between 1976 and 1989. The infants comprised 16 cases of rhesus haemolytic disease, of whom three had endocardial fibroelastosis, and 18 cases of non-rhesus hydrops, of whom seven had endocardial fibroelastosis. Intrauterine congestive heart failure was thought to have been the probable cause of hydrops in eight of the 10 infants with endocardial fibroelastosis. None of an age matched control group without endocardial fibroelastosis had evidence of congestive cardiac failure. These observations support the hypothesis that endocardial fibroelastosis is an endocardial response to chronic prenatal myocardial stress.
Asunto(s)
Fibroelastosis Endocárdica/patología , Endocardio/patología , Hidropesía Fetal/patología , Autopsia , Fibroelastosis Endocárdica/etiología , Eritroblastosis Fetal/complicaciones , Femenino , Muerte Fetal , Insuficiencia Cardíaca/complicaciones , Humanos , Hidropesía Fetal/complicaciones , Recién Nacido , Masculino , EmbarazoAsunto(s)
Neoplasias Primarias Múltiples/etiología , Neoplasias Inducidas por Radiación/etiología , Neurilemoma/etiología , Neoplasias del Sistema Nervioso Periférico/etiología , Adulto , Neoplasias de la Mama/radioterapia , Femenino , Enfermedad de Hodgkin/radioterapia , Humanos , Persona de Mediana Edad , Neoplasias Primarias Múltiples/patología , Neoplasias Inducidas por Radiación/patología , Neurilemoma/patología , Neoplasias del Sistema Nervioso Periférico/patologíaRESUMEN
A polyclonal antiserum to toxic shock syndrome toxin (TSST-1) and a standard immunoperoxidase technique were used on formalin fixed tissues from 50 cases of sudden infant death syndrome (SIDS) to determine if the syndrome was associated with bacterial infection. There was strong specific staining in the renal tubular cells in nine (18%) cases. A similar pattern of staining was seen in three of a series of 50 kidneys selected for comparison from a wide range of necropsy cases. The staining was finely granular within the cytoplasm of proximal convoluted tubular cells and diffuse in tubular cell nuclei. In an attempt to validate the staining pattern the immunoperoxidase technique was also performed on formalin fixed kidneys from rats which had been given intravenous injections of crude bacterial products containing TSST-1. These showed coarse granular cytoplasmic staining in proximal convoluted tubules with some diffuse nuclear staining. This pattern was not seen in controls injected with saline. These results indicate that TSST-1 might have a pathogenic role in some cases of SIDS.
Asunto(s)
Toxinas Bacterianas , Staphylococcus aureus , Muerte Súbita del Lactante/etiología , Superantígenos , Enterotoxinas , Humanos , Lactante , Túbulos Renales/análisisRESUMEN
A 52 year old man with newly diagnosed polycythaemia vera in proliferative phase developed widespread extramedullary haemopoiesis (EMH), and died as a result of cervical cord compression. At necropsy, microscopic areas of primitive cells characteristic of granulocytic sarcoma were found within a large tumour of EMH in the right retroperitoneal region. Ultrastructural analysis showed unusual hexagonal inclusions within the cytoplasm of these primitive cells. Clinicians should be aware of the possibility of discrete haemopoietic tumours, whether EMH or granulocytic sarcoma, in patients with polycythaemia vera as well as in other myeloproliferative disorders.