Carcinoid tumor of cecal appendix: one-year incidence at the Santa Marcelina Hospital / Tumor Carcinoide de apêndice cecal: incidência em um ano no Hospital Santa Marcelina

Introduction: Carcinoid tumors are neuroendocrine malignancies that originate in the neuroectodermal cells of the Amine, Peptide Uptake and Decarboxylation system dispersed in the gastrointestinal mucosa and representing about 80-88% of tumors of cecal appendix. These are tumors usually diagnosed at appendectomies, and it is estimated that from each 100 appendectomies yearly performed, at least one case is a neuroendocrine tumor. Objectives: To report the experience of an University Teaching Hospital in health and reference at the east side of São Paulo and great São Paulo in cases of these rare appendicular tumors, with emphasis on the importance of these descriptions, as probably are rare those surgeons in particular who will acquire extensive wisdom in these cases. Method: Retrospective analysis of 237 patients who underwent appendectomy from September 2010 to September 2012 in the Hospital Santa Marcelina-SP. We evaluated data on age, gender, initial clinical presentation and surgical findings of patients undergoing appendectomy with subsequent anatomic and immunopathological diagnosis of carcinoid tumor of cecal appendix. Results: The presence of a carcinoid tumor of the appendix was observed in 5 patients, which corresponds to 2.1% of all appendectomies performed. Regarding gender, 4 patients (80%) were female and the average age was 34.2 years, with a range from 17 to 68 years. In all patients the initial hypothesis for surgery indication was acute appendicitis, with an intraoperative finding of necroperforated phase acute appendicitis in 3 patients (60%). Conclusion: The therapeutical conduct after the diagnosis of carcinoid tumors of the appendix must be based on the data provided by pathological and immunohistochemical studies, besides the judicious judgment of the attending physician. (AU)

Introdução: Os tumores carcinoides são neoplasias malignas neuroendócrinas que se originam em células neuroectodérmicas do sistema APUD (Amine, Peptide Uptake and Decarboxylation), dispersas na mucosa gastrointestinal e que representam cerca de 80-88% das neoplasias do apêndice cecal. São tumores diagnosticados geralmente durante apendicectomias e estima-se que de cada 100 apendicectomias realizadas por ano, ao menos um caso será TNE. Objetivos: Objetiva-se nesse artigo relatar experiência de Hospital Universitário e de Ensino (HUE) em saúde e referência na zona leste de São Paulo e grande São Paulo em casos desses raros tumores apendiculares, com ênfase na importância dessas descrições, já que provavelmente raros cirurgiões em particular irão adquirir uma extensa sapiência nesses casos. Método: Análise retrospectiva de 237 pacientes submetidos à apendicectomia no período de setembro de 2010 a setembro de 2012 no Hospital Santa Marcelina-SP. Foram avaliados os dados referentes a idade, sexo, quadro clínico inicial, achados operatórios dos pacientes submetidos à apendicectomia com posterior diagnóstico anatomopatológico e imunopatológico de tumor carcinoide de apêndice. Resultados: Verificou-se a presença de tumor carcinoide de apêndice em 5 pacientes, o que corresponde a 2,1% das apendicectomias realizadas. Com relação ao gênero, 4 pacientes (80%) eram mulheres e a média de idade foi de 34,2 anos, com variação de 17 a 68 anos. Em todos os pacientes a hipótese inicial para indicação de cirurgia fora de apendicite aguda, com achado intra-operatório de apendicite aguda em fase necroperfurada em 3 pacientes (60%). Conclusão: A conduta após o diagnóstico de tumores carcinoides de apêndice cecal deve ser alicerçada nos dados fornecidos por exames anatomopatológicos e imunoistoquímicos, além do julgamento criterioso do médico assistente. (AU)

Tumores malignos del apéndice cecal en pacientes operados por apendicitis en la IV Región de Chile / Malignant appendicular tumors

Background: The most common malignant appendicular neoplasm is the neuroendocrine well-differentiated tumor (NET). Other malignant tumors are uncommon, reported with a frequency of 0.2 percent of all appendectomies. Aim: To describe the characteristics of appendiceal malignant tumors found in regional hospitals of Northern Chile. Material and Methods: All patients operated for acute appendicitis between 2005 and 2011, in which a malignant appendiceal tumor was found in the pathological study, were analyzed. Clinical and survival information was obtained from medical records and national death registries. Results: We analyzed 8.972 appendectomy records. Appendicular tumors were found in 40 patients (0.4 percent). Thirty patients (0.3 percent) had malignant tumors. NET was the most common tumor found in 19 patients (63 percent). The tumor was smaller than 1 cm in 16 cases (53 percent). In 5 patients (17 percent), the tumor was larger than 2 cm with the appendicular base infiltrated by malignant cells. Most patients (83 percent) presented with stage I tumors. In 23 patients (77 percent, the appendectomy was considered sufficient and definitive treatment. Conclusions: In this series of patients, appendicular tumors had similar characteristics to those reported abroad. All patients had a favorable evolution without associated mortality or tumor relapse...

Introducción: El tumor apendicular maligno encontrado con mayor frecuencia es el tumor neuroendocrino bien diferenciado (NET). Otros tumores malignos se reportan con una frecuencia menor a 0,2 por ciento de todas las apendicectomías. El objetivo del presente estudio es describir las características de los tumores malignos del apéndice en la IV Región de Chile. Material y Método: Se estudió una cohorte histórica constituida por pacientes operados en los hospitales de La Serena, Coquimbo y Ovalle entre enero de 2005 y diciembre de 2011. Los resultados se reportan mediante estadística descriptiva. Resultados: Se estudiaron 8.972 apendicectomías. En 40 pacientes (0,4 por ciento) se diagnosticaron tumores apendiculares de los cuales 30 (0,3 por ciento) fueron malignos y fueron incluidos en este análisis. El tipo histológico maligno más frecuente fue el NET en 19 casos (63 por ciento). En 16 casos (53 por ciento) el tumor fue menor a 1 cm. En 5 casos (17 por ciento) el tumor fue mayor a 2 cm con compromiso de la base. La mayoría de los pacientes (83 por ciento) se presentaron con tumores en estadio I y los demás con tumores en estadio II. En 23 pacientes (77 por ciento) la apendicectomía fue considerada como el tratamiento definitivo. Conclusiones: Las características generales de los tumores malignos del apéndice en los pacientes operados por apendicitis en la IV Región de Chile son similares a las características reportadas en series nacionales e internacionales. En la presente cohorte, los pacientes tuvieron una evolución favorable sin mortalidad asociada al tumor y sin recurrencias conocidas hasta la fecha actual...

Intestinal and xxtraintestinal neoplasia in patents with inflammatory bowel diisease in a tertiary care hospital

Context The development of neoplasia is an important concern associated with inflammatory bowel disease (IBD), especially colorectal cancer (CRC). Objectives Our aim was to determine the incidence of intestinal and extraintestinal neoplasias among patients with inflammatory bowel disease. Methods There were retrieved information from 1607 patients regarding demographics, disease duration and extent, temporal relationship between IBD diagnosis and neoplasia, clinical outcomes and risk factors for neoplasia. Results Crohn's disease (CD) was more frequent among women (P = 0.0018). The incidence of neoplasia was higher in ulcerative colitis (UC) when compared to CD (P = 0.0003). Eight (0.99%) patients developed neoplasia among 804 with CD: 4 colorectal cancer, 2 lymphomas, 1 appendix carcinoid and 1 breast cancer. Thirty (3.7%) patients developed neoplasia among the 803 UC: 13 CRC, 2 lymphomas and 15 extraintestinal tumors. While CRC incidence was not different among UC and CD (1.7% vs 0.5%; P = 0.2953), the incidence of extraintestinal neoplasias was higher among UC (2.1% vs 0.5%, P = 0.0009). Ten (26.3%) patients out of 38 with neoplasia died. Conclusions CRC incidence was low and similar in both diseases. There was a higher incidence of extraintestinal neoplasia in UC when compared to CD. Neoplasias in IBD developed at a younger age than expected for the general population. Mortality associated with malignancy is significant, affecting 1/4 of the patients with neoplasia. .

Contexto O desenvolvimento de neoplasias se constitui em preocupação constante em pacientes com doenças inflamatórias intestinais (DII), especialmente o câncer colorretal (CCR). Objetivos Determinar a incidência de neoplasias intestinais e extra-intestinais entre pacientes com DII. Métodos Foram obtidas informações de 1607 pacientes, quanto a dados demográficos, duração e extensão da doença, relação temporal entre diagnóstico das DII e neoplasia, evolução clínica e fatores de risco para neoplasia. Resultados Doença de Crohn (DC) foi mais frequente entre as mulheres (P = 0.0018). A incidência de neoplasia foi maior nos doentes com retocolite ulcerativa (RCU) em relação aos com DC (P = 0.0003). Oito (0.99%) pacientes desenvolveram neoplasia entre 804 com DC: quatro tumores colorretais, dois linfomas, um carcinóide de apêndice e um câncer de mama. Trinta (3.7%) pacientes desenvolveram neoplasia entre os 803 RCU: 13 CCR, 2 linfomas e 15 tumores extra-intestinais. Enquanto a incidência de CCR não diferiu entre RCU e DC (1.7% vs 0.5%; P = 0.2953), a incidência de neoplasias extraintestinais foi maior na RCU (2.1% vs 0.5%, P = 0.0009). Dez (26.3%) pacientes de um total de 38 com neoplasia, evoluíram a óbito durante o seguimento. Conclusões A incidência de CCR foi baixa e similar em ambas as doenças inflamatórias. Observou-se incidência maior de neoplasia extra-intestinal na RCU quando comparada à DC. Neoplasias em doenças inflamatórias se desenvolveram em idade mais precoce do que a esperada para a população geral. A mortalidade associada a neoplasias é significativa, afetando 1/4 dos pacientes. .

Carcinoide gastrointestinal: experiencia de siete años en el Instituto Nacional del Cáncer (2000-2006) / Carcinoid tumors: retrospective review of 25 patients

Background: The incidence of carcinoid tumors (CT) has increased in the last five decades. These supposedly benign tumors may not always behave as such. The largest series of CT show that the most common primary tumor site (appendiceal) changed over the years. Aim: To evaluate the management of gastrointestinal CT at the National Cancer Institute from 2000 to 2006, describe their anatomic location and estimate disease specific survival. Material and Methods: Review of the database of the Cancer Committee, during a period of 7 years, looking for patients with a pathological diagnosis of CT, whose clinical records were analyzed. Results: The records of 25 patients with gastrointestinal CT, aged 18 to 79 years (19 females) were analyzed. Five years disease specific survival was 91.1 percent. The most common location was appendiceal in 56 percent of cases, with a 5 years disease specific survival of 100 percent. Right hemicolectomy was performed in seven patients with appendiceal carcinoid. Other seven patients only underwent appendectomy. Five years disease specific survival was 100 percent in both groups. Conclusions: The appendix is still the most common location of CT in this series and long term survival is acceptable.

Los tumores carcinoides (TC) gastrointestinales han aumentando su incidencia en las últimas 5 décadas. Inicialmente estos tumores eran considerados de comportamiento benigno, sin embargo, con el tiempo han mostrado ser capaces de diseminarse y tener una evolución más agresiva. En general se considera al apéndice cecal como la ubicación más frecuente de TC pero esto ha cambiado en el último tiempo según publicaciones internacionales. El objetivo de este trabajo es presentar nuestra experiencia en el manejo de los TC gastrointestinales en el período 2000-2006 en el Instituto Nacional del Cáncer (INC) reportando una serie de casos, describir su ubicación anatómica y estimar la sobrevida específica de enfermedad. Método: Estudio descriptivo. Se revisó la base de datos del Comité Oncológico del INC, durante un período de 7 años, incluyendo pacientes con diagnóstico anatomopatológico de TC. Se revisaron las fichas clínicas y se recolectaron registros de defunción. Resultados: 25 pacientes con diagnóstico de TC confirmado por patología, presentando una sobrevida específica de enfermedad a 5 años de 91,1 por ciento. La ubicación más frecuente de TC fue apéndice cecal (56 por ciento) y este grupo de pacientes presenta sobrevida específica de enfermedad de 100 por ciento a 5 años. De los TC apendiculares, en 7 pacientes se realizó una hemicolectomía derecha en un segundo tiempo, y en 7 pacientes sólo se realizó apendicectomía, con sobrevida específica de enfermedad de 100 por ciento en ambos grupos a 5 años (p = 0,475). Conclusiones: El apéndice cecal continúa siendo el lugar más frecuente de presentación del TC y la sobrevida alejada es adecuada en esta serie de pacientes.

Tumor carcinoide na extremidade distal do apêndice no serviço de cirurgia da Irmandade Santa Casa de Misericórdia de Porto Alegre / Carcinoid tumor in the distal end of the appendix in the general surgery unit of ISCMPA

Introdução: Este artigo descreve os resultados de uma coorte retrospectiva dos pacientes da ISCMPA, discutindo as características do tumor carcinoide quando localizado no apêndice. Métodos: Foram revisados os registros do banco de dados do Serviço de Patologia da ISCMPA de todas as apendicectomias realizadas entre 2000 e 2007 com posterior revisão manual dos registros de cada paciente. Resultados: Foram identificadas 3.730 apendicetomias. A prevalência de tumores carcinoides foi 0,4%. A idade mediana do paciente ao diagnóstico de tumor carcinoide foi de 26 anos, com uma distância interquartil de 20 anos. O sexo mais acometido foi o masculino. Em 46,67% dos casos, a extremidade distal foi o local do tumor e em 40% dos casos o tumor ultrapassou a camada serosa. Conclusões: Houve dificuldades em se definir claramente se as alterações no apêndice identificadas no transoperatório correspondiam a tumor carcinoide. A congelação no transoperatório ajudou a definir o tipo de tratamento cirúrgico mais adequado e a identificar metástases de outras patologias sistêmicas

Introduction: This paper describes the results of a retrospective cohort of patients of the ISCMPA, discussing the features of carcinoid tumors occurring in the appendix. Methods: We searched the database of the ISCMPA Department of Pathology for all the records of appendectomies performed between 2000 and 2007, with subsequent manual review of individual patient records. Results: 3,730 appendectomies were identified. The prevalence of carcinoid tumors was 0.4%. The median age at diagnosis of carcinoid tumor was 26 years with an interquartile range of 20 years. Males were more affected than females. In 46.67% of the cases the distal end was the site of the tumor and in 40% the tumor exceeded the serosa layer. Conclusions: There were difficulties in clearly defining if the changes in the appendix identified during surgery corresponded to a carcinoid tumor. Transoperative freezing helped determine the most appropriate type of surgical approach and identify metastases of other systemic diseases

Appendiceal tumour--retrospective clinicopathological analysis.

Appendiceal tumours are rare and often discovered unexpectedly in an acute situation in which decision-making is difficult. We report the spectrum of appendiuar tumours seen in our institution over a period of more than 10 years, and discuss the clinicopathological behaviour, investigations, surgical procedures and outcomes in these patients. We have also reviewed the literature with regard to appendiceal tumours. Appendicular tumours were identified from the database of 1646 appendictomies (18% in children) performed in single centre and case notes were reviewed. Clinical presentation, investigations, histopathology, surgical procedures and outcome were analysed. Twelve patients with appendiceal tumours were identified (0.72%): 8 carcinoid, 2 mucinous (mucocele) and 2 adenocarcinoma. All the patients with a carcinoid tumour presented with features suggestive of acute appendicitis and were diagnosed postoperatively following appendicectomy and formal histology. No further surgical intervention was required as these lesions were less than 1cm away from the base of the appendix. One of the patient with mucinous cystadenoma presented acutely and underwent an appendicectomy; in the other patient with chronic pain, apreoperative MRI suggested the diagnosis leading to a planned hemicolectomy as the lesion was close to the base of the appendix. While one of the patient with an adenocarcinoma localized to the appendix did well following a right hemicolectomy, the other patient with disseminated disease succumbed within a year. Carcinoid tumours are the commonest appendiceal tumours, which present often as acute appendicitis. While appendicectomy would be adequate in most of these patients, in patients with a cystadenoma close to the base of the appendix or in case of a carcinoma, a right hemicolectomy is the appropriate option. While the prognosis is good in patients with carcinoid tumour and cystadenoma, it remains dismal in patients with disseminated malignant disease.