Association of expression of inflammatory response genes and DNA repair genes in colorectal carcinoma.

Inflammation is an important etiological factor of colorectal carcinoma and may be related to colorectal carcinoma growth and proliferation. This study aimed to verify whether the presence of chronic inflammation represented by tumor necrosis factor-α, interleukin-2, interleukin-6, and interleukin-10 gene expression is related to hMLH1, hMSH2, hMSH6, and PMS2 gene expression and the corresponding protein levels of these genes from the DNA repair system. A total of 83 patients were operated on for curative or palliative colorectal carcinoma. Expression of the inflammatory response genes tumor necrosis factor-α, interleukin-2, interleukin-6, and interleukin-10 as well as expression of the hMLH1, hMSH2, hMSH6, and PMS2 genes of the DNA repair system (mismatch repair) and the expression levels of the corresponding mismatch repair proteins were measured in neoplastic tissue by reverse transcription polymerase chain reaction and immunohistochemistry, respectively. Associations were observed between hMSH6 mRNA expression and interleukin-2 mRNA expression (p = 0.026) as well as between hMLH1 and hMSH2 gene expression and tumor necrosis factor-α gene expression (p = 0.042). Higher tissue levels of interleukin-2 and tumor necrosis factor-α gene expression were associated with lower hMSH6, hMLH1, and hMSH2 gene expression.

Extensive colorectal lymphomatous polyposis complicated by acute intestinal obstruction: a case report.

BACKGROUND: Multiple lymphomatous polyposis is a rare type of gastrointestinal lymphoma that extensively infiltrates the intestine. Multiple lymphomatous polyposis originates from the mantle zone of the lymphoma follicle and is considered to be a mantle cell lymphoma, which is a relatively aggressive type of B-cell non-Hodgkin's lymphoma. We report an unusual case of a patient with multiple lymphomatous polyposis with extensive colorectal involvement and acute intestinal obstruction, an atypical complication of this rare disease. On the basis of this case study, the pitfalls in gastrointestinal tract lymphomatous polyposis diagnosis and prognosis, as well as the treatment options, are discussed. CASE PRESENTATION: Our patient was a 76-year-old white woman with asthenia, cramps, and swelling in the lower left quadrant of the abdomen, as well as weight loss within the previous 5 months. A colonoscopy revealed polyps in the rectum, sigmoid colon, descending colon, and right and left colic flexures. A biopsy revealed lymphomatous infiltration of the intestinal wall. Because of the large size of the polypoid masses, which narrowed the colonic lumen in multiple locations, the patient developed acute intestinal obstruction and was referred for laparotomy. She underwent a total proctocolectomy with a permanent ileostomy and a left salpingo-oophorectomy. Microscopic examination showed the presence of a multicentric, low-grade, small lymphocytic lymphoma. Immunohistochemical analysis revealed positive immunostaining for CD79a, CD20, and CD45. These results were consistent with the diagnosis of mantle cell lymphoma. Two weeks after surgery and prior to discharge, but before the beginning of chemotherapy, the patient's general condition worsened as she experienced a severe and progressive respiratory tract infection, advanced respiratory insufficiency, and septic shock, and she ultimately died. CONCLUSIONS: Mantle cell lymphoma develops as a progressive and aggressive disease with widespread polyposis of the gastrointestinal tract. The intensive chemotherapeutic regimens usually result in the regression of macroscopic and microscopic lesions; however, remissions are short in duration, and the median length of patient survival is 3-4 years. Mantle cell lymphoma is a rare disease that should be part of the differential diagnosis of polypoid diseases of the large intestine.

Detection of DNA repair protein in colorectal cancer of patients up to 50 years old can increase the identification of Lynch syndrome?

The aim of this study was to compare the results of protein level of the DNA mismatch repair genes with the clinical diagnosis of Lynch syndrome according to the Amsterdam II criteria in patients 50 years and younger who underwent surgery for colorectal cancer. The subjects of analysis were 48 patients 50 years old and younger. Immunohistochemistry assays were performed to detect proteins from the DNA mismatch repair genes. Clinicopathological data and Amsterdam II criteria for the diagnosis of hereditary nonpolyposis colorectal cancer were obtained by analyzing medical records. Two (4 %) patients satisfied the Amsterdam II criteria for Lynch syndrome, and both presented levels of all of the studied mismatch repair proteins. A total of 13 (27 %) patients exhibited the absence of protein levels of the studied mismatch repair genes. None of these patients were considered suspicious for Lynch syndrome according to the Amsterdam II criteria. Screening for the level of proteins of the mismatch repair system in all colorectal cancer patients 50 years and younger can increase the identification of patients with suspicion of Lynch syndrome.

Carcinoid tumor of the duodenum: a rare tumor at an unusual site. Case series from a single institution.

CONTEXT: Duodenal carcinoids are extremely rare, and their characteristics and biological behavior have not been fully elucidated. OBJECTIVE: To analyze the clinicopathological characteristics of patients with resected duodenal carcinoids. METHODS: Twenty patients (12 females and 8 males) were investigated. Their average age was 66.4 ± 5.8 years old (43 to 88 years old). The data corresponding to the clinical picture, diagnosis, treatment, and prognosis of patients with duodenal carcinoid tumors subjected to resection over a period of 18 years (1993-2011) were analyzed. RESULTS: The most common symptoms were dyspepsia (50%) and epigastric pain (45%) followed by weight loss (10%) and vomiting (5%). Carcinoid syndrome was not observed in any patient. The lesion was located on the first part of the duodenum in 15 (75%) patients, the second part in 4 (20%) patients, and the third part in 1 (5%) patient. The diagnosis of a carcinoid tumor was established through an endoscopic excision biopsy in 19 (95%) patients and an histopathological examination of the surgical specimen in 1 (5%) patient. The average tumor size was 1.1 cm ± 0.4 cm (0.3 cm to 6.0 cm). Nineteen (95%) patients were initially treated by endoscopic resection of the duodenal lesion. One patient (5%), whose tumor was on the third part of the duodenum underwent a duodenectomy of the third and fourth duodenal parts and duodenojejunal anastomosis. The duodenal carcinoid resection margin was involved in four (20%) patients. Four (20%) patients were subjected to a partial gastrectomy to fully remove the lesion. The tumor was restricted to the submucosal layer in 16 (80%) cases, and it penetrated into the muscular layer in 4 (20%) cases. All patients exhibited positive chromogranin A, neuron-specific enolase, and/or synaptophysin immunostaining. The average duration of the follow-up period was 39.6 months (3 to 96 months). Twelve (60%) of the 20 cases in this series are alive without any evidence of active disease. Only one (5%) patient died due to liver metastases of the duodenal carcinoid. CONCLUSIONS: Duodenal carcinoids are rare and indolent tumors usually associated with a benign progression. Duodenoscopy, computerized tomography, and endoscopic ultrasound should be performed to evaluate the tumor size, the level of wall invasion, and the presence of regional or distant lymphatic metastases. Endoscopic removal of tumors smaller than 1.0 cm without periampullary localization or evidence of muscular propria layer invasion assessed by histology and/or endoscopic ultrasound is recommended. The endoscopic resection with a carcinoid tumor size between 1.0 cm and 2.0 cm can be incomplete and require new endoscopic resection or even surgical removal. Duodenal carcinoid larger than 2.0 cm require full-thickness resection and concomitant lymphadenectomy.

CARCINOID TUMOR OF THE DUODENUM: a rare tumor at an unusual site. Case series from a single institution

Context Duodenal carcinoids are extremely rare, and their characteristics and biological behavior have not been fully elucidated. Objective To analyze the clinicopathological characteristics of patients with resected duodenal carcinoids. Methods Twenty patients (12 females and 8 males) were investigated. Their average age was 66.4 ± 5.8 years old (43 to 88 years old). The data corresponding to the clinical picture, diagnosis, treatment, and prognosis of patients with duodenal carcinoid tumors subjected to resection over a period of 18 years (1993-2011) were analyzed. Results The most common symptoms were dyspepsia (50%) and epigastric pain (45%) followed by weight loss (10%) and vomiting (5%). Carcinoid syndrome was not observed in any patient. The lesion was located on the first part of the duodenum in 15 (75%) patients, the second part in 4 (20%) patients, and the third part in 1 (5%) patient. The diagnosis of a carcinoid tumor was established through an endoscopic excision biopsy in 19 (95%) patients and an histopathological examination of the surgical specimen in 1 (5%) patient. The average tumor size was 1.1 cm ± 0.4 cm (0.3 cm to 6.0 cm). Nineteen (95%) patients were initially treated by endoscopic resection of the duodenal lesion. One patient (5%), whose tumor was on the third part of the duodenum underwent a duodenectomy of the third and fourth duodenal parts and duodenojejunal anastomosis. The duodenal carcinoid resection margin was involved in four (20%) patients. Four (20%) patients were subjected to a partial gastrectomy to fully remove the lesion. The tumor was restricted to the submucosal layer in 16 (80%) cases, and it penetrated into the muscular layer in 4 (20%) cases. All patients exhibited positive chromogranin A, neuron-specific enolase, and/or synaptophysin immunostaining. The average duration of the follow-up ...

Contexto Carcinoides duodenais são extremamente raros e as características e o comportamento biológico dessa neoplasia permanecem indefinidos. Objetivo Analisar as características clinicopatológicas de doentes com carcinoide duodenal ressecado. Métodos Vinte doentes (12 mulheres e 8 homens) foram estudados. A média de idade dos doentes foi de 66,4 ± 5,8 anos (43 a 88 anos). Os dados do quadro clínico, diagnóstico, tratamento e prognóstico dos doentes com tumor carcinoide do duodeno submetidos a ressecção da lesão no período de 18 anos (1993-2011) foram analisados. Resultados Os sintomas mais frequentes foram dispepsia (50%) e epigastralgia (45%), seguidos por perda de peso (10%) e vômitos (5%). Não foram observados doentes com síndrome carcinoide. A lesão estava localizada na primeira porção do duodeno em 15 (75%) pacientes, na segunda porção em 4 (20%) e na terceira porção em 1 (5%). O diagnóstico de tumor carcinoide foi estabelecido pela biopsia endoscópica excisional em 19 (95%) pacientes e pelo exame histopatológico da peça cirúrgica em um (5%). O tamanho médio dos tumores foi de 1,1 cm ± 0,4 cm (0,3 cm a 6,0 cm). Dezenove (95%) doentes foram tratados, inicialmente, por ressecção endoscópica da lesão duodenal e um (5%) com lesão na terceira porção duodenal foi submetido a duodenectomia da terceira e quarta porções do duodeno e duodenojejunoanastomose. A margem de ressecção do carcinoide duodenal estava comprometida em quatro (20%) casos e em quatro (20%) pacientes foi realizada gastrectomia parcial para retirada completa da lesão. O tumor estava limitado à camada submucosa em 16 (80%) casos e penetrava a camada muscular ...

Carcinoid tumor of the duodenum

ContextDuodenal carcinoids are extremely rare, and their characteristics and biological behavior have not been fully elucidated.ObjectiveTo analyze the clinicopathological characteristics of patients with resected duodenal carcinoids.MethodsTwenty patients (12 females and 8 males) were investigated. Their average age was 66.4 ± 5.8 years old (43 to 88 years old). The data corresponding to the clinical picture, diagnosis, treatment, and prognosis of patients with duodenal carcinoid tumors subjected to resection over a period of 18 years (1993-2011) were analyzed.ResultsThe most common symptoms were dyspepsia (50%) and epigastric pain (45%) followed by weight loss (10%) and vomiting (5%). Carcinoid syndrome was not observed in any patient. The lesion was located on the first part of the duodenum in 15 (75%) patients, the second part in 4 (20%) patients, and the third part in 1 (5%) patient. The diagnosis of a carcinoid tumor was established through an endoscopic excision biopsy in 19 (95%) patients and an histopathological examination of the surgical specimen in 1 (5%) patient. The average tumor size was 1.1 cm ± 0.4 cm (0.3 cm to 6.0 cm). Nineteen (95%) patients were initially treated by endoscopic resection of the duodenal lesion. One patient (5%), whose tumor was on the third part of the duodenum underwent a duodenectomy of the third and fourth duodenal parts and duodenojejunal anastomosis. The duodenal carcinoid resection margin was involved in four (20%) patients. Four (20%) patients were subjected to a partial gastrectomy to fully remove the lesion. The tumor was restricted to the submucosal layer in 16 (80%) cases, and it penetrated into the muscular layer in 4 (20%) cases. All patients exhibited positive chromogranin A, neuron-specific enolase, and/or synaptophysin immunostaining. The average duration of the follow-up period was 39.6 months (3 to 96 months). Twelve (60%) of the 20 cases in this series are alive without any evidence of active disease. Only one (5%) patient died due to liver metastases of the duodenal carcinoid.ConclusionsDuodenal carcinoids are rare and indolent tumors usually associated with a benign progression. Duodenoscopy, computerized tomography, and endoscopic ultrasound should be performed to evaluate the tumor size, the level of wall invasion, and the presence of regional or distant lymphatic metastases. Endoscopic removal of tumors smaller than 1.0 cm without periampullary localization or evidence of muscular propria layer invasion assessed by histology and/or endoscopic ultrasound is recommended. The endoscopic resection with a carcinoid tumor size between 1.0 cm and 2.0 cm can be incomplete and require new endoscopic resection or even surgical removal. Duodenal carcinoid larger than 2.0 cm require full-thickness resection and concomitant lymphadenectomy.

ContextoCarcinoides duodenais são extremamente raros e as características e o comportamento biológico dessa neoplasia permanecem indefinidos.ObjetivoAnalisar as características clinicopatológicas de doentes com carcinoide duodenal ressecado.MétodosVinte doentes (12 mulheres e 8 homens) foram estudados. A média de idade dos doentes foi de 66,4 ± 5,8 anos (43 a 88 anos). Os dados do quadro clínico, diagnóstico, tratamento e prognóstico dos doentes com tumor carcinoide do duodeno submetidos a ressecção da lesão no período de 18 anos (1993-2011) foram analisados.ResultadosOs sintomas mais frequentes foram dispepsia (50%) e epigastralgia (45%), seguidos por perda de peso (10%) e vômitos (5%). Não foram observados doentes com síndrome carcinoide. A lesão estava localizada na primeira porção do duodeno em 15 (75%) pacientes, na segunda porção em 4 (20%) e na terceira porção em 1 (5%). O diagnóstico de tumor carcinoide foi estabelecido pela biopsia endoscópica excisional em 19 (95%) pacientes e pelo exame histopatológico da peça cirúrgica em um (5%). O tamanho médio dos tumores foi de 1,1 cm ± 0,4 cm (0,3 cm a 6,0 cm). Dezenove (95%) doentes foram tratados, inicialmente, por ressecção endoscópica da lesão duodenal e um (5%) com lesão na terceira porção duodenal foi submetido a duodenectomia da terceira e quarta porções do duodeno e duodenojejunoanastomose. A margem de ressecção do carcinoide duodenal estava comprometida em quatro (20%) casos e em quatro (20%) pacientes foi realizada gastrectomia parcial para retirada completa da lesão. O tumor estava limitado à camada submucosa em 16 (80%) casos e penetrava a camada muscular própria em 4 (20%). Todos os pacientes apresentaram imunomarcação positiva para cromogranina A, enolase neurônio-específica ou sinaptofisina. A média do período de seguimento foi de 39,6 meses (3 a 96 meses). Dos 20 casos desta série, 12 (60%) permanecem vivos e sem evidência de doença ativa e apenas 1 (5,0%) faleceu por metástase hepática do carcinoide duodenal.ConclusõesCarcinoides duodenais são tumores raros e indolentes normalmente associados a bom prognóstico. Duodenoscopia, tomografia computadorizada e ultrassonografia endoscópica devem ser realizadas para avaliar o tamanho do tumor, o nível de invasão da parede e a presença de metástases linfáticas regionais e/ou distantes. Remoção endoscópica de tumores menores que 1,0 cm, sem localização periampolar ou evidência de invasão da camada muscular própria avaliada pela histologia e/ou ultrassonografia endoscópica é recomendada. A ressecção endoscópica de tumor carcinoide com tamanho entre 1,0 cm e 2,0 cm pode ser incompleta e requerer nova ressecção endoscópica ou mesmo remoção cirúrgica. Carcinoides duodenais maiores que 2,0 cm necessitam de ressecção com espessura total e linfadenectomia concomitante.

Gallbladder adenocarcinoma: evaluation of the prognostic factors in 100 resectable cases in Brazil.

BACKGROUND: In spite its relative rarity, gallbladder adenocarcinoma is a neoplasm who presents an aggressive biologic behavior. The single curative treatment has been radical surgical resection with free margin. Prognostic factors has been studied because are very important to identify long-term survival patients which may benefit of aggressive surgical resection. AIM: To evaluate long-term prognostic predictors from gallbladder cancer. METHODS: The medical records of all patients that presented confirmed histological diagnosis of gallbladder adenocarcinoma operated over a 14 year period were identified and retrospectively reviewed. Uni and multivariate analysis was done. RESULTS: Total sample was 100 patients. Median age was 71 years (34 to 93). There were 17 men and 83 women. Lesion distribution according to TNM stage system was: I (n=22), II (n=59), III (n=6), IV (n=4) and unknown (n=9). Fifty two patients underwent radical resection (R0) while 48 to palliative surgery (R1-R2). Overall major morbidity was 14%, while postoperative surgical mortality rate (30th postoperative day) was 12 %. Five-year survival rate was 28% while median of survival was 10 months. Multivariate analysis identified six prognostic factors: T stage, serum level of CA 19.9, gallbladder perforation, lymphatic embolization, surgical historical cohort (after 2002) and hilar lymphadenectomy. CONCLUSION: Prognostic factors were: T stage, serum level of CA 19.9, gallbladder perforation, lymphatic embolization, surgical historical cohort and hilar lymphadenectomy.

Adenocarcinoma da vesícula biliar: avaliação dos fatores prognósticos em 100 casos ressecados no Brasil / Gallbladder adenocarcinoma: evaluation of the prognostic factors in 100 resectable cases in Brazil

RACIONAL: A despeito da sua relativa raridade, o adenocarcinoma de vesícula biliar é neoplasia que apresenta comportamento biológico agressivo. O único tratamento curativo tem sido a ressecção cirúrgica radical com margem livre. Fatores prognósticos têm sido estudados por serem importantes para identificar pacientes que podem se beneficiar de ressecção cirúrgica agressiva. OBJETIVO: Avaliar preditores prognósticos em longo prazo de pacientes com câncer da vesícula biliar. MÉTODOS: Foram identificados e retrospectivamente revisados os prontuários médicos de todos os doentes submetidos a tratamento cirúrgico que apresentavam diagnóstico histológico confirmado de adenocarcinoma de vesícula biliar durante período de 14 anos. Os dados foram submetidos à análise estatística uni e multivariada. RESULTADOS: A amostra total foi de 100 doentes. A mediana de idade foi de 71 anos (34 a 93). Houve 17 mulheres e 83 homens. A distribuição das lesões de acordo com o sistema de estadiamento TNM foi: I (n=22), II (n=59), III (n=6), IV (n=4) e desconhecido (n=9). Cinquenta e dois doentes foram submetidos à ressecção radical (R0) enquanto 48 à cirurgia paliativa (R1-R2). A morbidade global foi de 14% enquanto que a mortalidade pós-operatória (até 30º dia do pós-operatório) foi de 12 %. A taxa de sobrevida em cinco anos foi de 28% enquanto a mediana de sobrevida foi de 10 meses. A análise multivariada identificou seis fatores prognósticos: estádio T, nível sérico de CA 19.9, perfuração da vesícula biliar, embolização linfática, coorte cirúrgico histórico e linfadenectomia hilar. CONCLUSÃO: O tratamento do câncer de vesícula biliar apresenta alta morbimortalidade. Os fatores prognósticos foram: estádio T, nível sérico de CA 19.9, perfuração da vesícula biliar, embolização linfática, coorte cirúrgico histórico e linfadenectomia hilar.

BACKGROUND: In spite its relative rarity, gallbladder adenocarcinoma is a neoplasm who presents an aggressive biologic behavior. The single curative treatment has been radical surgical resection with free margin. Prognostic factors has been studied because are very important to identify long-term survival patients which may benefit of aggressive surgical resection. AIM: To evaluate long-term prognostic predictors from gallbladder cancer. METHODS: The medical records of all patients that presented confirmed histological diagnosis of gallbladder adenocarcinoma operated over a 14 year period were identified and retrospectively reviewed. Uni and multivariate analysis was done. RESULTS: Total sample was 100 patients. Median age was 71 years (34 to 93). There were 17 men and 83 women. Lesion distribution according to TNM stage system was: I (n=22), II (n=59), III (n=6), IV (n=4) and unknown (n=9). Fifty two patients underwent radical resection (R0) while 48 to palliative surgery (R1-R2). Overall major morbidity was 14%, while postoperative surgical mortality rate (30th postoperative day) was 12 %. Five-year survival rate was 28% while median of survival was 10 months. Multivariate analysis identified six prognostic factors: T stage, serum level of CA 19.9, gallbladder perforation, lymphatic embolization, surgical historical cohort (after 2002) and hilar lymphadenectomy. CONCLUSION: Prognostic factors were: T stage, serum level of CA 19.9, gallbladder perforation, lymphatic embolization, surgical historical cohort and hilar lymphadenectomy.

Metástase esplênica isolada de adenocarcinoma do sigmoide: relato de caso / Isolated splenic metastasis from sigmoid colon adenocarcinoma: case report

INTRODUÇÃO: Metástases esplênicas solitárias oriundas de carcinomas colorretais são raras, com 41 casos descritos na literatura inglesa até 2007. Muitos pacientes são assintomáticos, e o diagnóstico é quase sempre feito por imagens radiológicas ou por elevações sanguíneas do antígeno carcinoembrionário (CEA), solicitados no seguimento pós-operatório desses pacientes. Relato do caso: Homem de 54 anos foi submetido à colectomia esquerda por carcinoma de sigmoide. O tumor foi estadiado como T3N0M0 e permaneceu assintomático por dez meses, com níveis normais de CEA. Notou-se, então, significativa elevação do antígeno e a tomografia computadorizada do abdômen revelou massa no polo inferior do baço, suspeita de metástase. A laparotomia confirmou o achado propedêutico, sendo realizada esplenectomia. O diagnóstico anatomopatológico foi de adenocarcinoma metastático, sem invasão dos linfonodos. A sobrevida acompanhada do paciente foi de 14 meses, livre de recidiva. CONCLUSÃO: Metástases esplênicas isoladas de carcinoma dos cólons são raras, e a esplenectomia oferece possibilidade de sobrevida expressiva.

Solitary metastatic metastasis from colorectal neoplasms is rare. Only 41 cases have been reported in the English literature until 2007. Most patients are asymptomatic, and the diagnosis is usually done by imaging studies or CEA (carcinoembrionic antigen) blood increases, which are required in the postoperative follow-up period. Case report: A 54-year-old man underwent an extended left colectomy for sigmoid colon cancer. The tumor was staged as T3N0M0. During ten months of the follow-up period, the patient remained asymptomatic with normal levels of laboratory tests, including CEA measurement. Then, there was a significant elevation of CEA, and the abdomen computed tomography revealed a mass in the spleen considered as an isolated metastasis. The patient underwent splenectomy. Histological diagnosis confirmed a metastatic adenocarcinoma with no lymph nodes invasion. The patient has been symptom-free during the 14 months of follow-up with normal blood CEA levels and negative radiological studies. CONCLUSION: Solitary spleen metastasis from colon carcinoma is rare, and splenectomy provides an expressive improvement in the survival.

Heterotopia pancreática: análise clínico-patológica de 18 doentes / Pancreatic heterotopias: clinicopathological analysis of 18 patients

OBJETIVO: Analisar as características clínico-patológicas do tecido pancreático heterotópico em órgãos digestivos abdominais. MÉTODOS: Realizamos estudo retrospectivo analisando 18 portadores de pâncreas heterotópico diagnosticados histologicamente. Seus dados clínicos e histopatológicos foram revistos. O tecido pancreático heterotópico foi classificado em três modelos histológicos: tipo I constituído por três componentes do pâncreas normal (ácinos, ductos e ilhotas), tipo II com dois componentes e tipo três com somente um componente. RESULTADOS: A média de idade foi de 52,7 anos, variando de 34 a 73 anos, com nove homens e nove mulheres. Sintomas foram observados em somente quatro doentes, sendo suas lesôes diagnosticadas por gastroscopia. Os 14 restantes eram assintomáticos e suas anomalias descobertas acidentalmente. A maioria das lesões situava-se no trato superior: sete (38,9%) no estômago, seis (33,3%) no duodeno e três (16,6%) no jejuno. A heterotopia localizou-se preferencialmente na submucosa (83,3%), mas também foi observada na muscular própria e na sub-serosa. Em sete (38,9%) espécimes todos os componentes pancreáticos foram constatados (tipo I), em oito (44,4%) estavam presentes glândulas exócrinas e ductos excretores (tipo II) e em três (16,7%) somente o tecido exócrino foi observado (tipo III). CONCLUSÃO: A heterotopia pancreática é rara. Doentes com ectopia pancreática diagnosticadas pelo estudo patológico, assintomáticos ou com sintomas discretos devem permanecer em observação. As lesões detectadas acidentalmente durante procedimentos cirúrgicos necessitam ser removidas por procedimentos conservadores.

OBJECTIVE: To analyze the clinical and pathological features of heterotopic pancreatic tissue in abdominal digestive organs. METHODS: We retrospectively studied 18 patients with histologically diagnosed heterotopic pancreas. Clinical and histopathologic data were reviewed. Heterotopic pancreatic tissues were classified in three histological models: Type I consists of three components of normal pancreas (acini, ducts and islets), type II with two components and type three with only one component. RESULTS: The mean age was 52.7 years, ranging from 34 to 73 years, nine of them men and nine women. Symptoms were observed in only four patients, and their lesions were diagnosed by gastroscopy. The remaining 14 were asymptomatic and their anomalies were discovered accidentally. Most of the lesions were located in the upper gastrointestinal tract: seven (38.9%) in the stomach, six (33.3%) in the duodenum and three (16.6%) in the jejunum. Heterotopia was mostly located in the submucosa (83.3%) but was also observed in the muscularis propria and in the sub-serosa. In seven specimens (38.9%) all pancreatic components were found (type I), in eight (44.4%) exocrine glands and excretory ducts were present (type II) and in three (16.7%) only exocrine tissue was observed (type III). CONCLUSION: Pancreatic heterotopia is rare. Patients with pancreatic ectopia diagnosed by pathological study, whether asymptomatic or with mild symptoms, should be observed. Lesions incidentally detected during surgeries need to be removed by conservative procedures.

Pancreatic heterotopias: clinicopathological analysis of 18 patients.

OBJECTIVE: To analyze the clinical and pathological features of heterotopic pancreatic tissue in abdominal digestive organs. METHODS: We retrospectively studied 18 patients with histologically diagnosed heterotopic pancreas. Clinical and histopathologic data were reviewed. Heterotopic pancreatic tissues were classified in three histological models: Type I consists of three components of normal pancreas (acini, ducts and islets), type II with two components and type three with only one component. RESULTS: The mean age was 52.7 years, ranging from 34 to 73 years, nine of them men and nine women. Symptoms were observed in only four patients, and their lesions were diagnosed by gastroscopy. The remaining 14 were asymptomatic and their anomalies were discovered accidentally. Most of the lesions were located in the upper gastrointestinal tract: seven (38.9%) in the stomach, six (33.3%) in the duodenum and three (16.6%) in the jejunum. Heterotopia was mostly located in the submucosa (83.3%) but was also observed in the muscularis propria and in the sub-serosa. In seven specimens (38.9%) all pancreatic components were found (type I), in eight (44.4%) exocrine glands and excretory ducts were present (type II) and in three (16.7%) only exocrine tissue was observed (type III). CONCLUSION: Pancreatic heterotopia is rare. Patients with pancreatic ectopia diagnosed by pathological study, whether asymptomatic or with mild symptoms, should be observed. Lesions incidentally detected during surgeries need to be removed by conservative procedures.

Colonic carcinoid tumors: a clinicopathologic study of 23 patients from a single institution / Tumores carcinóides do cólon: estudo clinicopatológico de 23 doentes de uma única instituição

Context: Colonic carcinoids, excluding those arising in the appendix, have proved to be extremely rare. Due to their rarity, the characteristics and behavior of this unusual malignancy remain unclear. Objective: To review the clinicopathologic features of patients operated on carcinoid tumors of the colon. Methods: Twenty-three patients (12 males and 11 females) were operated on colonic carcinoids. The mean age of the patients was 63.0 ± 12.9 years (42 to 85 years). The clinical and histopathological data of patients who were pathologically diagnosed as having carcinoid tumors and submitted to surgical treatment over a 30-year period (1977-2007) were gathered. Actuarial patient survival was estimated using the Kaplan-Meier method, with carcinoid-specific death as the outcome. Results: The mean time elapsed between onset of symptoms and surgical treatment was 8.3 months (1.5 to 20 months). The most frequent symptoms or signs encountered were abdominal pain followed by anorexia or weight loss, diarrhea, abdominal tenderness, palpable abdominal mass, and rectal bleeding. No carcinoid syndrome was noted. The lesion was located in the cecum in 16 (69.6 percent) patients, in the sigmoid in 3 patients (13.0 percent), in the ascending colon in 3 patients (13.0 percent), and in the transverse colon in one patient (4.3 percent). Twenty-one (91.3 percent) patients were operated on curative intent. Spreading of the disease to the liver and peritoneum was found in two (8.7 percent) patients who submitted to intestinal bypass. The mean size of the largest mass was 3.7 ± 1.2 cm (1.5 to 6.2 cm). There were multiple (two or more) lesions in three cases (13.0 percent). In the resected cases, the lymph nodes were compromised in 10 patients (47.6 percent) and disease-free in 11 (52.4 percent). Venous invasion and neural infiltration were both present in five (23.8 percent) patients. The tumors had penetrated the muscularis propria in all resected cases...

Contexto: Carcinóides cólicos, excluindo aqueles que se originam no apêndice cecal, são extremamente raros. Devido a esta raridade, as características e comportamento desta neoplasia permanecem indefinidas. Objetivo: Rever as características clinicopatológicas de doentes operados por carcinoma cólico. Método: Vinte e três doentes (12 homens e 11 mulheres) foram operados. A média de idade dos doentes foi de 63,0 ± 12,9 anos (42 a 85 anos). Os dados clínicos e histopatológicos dos doentes com diagnóstico patológico de tumor carcinóide e submetidos ao tratamento cirúrgico no período de 30 anos (1977-2007) foram obtidos a partir dos prontuários. A sobrevivência atuarial dos doentes foi estimada pelo método de Kaplan-Meier, considerando-se como óbito específico aquele devido ao tumor carcinóide. Resultados - A média de tempo decorrida entre o início dos sintomas e o tratamento cirúrgico foi de 8,3 meses (1,5 a 20 meses). O sintoma mais frequente encontrado foi dor abdominal seguida por anorexia ou perda de peso, diarréia, massa abdominal palpável e sangramento pelo reto. Não foram observados doentes com síndrome carcinóide. A lesão estava localizada no ceco em 16 (69,6 por cento) doentes, no cólon sigmóide em 3 (13,0 por cento), no colo ascendente em 3 (13,0 por cento) e no cólon transverso em 1 (4,3 por cento). Vinte e um (91,3 por cento) doentes foram operados com intenção curativa. Disseminação da doença para o fígado e peritônio foi encontrada em dois (8,7 por cento) doentes que foram submetidos ao desvio intestinal. A média do tamanho das lesões intestinais foi 3,7 ± 1,2 cm (1,5 a 6,2 cm). Lesões múltiplas (duas ou mais) ocorreram em três (13,0 por cento) casos. Nas lesões ressecadas, os linfonodos estavam comprometidos em 10 (47,6 por cento) e livres em 11 (52,4 por cento). Invasão venosa e infiltração neural estavam ambas presentes em cinco (23,8 por cento) doentes. A neoplasia penetrava a muscularis propria em todos as lesões ressecadas...

Colonic carcinoid tumors: a clinicopathologic study of 23 patients from a single institution.

CONTEXT: Colonic carcinoids, excluding those arising in the appendix, have proved to be extremely rare. Due to their rarity, the characteristics and behavior of this unusual malignancy remain unclear. OBJECTIVE: To review the clinicopathologic features of patients operated on carcinoid tumors of the colon. METHODS: Twenty-three patients (12 males and 11 females) were operated on colonic carcinoids. The mean age of the patients was 63.0 +/- 12.9 years (42 to 85 years). The clinical and histopathological data of patients who were pathologically diagnosed as having carcinoid tumors and submitted to surgical treatment over a 30-year period (1977-2007) were gathered. Actuarial patient survival was estimated using the Kaplan-Meier method, with carcinoid-specific death as the outcome. RESULTS: The mean time elapsed between onset of symptoms and surgical treatment was 8.3 months (1.5 to 20 months). The most frequent symptoms or signs encountered were abdominal pain followed by anorexia or weight loss, diarrhea, abdominal tenderness, palpable abdominal mass, and rectal bleeding. No carcinoid syndrome was noted. The lesion was located in the cecum in 16 (69.6%) patients, in the sigmoid in 3 patients (13.0%), in the ascending colon in 3 patients (13.0%), and in the transverse colon in one patient (4.3%). Twenty-one (91.3%) patients were operated on curative intent. Spreading of the disease to the liver and peritoneum was found in two (8.7%) patients who submitted to intestinal bypass. The mean size of the largest mass was 3.7 +/- 1.2 cm (1.5 to 6.2 cm). There were multiple (two or more) lesions in three cases (13.0%). In the resected cases, the lymph nodes were compromised in 10 patients (47.6%) and disease-free in 11 (52.4%). Venous invasion and neural infiltration were both present in five (23.8%) patients. The tumors had penetrated the muscularis propria in all resected cases. Four (17.4%) patients had a second non-carcinoid primary tumor. Three (13.0%) patients died due to postoperative complications and five (21.7%) patients died from metachronous metastases or local recurrence. Fifteen patients (65.2%) remain alive without evidence of active disease. The mean follow-up period was 12 years (1.2 to 18 years), whereas the mean global survival was 50.7 +/- 34.2 months and the crude survival rate at 5 years was 62.7%. CONCLUSIONS: Carcinoid tumors of the colon are frequently right-sided and may be clinically occult until an advanced stage is reached. Based on the relatively poor survival rates reported, it is recommended that, in addition to standard surgical resection, vigorous surveillance for metastatic disease must be performed, particularly during the first 2 years after surgery. In addition, these patients require evaluation of the entire gastrointestinal tract for evidence of coexisting malignancy, along with an extended period of follow-up, because tumor recurrences after 5 years are not uncommon.

Pancreatic heterotopia in the gall bladder: a case report and literature review / Heterotopia pancreática na vesícula biliar: relato de caso e revisão da literatura

Pancreatic heterotopia in the gall bladder is a rare entity, with approximately 31 cases reported in English language literature until 2006; there are no Brazilian studies on the topic. As it is usually asymptomatic, the definitive diagnosis is made by the anatomopathological material excised due to common diseases of the organ, such as lithiasis, inflammatory processes, polyps and tumors. A case is reported of a 40-year-old female patient submitted to surgery with a diagnosis of lithiasic cholecystopathy. Ectopy was evidenced by the histological study, due to the presence of acinar and ductal components of the pancreas and gall bladder corpus. The authors used their own pathological classification for this ectopy, and recommended that this entity be included more frequently in the differential diagnosis of alithiasic cholecystopathy and intramural and exophytic lesions of the gall bladder.

A heterotopia pancreática na vesícula biliar é entidade rara, com cerca de 31 casos relatados na literatura inglesa até 2006, inexistindo trabalhos nacionais a respeito. Quase sempre assintomática, seu diagnóstico definitivo é realizado pelo estudo anatomopatológico do material extirpado em virtude de doenças comuns do órgão, como a litíase, processos inflamatórios, pólipos e tumores. Relata-se o caso de uma paciente de 40 anos, operada com diagnóstico de colecistopatia litiásica. A ectopia foi evidenciada pelo estudo histológico, devido à presença dos componentes acinar e ductal do pâncreas no corpo vesicular. Os autores utilizaram a classificação patológica própria para essa ectopia e recomendam a inclusão mais frequente desta entidade no diagnóstico diferencial das colecistopatias alitiásicas e das lesões intramurais e exofíticas da vesícula biliar.

Intestinal metaplasia in gallbladders: prevalence study.

CONTEXT AND OBJECTIVE: Gallbladder cancer is usually diagnosed at a late stage and generally results in death. Discovery of predisposing factors for this neoplasia could prevent this outcome. In this study, we assess the presence of one of these factors: intestinal metaplasia in gallbladders with stones and inflammatory processes. DESIGN AND SETTING: Cross-sectional study in Hospital do Servidor Público Estadual de São Paulo. METHOD: The first 80 gallbladders from patients who underwent elective cholecystectomy between April and August 2002, presenting stones and chronic inflammation, were studied. The patients were divided into groups according to their age: CC1, from 15 to 40 years; CC2, from 41 to 60 years; and CC3, from 61 to 85 years. RESULTS: Twenty-one patients (26%) were male, while 59 (74%) were female. In the group CC1, intestinal metaplasia was present in 85.71% of the 21 patients studied; in CC2, in 79.41% of 34 patients; and in CC3, in 56.00% of 25 patients. These differences presented statistical significance (p = 0.04542). CONCLUSION: Intestinal metaplasia is extremely frequent in gallbladders with inflammation and lithiasis, especially in younger patients.

Intestinal metaplasia in gallbladders: prevalence study / Metaplasia intestinal na vesícula biliar: estudo da prevalência

CONTEXT AND OBJECTIVE: Gallbladder cancer is usually diagnosed at a late stage and generally results in death. Discovery of predisposing factors for this neoplasia could prevent this outcome. In this study, we assess the presence of one of these factors: intestinal metaplasia in gallbladders with stones and inflammatory processes. DESIGN AND SETTING: Cross-sectional study in Hospital do Servidor Público Estadual de São Paulo. METHOD: The first 80 gallbladders from patients who underwent elective cholecystectomy between April and August 2002, presenting stones and chronic inflammation, were studied. The patients were divided into groups according to their age: CC1, from 15 to 40 years; CC2, from 41 to 60 years; and CC3, from 61 to 85 years. RESULTS: Twenty-one patients (26 percent) were male, while 59 (74 percent) were female. In the group CC1, intestinal metaplasia was present in 85.71 percent of the 21 patients studied; in CC2, in 79.41 percent of 34 patients; and in CC3, in 56.00 percent of 25 patients. These differences presented statistical significance (p = 0.04542). CONCLUSION: Intestinal metaplasia is extremely frequent in gallbladders with inflammation and lithiasis, especially in younger patients.

CONTEXTO E OBJETIVO: O câncer da vesicular biliar é diagnosticado tardiamente na maioria das vezes, levando invariavelmente a morte rápida. A detecção de fatores predisponentes ao aparecimento dessa neoplasia pode evitar esse desfecho. Avaliamos, neste estudo, a presença de um desses fatores, a metaplasia intestinal em vesículas biliares com cálculos e inflamação. TIPO DE ESTUDO E LOCAL: Estudo transversal, realizado Hospital do Servidor Público Estadual de São Paulo. MÉTODOS: Foram estudadas as primeiras 80 vesículas biliares de pacientes submetidos a colecistectomias eletivas entre abril e outubro de 2002, que apresentavam cálculos e inflamações crônicas. Os pacientes foram divididos em grupos segundo a faixa etária: CC1 de 15 a 40 anos, CC2 de 41 a 60 anos e CC3 de 61 a 85 anos. RESULTADOS: 21 pacientes (26 por cento) eram do sexo masculino, enquanto 59 (74 por cento) do sexo feminino. No grupo CC1, foram estudados 21 pacientes e encontrou-se metaplasia intestinal em 85,17 por cento. Já no grupo CC2 a presença de metaplasia intestinal foi de 79,41 por cento em 34 pacientes, e no grupo CC3, de 56,00 por cento em 25 pacientes avaliados. As diferenças tiveram significância estatística (p = 0,04542). CONCLUSÃO: A prevalência de metaplasia intestinal é extremamente alta em vesículas biliares com colecistite crônica e litíase, particularmente em pacientes jovens.

Retroperitoneal unicentric Castleman's disease (giant lymph node hyperplasia): case report.

CONTEXT AND OBJECTIVE: Castleman's disease, or giant lymph node hyperplasia, is a rare disorder of the lymphoid tissue that causes lymph node enlargement. It is considered benign in its localized form, but aggressive in the multicentric type. The definitive diagnosis is based on postoperative pathological findings. The aim here was to describe a case of retroperitoneal unicentric Castleman's disease in the retroperitoneum. CASE REPORT: A 61-year old white male with weight loss and listlessness presented with moderate arterial hypertension and leukopenia. Abdominal tomography revealed a 5 x 4 x 5 cm oval mass of low attenuation, with inner calcification and intense enhancement on intravenous contrast, located in the retroperitoneal region, between the left kidney and the aorta, at the renal hilus. Exploratory laparotomy revealed a non-pulsatile solid oval mass situated in the retroperitoneum, adjacent to the left renal hilus. The retroperitoneal lesion was removed in its entirety. Examination of frozen samples revealed benign lymph node tissue and histopathological examination of the surgical sample revealed hyaline-vascular giant lymph node hyperplasia (Castleman's disease). The patient was discharged on the 12th day without significant events. Two months after the operation, the patient was readmitted with severe cardiac insufficiency, acute renal failure and bronchopneumonia, which progressed to acute respiratory insufficiency, sepsis and death.

Retroperitoneal unicentric Castleman's disease (giant lymph node hyperplasia): case report

CONTEXT AND OBJECTIVE: Castleman's disease, or giant lymph node hyperplasia, is a rare disorder of the lymphoid tissue that causes lymph node enlargement. It is considered benign in its localized form, but aggressive in the multicentric type. The definitive diagnosis is based on postoperative pathological findings. The aim here was to describe a case of retroperitoneal unicentric Castleman's disease in the retroperitoneum. CASE REPORT: A 61-year old white male with weight loss and listlessness presented with moderate arterial hypertension and leukopenia. Abdominal tomography revealed a 5 x 4 x 5 cm oval mass of low attenuation, with inner calcification and intense enhancement on intravenous contrast, located in the retroperitoneal region, between the left kidney and the aorta, at the renal hilus. Exploratory laparotomy revealed a non-pulsatile solid oval mass situated in the retroperitoneum, adjacent to the left renal hilus. The retroperitoneal lesion was removed in its entirety. Examination of frozen samples revealed benign lymph node tissue and histopathological examination of the surgical sample revealed hyaline-vascular giant lymph node hyperplasia (Castleman's disease). The patient was discharged on the 12th day without significant events. Two months after the operation, the patient was readmitted with severe cardiac insufficiency, acute renal failure and bronchopneumonia, which progressed to acute respiratory insufficiency, sepsis and death.

CONTEXTO E OBJETIVO: A doença de Castleman ou hiperplasia linfonodal gigante é desordem rara dotecido linfóide que causa linfadenomegalia. É considerada afecção benigna em sua forma localizada, porém agressiva no tipo multicêntrico. O diagnóstico definitivo está baseado nas achados anatomopatológicos pós-operatórios. O objetivo foi descrever um caso de doença de Castleman (hiperplasia linfonodal gigante) unicêntrica retroperitoneal localizada no retroperitônio. RELATO DO CASO: Homem, 61 anos, branco, com emagrecimento e adinamia, apresentava hipertensão arterial moderada e leucopenia. A tomografia abdominal revelou massa ovalada de 5 x 4 x 5 cm, hipoatenuante, com calcificação de permeio e intenso realce ao contraste intravenoso, localizado na região retroperitoneal, entre o rim esquerdo e a aorta, no hilo renal. A laparotomia exploradora revelou massa sólida, não pulsátil, ovalada, situada no retroperitônio, próximo ao hilo renal esquerdo e que foi retirada completamente. O exame de congelação mostrou tecido linfonodal benigno e o exame histopatológico da peça cirúrgica revelou hiperplasia linfonodal gigante (doença de Castleman), forma hialina-vascular. O doente obteve alta no 12º dia. Após dois meses da operação, reinternou com quadro de insuficiência cardíaca grave, insuficiência renal e broncopneumonia, evoluindo com insuficiência respiratória aguda, sepse e óbito.

Curative resection plus adjuvant chemotherapy for early stage primary gastric non-Hodgkin's lymphoma: a retrospective study with emphasis on prognostic factors and treatment outcome.

BACKGROUND: There is controversy regarding the optimal therapy for primary non-Hodgkin gastric lymphoma with some authors defending surgical extirpation either alone or in association with radiotherapy and or chemotherapy, especially in relation to the earlier stages of the disease. AIM: To analyze the clinical-pathological features and the results of management approaches for patients with primary early-stage non-Hodgkin's lymphoma of the stomach operated in Surgical Gastroenterology Department, "Hospital do Servidor Público Estadual", São Paulo, SP, Brazil. The literature is reviewed to highlight the aspects of diagnosis, prognostic factors and role of the various treatment regimens. METHOD: Sixteen patients with primary early-stage gastric lymphoma underwent curative surgical treatment. The variables analyzed were age, sex, location, size, type of surgery, number of lesions, depth of invasion, histological type in accordance with Kiel's classification, involvement of lymph nodes, Ann Arbor stage classification modified by Musshoff and Schmidt-Vollmer, histological grade, margins, adjuvant therapy, clinical course and survival. RESULTS: Ten patients (62.5%) underwent subtotal gastrectomy and six (37.5%) underwent total gastrectomy. The majority (9/56.2%) of the lesions were located in the antrum. Single lesions (10/62.5%) were more frequent than multiple lesions (6/37.5%). Thirteen patients (81.2%) were classified as stage IE and three (18.7%) as stage IIE1. Primary gastric lymphoma classified histologically as low or high grade was presented by 10 (62.5%) and 6 (37.5%) patients, respectively. The most frequent histological types were the lymphoplasmocytic cytoid (4/25.0%) and centroblastic (4/25.0%). Ten patients (62.5%) received adjuvant treatment (chemotherapy and/or radiotherapy). Nine patients (56.2%), all in stage IE, reached a survival greater than 5 years and of these eight (50.0.%) had received adjuvant therapy. Two (12.5%) patients with stage IIE1 presented peritoneal relapse and died 3.0 years and 3.5 years after their respective operations. The mean overall survival was 42.5 months. CONCLUSIONS: Among the patients with primary early-stage gastric lymphoma (IE and IIE1), the gastric resection enabled an accurate clinicopathological staging, in addition to obtaining sufficient material for histopathological study and extirpation of the lesion. Furthermore, for patients with stage IE disease, the gastric resection combined with adjuvant therapy was associated with a greater than 5-year survival. Until prospective randomized studies are realized in order to evaluate the real efficacy of the different types of treatment for primary early-stage gastric lymphoma, management approaches should be individually tailored.

Curative resection plus adjuvant chemotherapy for early stage primary gastric non-Hodgkin's lymphoma: a retrospective study with emphasis on prognostic factors and treatment outcome

RACIONAL: A terapêutica do linfoma não-Hodgkin gástrico primário é controversa, com defensores da extirpação cirúrgica, da radioterapia e quimioterapia isoladas ou combinadas, especialmente em relação aos estádios mais iniciais. OBJETIVOS: Analisar as características clínico-patológicas e os resultados do tratamento nos doentes operados no Serviço de Gastroenterologia Cirúrgica do Hospital do Servidor Público Estadual, São Paulo, SP, com linfoma gástrico primário em estádio inicial. Realizar revisão da literatura, destacando os aspectos diagnósticos, fatores prognósticos e o papel das diferentes modalidades de tratamento. MÉTODO: Dezesseis doentes com linfoma gástrico primário no estádio inicial foram submetidos ao tratamento cirúrgico curativo. Idade, sexo, localização, tamanho, tipo de operação, número de lesões, profundidade da invasão, tipo histológico de acordo com a classificação de Kiel, comprometimento linfonodal, estádio pela classificação de Ann Arbor modificada por Musshoff e Schmidt-Vollmer, grau histológico, margens, terapia adjuvante, evolução e sobrevivência. RESULTADOS: Dez (62,5%) doentes foram submetidos a gastrectomia subtotal e seis (37,5%) a gastrectomia total. A maioria (9/56,2%) das lesões estava localizada no antro. Lesões únicas (10/62,5%) foram mais freqüentes que as múltiplas (6/37,5%). Treze doentes (81,2%) foram classificados no estádio IE e três (18,7%) no estádio IIE1. Dez (62,5%) enfermos apresentaram linfoma gástrico primário de baixo grau e seis (37,5%) de alto grau. Os tipos histológicos mais freqüentes foram o linfoplasmocítico citóide (4/25.0%) e o centroblástico (4/25,0%). Dez doentes (62,5%) receberam tratamento adjuvante (quimioterapia e/ou radioterapia). Nove enfermos (56,2%), todos no estádio IE, atingiram sobrevivência maior que 5 anos e oito (50,0%) receberam tratamento adjuvante. Dois (12,5%) doentes no estádio IIE1 tiveram recidiva peritonial e faleceram 3,0 anos e 3,5 anos após suas respectivas operações. A média global de sobrevivência foi de 42,5 meses...