Plasma level of LDL-cholesterol at diagnosis is a predictor factor of breast tumor progression.

BACKGROUND: Among women, breast cancer (BC) is the leading cancer and the most common cause of cancer-related death between 30 and 69 years. Although lifestyle and diet are considered to have a role in global BC incidence pattern, the specific influence of dyslipidemia in BC onset and progression is not yet completely understood. METHODS: Fasting lipid profile (total cholesterol, LDL-C, HDL-C, and triglycerides) was prospectively assessed in 244 women with BC who were enrolled according to pre-set inclusion criteria: diagnosis of non-hereditary invasive ductal carcinoma; selection for surgery as first treatment, and no history of treatment with lipid-lowering or anti-diabetic drugs in the previous year. Pathological and clinical follow-up data were recorded for further inclusion in the statistical analysis. RESULTS: Univariate associations show that BC patients with higher levels of LDL-C at diagnosis have tumors that are larger, with higher differentiation grade, higher proliferative rate (assessed by Ki67 immunostaining), are more frequently Her2-neu positive and are diagnosed in more advanced stages. Cox regression model for disease-free survival (DFS), adjusted to tumor T and N stages of TNM classification, and immunohistochemical subtypes, revealed that high LDL-C at diagnosis is associated with poor DFS. At 25 months of follow up, DFS is 12% higher in BC patients within the third LDL-C tertile compared to those in the first tertile. CONCLUSIONS: This is a prospective study where LDL-C levels, at diagnosis, emerge as a prognostic factor; and this parameter can be useful in the identification and follow-up of high-risk groups. Our results further support a possible role for systemic cholesterol in BC progression and show that cholesterol metabolism may be an important therapeutic target in BC patients.

5-Bromo-2’-deoxyuridine induces visible morphological alteration in the DNA puffs of the anterior salivary gland region of Bradysia hygida (Diptera, Sciaridae)

5-Bromo-2’-deoxyuridine (BrdUrd) has long been known to interfere with cell differentiation. We found that treatment ofBradysia hygida larvae with BrdUrd during DNA puff anlage formation in the polytene chromosomes of the salivary gland S1 region noticeably affects anlage morphology. However, it does not affect subsequent metamorphosis to the adult stage. The chromatin of the chromosomal sites that would normally form DNA puffs remains very compact and DNA puff expansion does not occur with administration of 4 to 8 mM BrdUrd. Injection of BrdUrd at different ages provoked a gradient of compaction of the DNA puff chromatin, leading to the formation of very small to almost normal puffs. By immunodetection, we show that the analogue is preferentially incorporated into the DNA puff anlages. When BrdUrd is injected in a mixture with thymidine, it is not incorporated into the DNA, and normal DNA puffs form. Therefore, incorporation of this analogue into the amplified DNA seems to be the cause of this extreme compaction. Autoradiographic experiments and silver grains counting showed that this treatment decreases the efficiency of RNA synthesis at DNA puff anlages.

Gastric and intestinal differentiation in Barrett's metaplasia and associated adenocarcinoma.

Intestinal metaplasia is a prerequisite criterion for the diagnosis of Barrett's metaplasia and the sole columnar esophageal lining associated with malignancy. It is recognized by the presence of goblet cells, but columnar non-goblet elements, producing gastric or intestinal proteins, are the prevalent cell population. The cellular heterogeneity of Barrett's metaplasia is well documented but the relationship between the distinct cell subtypes and neoplasia is unclear. Our aim was to clarify the relationship between the different metaplastic populations and malignancy in order to investigate putative markers for risk stratification of Barrett's patients. We studied 46 columnar-lined esophageal segments, 15 with associated adenocarcinoma. The presence of the gastric, MUC5AC and MUC6, and the intestinal, MUC2, proteins was evaluated in metaplastic (columnar and goblet) and neoplastic cells. In neoplasia MUC5AC and MUC6 were detected in 100% and 86.6% of the cases, respectively. In metaplasia there were no differences in MUC5AC and MUC6 immunoreactivity, between cases with and without associated neoplasia, except for goblet elements producing MUC6 that were exclusive of metaplasia adjacent to adenocarcinoma (P < 0.05). MUC2 was present in 86.6% of the neoplasia. In metaplasia it was restricted to Barrett's cases and was more frequent in areas with intestinal metaplasia. Columnar-lined esophagus without intestinal metaplasia did not express MUC2. Our study suggests a relationship between the metaplastic population with gastric phenotype and malignancy, and points to the involvement of columnar as well as goblet elements in tumorigenesis. The association between goblet cells aberrantly producing MUC6 and the presence of neoplasia suggests they may be useful for risk stratification.

Enterocytic columnar non-goblet cells of Barrett's esophagus--an immunohistochemical demonstration of association with malignant evolution.

Barrett's epithelium (BE), the esophageal columnar-lining with intestinal differentiation, is a premalignant condition predisposing to adenocarcinoma. Columnar cells are the prevalent element of BE, but the hallmark of intestinal differentiation is the goblet population that defines the specialised columnar epithelium (SCE). We have demonstrated that columnar cells adjacent to Barrett's adenocarcinoma (BA) exhibit enterocytic features in areas with and without SCE. Nevertheless, the relationship between malignancy and the presence of these elements is not established. To investigate whether intestinal differentiated cells, other than goblet cells, are associated to neoplasia we compared the prevalence of enterocytic features in columnar elements with and without associated BA through the use of sucrase-isomaltase (SI) immunoreactivity in 31 columnar esophageal segments (CLES) and 12 BA. In metaplasia, SI was only expressed at the columnar cells. Apical staining was exclusive of CLES with SCE. SI was present at the cytoplasm in 22.2% of CLES without SCE. Apical SI occurred in BE with and without carcinoma, similarly in areas with and without SCE (p = 0.11 and p = 0.50, respectively). In areas with SCE, columnar cells with apical SI were more frequent in cases of BE adjacent to carcinoma than in cases without neoplasia but the difference did not reach significance (p = 0.053). In areas without SCE, apical SI was significantly (p = 0.01) more frequent in cases with carcinoma. Apical SI was equally found in neoplastic as in metaplastic areas, with and without SCE, (p = 0.07 and p = 0.40, respectively). In conclusion this study on the frequency of SI on CLES with and without neoplasia demonstrated that additionally to SCE, metaplastic enterocytic cells are also associated with malignancy. It also confirmed that the presence of intestinal features are underestimated if only goblet elements are used for its identification, reinforcing the utility of the immunohistochemical recognition of enterocytic characteristics for establishing the diagnosis of BE.

Recurrent columnar-lined esophageal segments--study of the phenotypic characteristics using intestinal markers.

Barrett's metaplasia is recognized by specialized columnar epithelium on the distal esophagus. The events involved in the transformation from squamous to Barrett's epithelium remain unclear. The present study describes the characteristics observed during the recurrence of four cases of columnar-lined esophagus. Red velvet, gastric-like, esophageal mucosa was observed to develop above the anastomosis during follow-up of four patients submitted to surgery for esophageal and junctional adenocarcinoma. The areas of recurrence were associated with reflux symptoms and inflammation, with ulceration in two cases. Biopsies from the upper gastrointestinal endoscopies were examined histologically using periodic acid-Schiff/Alcian blue to detect acid mucins and a monoclonal antibody raised against the enterocytic enzyme sucrase-isomaltase. In all cases the recurrent columnar-lined segments displayed intestinal features recognized morphologically, histochemically, and/or immunohistochemically. There was no evidence of specialized columnar epithelium in three cases. The fourth patient developed specialized columnar epithelium during the tenth year of surveillance. The presence of AB-positive columnar cells was a frequent and early event. Columnar cells with unequivocal apical sucrase-isomaltase were observed only in association with specialized columnar epithelium. Four conclusions were reached: that the development of columnar-lined mucosa without specialized columnar epithelium may be the earliest event in Barrett's metaplasia; that histochemistry is a useful method of recognizing a population with cryptic intestinal features; that acid mucin secretion precedes the production of enterocytic enzymes by columnar cells; and that a cell population with enterocytic differentiation, as assessed by sucrase-isomaltase expression, is associated with the development of specialized columnar epithelium. These characteristics of Barrett's esophagus development are clinically relevant as they suggest that patients with columnar-lined esophagus without specialized columnar epithelium may acquire 'true' intestinal phenotype, justifying them being considered as high- risk patients.

Adenocarcinoma of the esophagogastric junction: could the characteristics of adjacent intestinal metaplasia help in the understanding of biopathogenesis?

We report a case of early adenocarcinoma arising in foci of intestinal metaplasia (IM) at a normal-appearing gastroesophageal junction (GEJ). The tumor infiltrated the submucosa without nodal involvement (T1N0). Non-neoplastic mucosa adjacent to neoplasia had foci of incomplete IM with a band-like CK20 positivity of the surface epithelium and a diffuse CK7 staining of both superficial and deep glands. There were histological features of reflux esophagitis as well as chronic non-atrophic, Helicobacter pylori-related pangastritis, without IM, at the extensively assessed gastric mucosa. In this case, the CK7/20 pattern of IM adjacent to neoplasia, the demonstration of reflux esophagitis, and the absence of IM in the stomach favor the theory that the pathogenesis of IM and associated adenocarcinoma of the GEJ is related to gastroesophageal reflux rather than H. pylori infection.

The control of BhB10-1 gene expression in the salivary gland of Bradysia hygida (Diptera, Sciaridae) is disrupted in vivo by a delayed effect of cycloheximide.

BhB10-1 is an amplified gene present in DNA puff B10. This gene is very active in the salivary gland regions S1 and S3 at the end of the larval development. Two transcripts of this gene, 1.3 and 1.1 kb in size, were detected. A secretory protein, SP23, is the product of BhB10-1. In this work, we present evidence supporting the hypothesis that a biphasic process of mRNA degradation is an important component in the control of BhB10-1 gene expression. The 1.3 kb transcript, by a process of poly(A) tail shortening, is converted to the inactive transcript of 1.1 kb which is detected during and after the period of SP23 expression. Cycloheximide in very low concentration, if applied at a proper time, can disrupt this process leading to extended periods of 1.3 kb RNA detection and SP23 synthesis. A tentative model is proposed to explain this phenomenon.

FISH studies in a girl with sporadic aniridia and an apparently balanced de novo t(11; 13)(p13; q33) translocation detect a microdeletion involving the WAGR region

O estudo citogenético convencional em uma menina com aniridia esporádica resultou em uma aparente translocaçäo balanceada t(11;13)(p13;q33) de novo. Entretanto, o estudo citogenético pela hibridaçäo in situ fluorescente (FISH) detectou a presença de uma deleçäo críptica 11p13p14, incluindo a regiäo WAGR e envolvendo aproximadamente 7.5 Mb de DNA, deletando os genes PAX6 e WT1. Estes resultados correlacionam-se com o quadro clínico da paciente e a coloca em alto risco de desenvolver tumor de Wilms. A ausência de retardo mental na paciente indica que a posiçäo distal do ponto de quebra poderá refinar o mapeamento do locus retardo mental na síndrome de genes contíguos WAGR (Wilms, aniridia, anomalias genitais e retardo mental).

A dual role of 20-hydroxyecdysone in the control of protein synthesis related to DNA puff activity in the anterior region of Bradysia hygida (Diptera, Sciaridae) salivary gland.

During the last 30 h of the larval stage, the salivary glands of Bradysia hygida show the amplification of some genes, resulting in the formation of two successive groups of DNA puffs, which direct the synthesis of two different sets of polypeptides. Incubation of anterior (S1) salivary gland regions, at age E7, beginning of first group of DNA puffs activity, in culture medium for 2 to 10 h results in a decrease in the synthesis of the polypeptides characteristic of this period. However, during subsequent incubation (from E7 to E7+12 h-24 h), when the second group of DNA puffs is active, S1 regions were able to synthesize some polypeptides characteristic of this period. The role of 20-OH ecdysone was studied, in vitro and in vivo, during these two periods of protein synthesis in S1 regions. The presence of the hormone was shown to be necessary to maintain, in vitro, the synthesis of the first set of polypeptides and was strongly inhibitory, in vitro and in vivo, to the synthesis of the second set of polypeptides. Thus, it is likely that the activity of the two distinct groups of DNA puffs is under opposite 20-OH-ecdysone control mechanisms.

Non-goblet cell population of Barrett's esophagus: an immunohistochemical demonstration of intestinal differentiation.

Barrett's esophagus develops with the following 2 distinct types of lining mucosa: with and without specialized intestinal metaplasia (SIM). Goblet cells found only in SIM areas identify an intestinal phenotype, recognized as the histological hallmark diagnosing Barrett's metaplasia, and selecting high-risk patients for endoscopic surveillance. The columnar non-goblet cells are the major component of the heterogeneous Barrett's metaplastic cell population and are present in areas either with or without SIM. Their significance in the differentiation of columnar-lined esophagus, and their relationship to malignancy, is still unclear. This immunohistochemical study used two markers of enterocytic differentiation, to explore the intestinal phenotype of the non-goblet cell population of Barrett's epithelium and Barrett's-associated adenocarcinoma cells. Sucrase-isomaltase (SI) and dipeptidilpeptidase IV (DPP) immunoexpression was assessed in paraffin-embedded samples of 12 surgical specimens containing Barrett's esophageal mucosa in association with adenocarcinoma/high grade dysplasia. Ileal mucosa and mucosa from normal gastric and esophageal segments of the surgical specimen were used as positive and negative controls, respectively. SI and DPP were expressed by the neoplastic cells and the columnar non-goblet, being negative in goblet cells. The localization of the enzymes was predominantly apical for SI and cytoplasmatic for DPP. There was immunoreactivity for SI in 58.3% of the carcinomas and in 66.6% of Barrett's mucosa, with equal frequency in areas with and without SIM. DPP was identified in 66.6% of the carcinomas, in 50% of the cases of Barrett's metaplasia with SIM, and in 75% of those without SIM. The columnar non-goblet cell components of Barrett's metaplasia contain small intestine enzymes in the areas either with or without SIM, which suggests that they identify an "incomplete form" of intestinal metaplasia. The demonstration that the two enzymes, SI and DPP, are produced by the columnar non-goblet cell metaplastic population and by the neoplastic cells of the associated adenocarcinoma, indicates that, in addition to the goblet cells, the non-goblet elements may also be involved in the malignant transformation of Barrett's esophagus.

A de novo complex chromosomal rearrangement with nine breakpoints characterized by FISH in a boy with mild mental retardation, developmental delay, short stature and microcephaly.

A de novo complex chromosome rearrangement (CCR) involving chromosomes 1, 6, 7, 15 and Y was detected in a boy with mental retardation, short stature, and microcephaly. Fluorescence in situ hybridisation (FISH) with whole chromosome painting libraries, band-specific cosmids and telomeric probes was essential for the characterisation of the rearrangement. The CCR was shown to be the result of at least nine chromosomal breaks and involved the alternating insertion of two segments of the short arm of chromosome 1 and two segments of the long arm of chromosome 6 into a novel derived chromosome 7. A non-reciprocal translocation between the distal short arm of the same chromosome 7 and the distal long arm of the Y chromosome was also found, together with a paracentric inversion of the long arm of chromosome 15. The only detectable imbalance was a deletion of the heterochromatic Yq telomeric region. FISH investigations in this case have revealed an additional complexity in this CCR, which has implications for reproductive risk assessment and genetic counselling.

Does the new TNM classification (1997) improve prognostic stratification in gastric cancer submitted to R0 surgery?

AIMS: To determine the eventual advantage of the new 1997 TNM as prognosis predictor for gastric cancer patients submitted to an R0 resection and to compare it with two other lymph-node involvement classifications, the 1990 TNM and the Okusa system. METHODS: From January 1980 to December 1995, an R0 resection was performed as primary therapy in 275 cases of gastric cancer. These operations consisted of a total or sub-total gastrectomy and of a D2 type lymph-node dissection. Tumour classification was performed according to 1990 and 1997 TNM systems, and to the Okusa lymph node classification. The statistical methods used to evaluate prognostic value were: Kaplan-Meier survival estimates; the log-rank test for univariate analysis; and Cox's model for multivariate analysis. RESULTS: The 1990 TNM showed the best stratification power in univariate analysis. In multivariate analysis, the Okusa classification was identified as the best prognostic index (P<0.01). The 1997 TNM showed worse stratification capability than the two other systems. CONCLUSIONS: In the present series, the new TNM (1997) did not improve the prognostic stratification of lymph-node involvement. An adequate and universal system for lymph-node stratification is necessary and further validation of these classifications is needed.

The DNA puff BhB10-1 gene encodes a glycine-rich protein secreted by the late stage larval salivary glands of Bradysia hygida.

We present the molecular characterization of a gene of Bradysia hygida DNA puff B10 whose temporal expression in the salivary gland correlates with the puff expansion. The transcription unit of this gene, named BhB10-1, was mapped in a 2-kb EcoRI genomic fragment that is amplified in the salivary gland of late fourth instar larvae. Its 1.3-kb transcript undergoes poly-A tail shortening during development, indicating that post-transcriptional controls as well as transcription activation are involved in the temporal regulation of the BhB10-1 gene. Analysis of the deduced amino acid sequence from the cDNA indicates that the BhB10-1 protein is a glycine-rich secretory protein. A BhB10-1-fusion protein expressed in bacteria was used to raise polyclonal antibodies. Using an immunopurified antibody, we identified the product of the DNA puff BhB10-1 gene as a 23-kDa polypeptide that is produced mainly by the salivary gland regions S1 and S3 and is present in the saliva of late larvae. This is the first direct identification of a protein encoded by a DNA puff amplified gene.

Short segments of Barrett's epithelium and intestinal metaplasia in normal appearing oesophagogastric junctions: the same or two different entities?

BACKGROUND: Endoscopic diagnosis of short segments of Barrett's epithelium (SSBE)' is difficult and its meaning in terms of the presence of specialised columnar epithelium (SCE) has not been prospectively evaluated. AIMS: To evaluate the prevalence of SCE in patients with an endoscopic diagnosis of SSBE and in individuals with normal appearing oesophagogastric junctions, and to compare the clinical characteristics of these two groups. PATIENTS: Thirty one patients with an endoscopic diagnosis of short Barrett's oesophagus, less than 3 cm in length (group A), and 44 consecutive patients with normal appearing oesophagogastric junctions (group B). METHODS: Multiple biopsies were performed in suspicious epithelium and at the oesophagogastric junction in groups A and B, respectively. RESULTS: Age and sex distribution were similar in both groups. Reflux symptoms were more frequent in group A (p < 0.001), as were endoscopic and histological signs of oesophagitis (p < 0.001 and p = 0.001, respectively). SCE was found in 61.3% of group A patients compared with 25% in group B (p < 0.002), with men predominating in group A while women were more frequent in group B (p = 0.02). The differences in reflux symptoms and endoscopic/histological oesophagitis remained significant. CONCLUSIONS: These results show that endoscopic diagnosis of SSBE is associated with a high prevalence of SCE, significantly higher than that found in normal appearing oesophagogastric junctions. Differences between patients with SCE in the two groups suggest they may represent two different entities.

Is Barrett's esophagus the precursor of most adenocarcinomas of the esophagus and cardia? A biochemical study.

OBJECTIVE: To obtain biochemical evidence that Barrett's esophagus (BE) is the precursor of most adenocarcinomas (Adc) of the esophagus and cardia. SUMMARY BACKGROUND DATA: Based on morphologic data, BE was previously proposed as the precursor of most Adc of the esophagus. This hypothesis would receive strong support if biochemical evidence were found to demonstrate a pattern common to BE and Adc of the esophagus and cardia. METHODS: We studied the presence of intestinal-type proteins sucrase-isomaltase (SI) and crypt Cell Antigen (CCAg) in BE, Barrett's Adc, and esophageal-cardial Adc without BE. In each case specimens were collected from normal esophagus, stomach, tumor, and BE mucosa when present. To study related conditions, five specimens of peptic esophagitis and of squamous cell carcinoma were also analyzed. An indirect immunofluorescence technique was employed and sections were analyzed with laser confocal microscopy imaging. RESULTS: Most Barrett's mucosa specimens stained positively for SI (93%) and CCAg (89%). These proteins were detected in BE independently of the type of metaplasia, the coexistence of dysplasia, or the presence of associated Adc. SI and CCAg were present in 25 (96%) and 24 (92%) of the cases of Adc respectively. No statistical difference was detected in SI and CCAg expression between Adc samples with and without BE, between BE and Adc samples with or without BE, and between tumors located in the esophagus versus the cardia. No staining for these proteins was detected in stomach or esophageal mucosa, in submucosal glands of the esophagus, in peptic esophagitis or squamous cell carcinoma. CONCLUSION: These data show that BE and Adc of the esophagus and cardia have a similar phenotype and support the hypothesis that most of these tumors probably originate from preexisting BE.

A 28-fold increase in secretory protein synthesis is associated with DNA puff activity in the salivary gland of Bradysia hygida (Diptera, Sciaridae).

When the first group of DNA puffs is active in the salivary gland regions S1 and S3 of Bradysia hygida larvae, there is a large increase in the production and secretion of new salivary proteins demonstrable by [3H]-Leu incorporation. The present study shows that protein separation by SDS-PAGE and detection by fluorography demonstrated that these polypeptides range in molecular mass from about 23 to 100 kDa. Furthermore, these proteins were synthesized mainly in the S1 and S3 salivary gland regions where the DNA puffs C7, C5, C4 and B10 are conspicuous, while in the S2 region protein synthesis was very low. Others have shown that the extent of amplification for DNA sequences that code for mRNA in the DNA puffs C4 and B10 was about 22 and 10 times, respectively. The present data for this group of DNA puffs are consistent with the proposition that gene amplification is necessary to provide some cells with additional gene copies for the production of massive amounts of proteins within a short period of time.

Partial characterization of cytoplasmic compartments involved in the endocytic process of Tritrichomonas foetus.

The endocytic pathway of Tritrichomonas foetus, a parasitic protozoan of cattle, was studied using (a) vital dyes, such as Lucifer yellow, neutral red and acridine orange, (b) cationized ferritin, (c) gold-labeled lactoferrin and lectins: HPA, UEA, PNA and LPA, and (d) DAMP (3-(2,4-dinitroanilino) 3' amino-N-methyldipropylamine). Light and confocal laser microscopy as well as transmission electron microscopy were used in this study. Assays were monitored by fluorescence and electron microscopy after exposing the parasites to different conditions. Cells that were incubated at 15 degrees C or 20 degrees C with gold-labeled lactoferrin and processed for electron microscopy show that of 15 degrees C this ligand is found only in an early endosomal compartment and at 20 degrees C it is found in late endosomes but not in lysosomes. Immunocytochemical data from cryosections using DAMP as a pH probe show that T. foetus has acidic compartments, with a pH range of 5.2 to 6.6, with variable morphology, localization and size. Lectin-binding sites and anionic sites were also internalized and appear to be associated with membranes lining the vacuoles. Images of patching and shedding of these sites were also observed when HPA and UEA were used.

Xp-duplications with and without sex reversal.

Duplications in Xp including the DSS (dosage sensitive sex reversal) region cause male to female sex reversal. We investigated two patients from families with Xp duplications. The first case was one of two sisters with karyotype 46,XY,der(22),t(X;22)(p11.3;p11)mat and unambiguous female genitalia. The living sister was developmentally retarded, and showed multiple dysmorphic features and an acrocallosal syndrome. The second case was a boy with a maternally inherited direct duplication of Xp21.3-pter with the breakpoint close to the DSS locus. He had multiple abnormalities and micropenis, but otherwise unambiguous male genitalia. We performed quantitative Southern blot analysis with probes from Xp22.13 to p21.2 to define the duplicated region. Clinical, cytogenetic, and molecular data from both patients were compared with those of previously reported related cases. A comparison of the extragenital symptoms revealed no differences between patients with or without sex reversal. In both cases, the symptoms were non-specific. Among 22 patients with a duplication in Xp, nine had unambiguous female genitalia and a well-documented duplication of the DSS region. Two patients with duplication of DSS showed ambiguous external genitalia. From these data, we conclude that induction of testicular tissue may start in these patients, but that the type of genitalia depends on the degree of subsequent degeneration by a gene in DSS.

Autosomal dominant osteosclerosis type Stanescu: the third family.

We describe a family with Stanescu osteosclerosis. The propositus and his mother were short and had cortical sclerosis of the long bones, deficient facial sinus development, cranial bone malformations, and normal intelligence. To the best of our knowledge, only two such families have been described previously. The autosomal dominant pattern of inheritance of this skeletal dysplasia is reinforced, as there are many other reportedly affected relatives, including the maternal grandfather, uncles, and aunts of the propositus. The findings of wormian bones and calcification of the falx, not previously described, may be added to the phenotype.

[The curative surgery of periampullary tumors. The results of 48 resections]. / Cirurgia de intenção curativa nos tumores peri-ampulares. Resultados de 48 ressecções consecutivas.

Periampullary tumors form a clinical entity with common symptoms, similar therapeutic options, unsatisfactory resectability rates and unfavorable prognosis. From April 1970 until March 1994, one hundred and twenty-seven patients with periampullary carcinoma were operated by our surgical team. In 48 of these patients, a resection for cure was performed (38%). Resectability rates varied according to the origin of these tumors, i.e., pancreas-20%, ampulla-76%; distal bile duct-71%, periampullary duodenum-88%. Pancreatic tumors showed a different resectability rate from the other periampullary carcinomas (p = 0.04). Forty-two of these patients had a pancreatoduodenectomy and in the remaining 6 cases a total pancreatectomy was performed. Fifteen patients had major post-operative morbidity (31%) and 8 of these cases died in-hospital (17%). Follow-up data was available in 81% of the patients, survival estimates were calculated according to the Kaplan-Meier method and survival comparisons were made with the Log-rank test. Median survival for resected pancreatic carcinoma was 6 months and for resected tumors of the ampulla 37 months. In this group of patients, pancreatic tumors showed a different survival rate from the remaining periampullary tumors (Log-rank-p = 0.002). This work evidences the need to improve management of periampullary tumors, particularly in-hospital mortality and long-term survival. To achieve these goals, patients with periampullary tumors should be treated in specialized centers and research to improve local and systemic control of this disease should be pursued.