Cancer incidence in eighteen cities of the State of São Paulo, Brazil.

OBJECTIVE: As in Brazil cancer registries are mostly based on large cities, there are no estimates per state or per region and information on the disease incidence in the vast in-land areas is very scarce. An incidence survey was conducted in 18 major cities of the state of São Paulo, excluding the capital, aiming to collect information about cancer incidence in the state of São Paulo. METHODS: Of the 18 cities in state of São Paulo included in the survey, all had available resources for cancer management. Data from the year of 1991 were collected by the personnel of the Instituto Brasileiro de Geografia e Estatística (Brazilian Institute of Statistics), who were especially trained by the study coordinators at the Fundação Oncocentro de São Paulo (Cancer Center of São Paulo). The collected data were processed and analyzed at the Oncocentro. Data collection, processing, and analyses were performed according to the recommendations of the International Agency for Research on Cancer. RESULTS: Although some discrepancies were observed in cancer incidence rates between the cities, results obtained for all 18 cities combined were remarkably close to those recently found for the city of São Paulo in the year 1993. One remarkable finding was the relatively high cancer incidence rates in both sexes in the city of Santos. CONCLUSIONS: The very similar all-sites cancer incidence rates found in the year 1991, when compared to those for the city of São Paulo in the year 1993, are suggestive that all regions have common cancer-related factors. Nevertheless, other explanations, such as the inclusion in the study of prevalent cases, as well as of non-residents, may have occurred in both studies, biasing the results. There is a need of further studies to confirm the high cancer incidence in Santos.

Consideraciones sobre la anatomía quirúrgica de los elevadores del ano y de los esfínteres anales en la agenesia anorrectal y anal / Surgical anatomy of the levator ani and anal sphincters in anorectal malformations

Durante la anorrectoplastía en 28 niños, de ambos sexos, con agenesia anorrectal del ano, y analizado microscópicamente el esfínter anal interno. Se realizóuna estimación del valor funcional de los mismos tanto por la apreciación visual, como por la intensidad de la respuesta a los estímulos eléctricos. También fue verificada la posibilidad de normalizar su anatomía. Se procedió del siguiente modo: fue hecha una incisión en cruz inclinada en el periné, los cuatro colgajos fueron disecados y el esfínter anal externo fue divulsionado en la línea media. En el periné se hizo una incisión arciforme,disección entre la uretra y el asa de los elevadores para la sección de la fístula. Se resecó un fragmento rectal que fue para análisis microscópico. Los tres músculos fueron movilizados para poder abrir el canal rectoanal. Se comprobaron las siguientes anomalías: el esfínter anal externo era una placa sin orificio más corta que lo habitual, el asa del elevador contornea la uretra en los niños y la vagina o una fístula en las niñas. En la cara interna del asa se encontró un haz muscular longitudinal. Solamente existía la parte rectal del esfínter anal interno, que estaba rodeando la fístula. En general, los tres músculos eran robustos y respondían a estímulos eléctricos. Estos fueron manipulados para quedar en posición anatómica semejante a la normal

Consideraciones sobre la anatomía quirúrgica de los elevadores del ano y de los esfínteres anales en la agenesia anorrectal y anal / Surgical anatomy of the levator ani and anal sphincters in anorectal malformations

Durante la anorrectoplastía en 28 niños, de ambos sexos, con agenesia anorrectal del ano, y analizado microscópicamente el esfínter anal interno. Se realizóuna estimación del valor funcional de los mismos tanto por la apreciación visual, como por la intensidad de la respuesta a los estímulos eléctricos. También fue verificada la posibilidad de normalizar su anatomía. Se procedió del siguiente modo: fue hecha una incisión en cruz inclinada en el periné, los cuatro colgajos fueron disecados y el esfínter anal externo fue divulsionado en la línea media. En el periné se hizo una incisión arciforme,disección entre la uretra y el asa de los elevadores para la sección de la fístula. Se resecó un fragmento rectal que fue para análisis microscópico. Los tres músculos fueron movilizados para poder abrir el canal rectoanal. Se comprobaron las siguientes anomalías: el esfínter anal externo era una placa sin orificio más corta que lo habitual, el asa del elevador contornea la uretra en los niños y la vagina o una fístula en las niñas. En la cara interna del asa se encontró un haz muscular longitudinal. Solamente existía la parte rectal del esfínter anal interno, que estaba rodeando la fístula. En general, los tres músculos eran robustos y respondían a estímulos eléctricos. Estos fueron manipulados para quedar en posición anatómica semejante a la normal

Esophageal schistosomiasis in a patient with megaesophagus.

The authors report a case of schistosomiasis of uncommon location. A patient submitted to esophagectomy for Chagas' megaesophagus was found to have eggs of Schistosoma mansoni upon pathologic examination of the surgical specimen. The authors discuss the anatomopathological aspects of schistosomiasis, as well as its occurrence in other rare sites. To date, no references in literature have been found on the existence of esophageal schistosomiasis, and its association to Chagas' megaesophagus has never been described.

[Hystological types and mortality for gastric cancer in São Paulo, Brazil] / Tipos histológicos e mortalidade por câncer gástrico em São Paulo.

The objective of this study was to analyze, according to Lauren's classification, the prevalence of a hystological type - intestinal or diffuse -, among gastric carcinomas. The authors reviewed 650 hystological sections from a Hospital in the City of São Paulo during a 30-year period, following the definitions of the above classification. After the 50's the intestinal type proved to be prevalent, reaching 62.74% of all cases of gastric cancer in the 80's, and showing a ratio of intestinal/diffuse type of 1.71. Other large series of cases whose diagnoses followed the nomenclature of WHO Hystological Classification showed to be inadequate for this study. The prevalence of the intestinal type of gastric carcinoma seems to disagree with the decreasing rates of mortality due to this disease in the State of São Paulo, in the same period. Further studies, such as the analysis of incidence rates and prevalence of Helicobacter pylori, among others, are necessary for a better understanding of these data.

Homozygosity for a novel splice site mutation (2790-2 A--->G) preceding exon 15 of the CFTR gene in a cystic fibrosis patient of North-East Italian descent.

We have been screening a cohort of 225 chromosomes from cystic fibrosis patients for mutations in the cystic fibrosis transmembrane regulator gene using a combination of DGGE,RNA-SSCP and DNA sequencing. A novel splice site mutation was detected by multiplex DGGE in a homozygous patient. Restriction-site generating PCR (RG-PCR) analysis demonstrated that both parents carried the same mutation. The molecular haplotype was the same. All the known ancestors came from the same (Veneto) region, and no consanguinity was documented up to the sixth generation.

Increased incidence of cystic fibrosis gene mutations in adults with disseminated bronchiectasis.

In order to identify a possible hereditary predisposition to the development of obstructive pulmonary disease of unknown origin, we have looked for the presence of Cystic Fibrosis Transmembrane Regulator (CFTR) gene mutations in unrelated patients with no signs of Cystic Fibrosis (CF). We screened for 70 common mutations, and also for rare mutations by denaturing gradient gel electrophoresis analysis. In this search, different CFTR gene mutations (R75Q, delta F508, R1066C, M1137V and 3667ins4) were found in five out of 16 adult Italian patients with disseminated bronchiectasis, a significant increase over the expected frequency of carriers. Moreover, three rare CFTR gene DNA polymorphisms (G576A, R668C, and 2736 A-->G), not deemed to be the cause of CF, were found in two patients, one of which was a compound heterozygote with R1066C. These results indicate that CFTR gene mutations, and perhaps also DNA polymorphisms, may be involved in the etiopathogenesis of at least some cases of bronchiectasis.

Analysis of the complete coding region of the CFTR gene in a cohort of CF patients from north-eastern Italy: identification of 90% of the mutations.

A complete coding-region analysis on 225 cystic fibrosis (CF) chromosomes from a cohort that includes all the affected subjects born in two North-Eastern Italian regions over eight years was performed. In a previous study, we identified mutations on 166/225 (73.8%) CF chromosomes after screening for 62 mutations. To characterise the remaining 59 CF chromosomes, we carried out automated direct DNA sequencing (exons 9 and 13), RNA single-strand conformation polymorphism (exons 1-8 and 10-12) and denaturing gradient gel electrophoresis (exons 14a-24) of the 27 exons and flanking regions of the CF transmembrane conductance regulator gene. We identified 22 mutations, four of which are novel, viz. 711 + 5G-->A, R709X, 3132delTG and 2790-2A-->G, and we characterised 90.2% (203/225) of the CF chromosomes. Taking advantage of the homogeneity of the sample, an evaluation of the most important clinical parameters, assessed at the age of 12 years, is presented. We confirm some previously reported genotype-phenotype correlations and we report a new nonsense mutation (R709X) associated with a pancreatic sufficient phenotype.

Complete detection of mutations in cystic fibrosis patients of Native American origin.

An increased incidence of cystic fibrosis (CF) has been reported in some populations of Native Americans of the Southwest such as the Pueblo, which is a genetic isolate. As the most common mutation found in Caucasians (delta F508) was absent and only one chromosome carried the G542X mutation, we decided to analyze the entire coding sequence of the CFTR gene in eight Pueblo CF patients. We have identified four different mutations: G542X, R1162X, 3849+10kbC-->T, and D648V that account for these 16 haplotypes. The R1162X was found on 11 chromosomes. Using intragenic microsatellites, we have compared the haplotypes of those chromosomes to those of Italian origin where the R1162X mutation was initially reported. These haplotypes turned out to be identical, suggesting a common origin and an admixture with Italian or Spanish settlers, followed by typical founder effect. In contrast the 3849+10kbC-->T mutation, which was found on three chromosomes, is associated with different haplotypes than those on chromosomes carrying the same mutation in Caucasians. A novel mutation, D648V, observed on one chromosome has not been found outside the Pueblo population.

Screening of 62 mutations in a cohort of cystic fibrosis patients from north eastern Italy: their incidence and clinical features of defined genotypes.

The frequency of 62 different CFTR mutations in 225 chromosomes from a CF birth cohort, which includes all the affected subjects born in northeast Italy during a 10-year period of time, was investigated. New mutations were also searched by the analysis of 15 different exons. The total proportion of CF chromosomes with detectable mutations is 73.78%. Therefore although a considerable improvement in CF mutation detection in our population has been achieved, the search for other common and uncommon mutations should be continued. Moreover a carrier screening program should be postponed until reaching a cumulative proportion of known CF alleles of at least 90%. The correlations between the genotypes which have been identified and the main clinical features added some new information to the classification of CF mutations as pancreatically severe or mild ones.

Nonspecific necrotizing petrositis: an unusual complication of otitis in children.

Three cases of an unusual form of suppurative chronic otitis media in children 2 and 3 years old are presented. The clinical feature common to all cases was otorrhea, beginning at the first months of life, with a silent evolution to a complete peripheral facial paralysis. In one case the facial paralysis was bilateral. The treatment applied was surgical: subtotal petrosectomy. The surgical findings were bloody granulation tissue, sequestering completely the otic capsule. The facial nerve was destroyed almost the total length of the Fallopian canal in all cases. The histopathologic examination revealed a destructive and nonspecific chronic inflammatory process. The culture showed Gram-negative organisms, such as Pseudomonas aeruginosa and Proteus mirabilis. Etiologic aspects involved in the process are discussed and the name non-specific necrotizing petrositis is suggested to individualize this rare form of otitis which, with morbidity, leads to severe complications such as facial paralysis and deafness.

Linfoma folicular gigante e esquistossomose mansonica. / Giant follicular lymphoma and schistosomiasis mansoni

Os autores relatam seis casos de Linfoma Folicular Gigante do baco associado com esquistossomose mansonica, todos em mulheres naturais de zonas endemicas e residentes em Sao Paulo ha mais de nove anos. Apresentam o resumo clinico e a anatomia patologica de cada caso e tecem comentarios etiopatogenicos e pidemiologicos, principalmente quanto a possivel implicacao da esquistossomose mansonica nessa doenca linfo-proliferativa. Estes casos somam-se a oito ja descritos anteriormente por outros Autores em area endemica da esquistossomose no Brasil

Atypia in adenomas in three populations with different risk for large bowel cancer: Cali, São Paolo, and New Orleans.

A double-blind study of atypia in colorectal adenomatous polyps was done with autopsy material from Cali, Colombia (low colon risk), São Paulo, Brazil (intermediate risk), and New Orleans, Louisiana (high risk). An interobserver reproducibility of 82% was obtained. The prevalence of atypia in polyps increased with the cancer risk and also with the size of polyps, but some exceptions to the rule were found. The frequency of atypia increased steadily with age in Cali, but it plateaued after age 50 in the higher risk populations.

Cancer and "cancer related" colorectal lesions in São Paulo, Brazil.

A series of 832 necropsy specimens were studied grossly with a magnifying lens and all lesions identified were studied microscopically. The age and sex-specific prevalence of adenomatous and hyperplastic polyps is reported and results are compared with those of other populations. A correlation was made between polyps and cancer of the colon and rectum (407 cases). The data suggest that São Paulo is a community in a transitional stage between intermediate and high risk of cancer of the colon. The epidemiologic characteristics of lower rectum cancer are peculiar to some populations and appear unrelated to colon cancer. The black population of São Paulo has a higher prevalence than that reported for African negroes. The data also implicate adenomatous polyps, diverticulosis and hemorrhoids as being probably related diseases.