Most small bowel cancers are revealed by a complication / A maioria dos cânceres de intestino delgado são revelados por uma complicação

ABSTRACT Objective To characterize the pattern of primary small bowel cancers in a tertiary East-European hospital. Methods A retrospective study of patients with small bowel cancers admitted to a tertiary emergency center, over the past 15 years. Results There were 57 patients with small bowel cancer, representing 0.039% of admissions and 0.059% of laparotomies. There were 37 (64.9%) men, mean age of 58 years; and 72 years for females. Out of 57 patients, 48 (84.2%) were admitted due to an emergency situation: obstruction in 21 (38.9%), perforation in 17 (31.5%), upper gastrointestinal bleeding in 8 (14.8%), and lower gastrointestinal bleeding in 2 (3.7%). There were 10 (17.5%) duodenal tumors, 21 (36.8%) jejunal tumors and 26 (45.6%) ileal tumors. The most frequent neoplasms were gastrointestinal stromal tumor in 24 patients (42.1%), adenocarcinoma in 19 (33.3%), lymphoma in 8 (14%), and carcinoids in 2 (3.5%). The prevalence of duodenal adenocarcinoma was 14.55 times greater than that of the small bowel, and the prevalence of duodenal stromal tumors was 1.818 time greater than that of the small bowel. Obstruction was the complication in adenocarcinoma in 57.9% of cases, and perforation was the major local complication (47.8%) in stromal tumors. Conclusion Primary small bowel cancers are usually diagnosed at advanced stages, and revealed by a local complication of the tumor. Their surgical management in emergency setting is associated to significant morbidity and mortality rates.

RESUMO Objetivo Caracterizar o padrão de neoplasias malignas primárias do intestino delgado em um hospital terciário de Leste Europeu. Métodos Estudo retrospectivo de pacientes com câncer de intestino delgado, internados em um hospital terciário e de emergência, ao longo dos últimos 15 anos. Resultados Foram avaliados 57 pacientes com neoplasias malignas gastrintestinais, o que representou 0,039% das admissões e 0,059% das laparotomias realizadas. Total de 37 (64,9%) pacientes masculinos, média de idade de 58 anos, e de 72 anos para mulheres. Dentre os 57 pacientes, 48 (84,2%) foram internados em situação de emergência: obstrução intestinal em 21 (38,9%), perfuração em 17 (31,5%), hemorragia digestiva alta em 8 (14,8%), e hemorragia digestiva baixa em 2 (3,7%). Houve 10 (17,5%) tumores duodenais, 21 (36,8%) jejunais e 26 (45,6%) ileais. As neoplasias mais frequentes foram tumor estromal gastrintestinal, em 24 (42,1%) pacientes, adenocarcinoma em 19 (33,3%), linfoma em 8 (14%) e carcinoides em 2 (3,5%). A prevalência de adenocarcinoma duodenal foi 14,55 vezes maior do que a do intestino delgado, e a prevalência de tumores estromais duodenais foi 1,818 vez maior do que a do intestino delgado. A obstrução intestinal foi complicação do adenocarcinoma em 57,9% dos casos, e a perfuração foi a principal complicação local (47,8%) dos tumores estromais. Conclusão As neoplasias malignas primárias do intestino delgado foram geralmente diagnosticadas em estado avançado e reveladas por uma complicação local do tumor. O tratamento cirúrgico em situação de emergência está associado à significativa morbimortalidade.

Sindrome carcinoide: achados ecocardiográficos na evolução clínica de um caso de síndrome carcinoide / Carcinoid syndrome: echocardiography findings in a clinical evolution of a carcinoid syndrome case

O comprometimento cardíaco na síndrome carcinoide ocorre em 50% dos pacientes, sendo o ecocardiograma um importante meio diagnóstico da doença. Os achados de exame físico podem passar despercebidos, como o sopro tricúspide e pulmonar, em virtude dabaixa pressão no território pulmonar. Apesar de alguns casos responderem bem aos análogos da somastotina, como o octeotride, a doença cardíaca evolui, inexoravelmente, para insuficiência cardíaca, sendo a indicação cirúrgica uma opção para determinados casos.

Tumor carcinoide recidivante de recto: presentación de un caso / Recurrent carcinoid tumor of the rectum: case report

Los tumores carcinoides son neoplasias que incluyen un grupo de lesiones muy heterogéneas y de lento crecimiento. La mayoría de ellos aparecen en tracto respiratorio y aparato digestivo, en apéndice, intestino delgado y recto en un 90% de los casos. La presentación en recto a menudo es tratable y curable y las metástasis están relacionadas con el tamaño del tumor, siendo los menores a 1 cm lesiones con poca probabilidad de metástasis y los mayores de 1 cm con frecuencia dan metástasis. Los tumores carcinoides a excepción de los de recto, pueden producir una variedad de sustancias endocrinas cuya manifestación clínica es el síndrome carcinoide. El tratamiento de elección es la cirugía y en los casos de lesiones pequeñas, la resección endoscópica suele ser suficiente si no sobrepasan la submucosa y no hay atipias. Presentamos el caso de un paciente masculino de 63 años, con síntomas pépticos y alteraciones del habito intestinal, evaluado con hallazgos de pólipo en recto, cuya histología reporto tumor carcinoide de 0.8 cm por lo que se practico resección mucosal. Un año después en control endoscópico se evidencio nueva lesión carcinoide en recto alto a 5cms por debajo de la lesión previa, la cual fue resecada endoscopicamente con hallazgos de invasión a la submucosa, al USE no se evidenció lesión carcinoide ni adenomegalias. Se refirió a Cirugía para tratamiento quirúrgico, se practicó colectomía izquierda sin evidencias histológicas de tumor en la pieza operatoria.

Carcinoid tumors are neoplasms that include a heterogeneous group of lesions and slow growth. Most of them appear in the respiratory and digestive tract, in appendix, small intestine and rectum in 90% of cases. The right presentation is often treatable and curable and metastases are associated with tumor size, being smaller than 1 cm lesions with low probability of metastasis and greater than 1 cm often give metastases. Carcinoid tumors except the rectum, can produce a variety of endocrine substances whose clinical manifestation is the carcinoid syndrome. The preferred treatment is surgery and in cases of small lesions, endoscopic resection is usually sufficient if not beyond the submucosa and no atypia. We report a male patient of 63 years, peptic symptoms and altered bowel habit, as assessed by rectal polyp findings, the histology reported carcinoid tumor of 0.8 cm so that practical mucosal resection. A year later endoscopic control evidenced rectal carcinoid reinjury high to 5cm below the previous injury, which was resected endoscopically with findings of invasion to the submucosa, there was no evidence to USE carcinoid lesion or lymphadenopathy. He referred to surgery for surgical treatment, left colectomy was performed without histologic evidence of tumor in the surgical specimen.

Periampullary carcinoid: An uncommon tumor at an unusual site.

Gastrointestinal carcinoid tumors represent a group of well-differentiated tumors originating from various neuroendocrine cells located in the gastrointestinal mucosa and submucosa. Consequently, there is diversity in their clinical presentation, incidence at specific anatomic sites, biological behavior, hormone production, morphologic characteristics, and immunophenotype. Periampullary carcinoids are extremely rare and less then 100 patients have been reported in the world literature, that too mostly as case reports. We are reporting two cases of periampullary carcinoids, one of which presented with rare manifestation as gastrointestinal bleed and both are doing well after successful pancreatoduodenectomy.

Síndrome carcinóide com manifestação cardíaca à esquerda / Carcinoid syndrome affecting left heart

Tumores carcinoides são neoplasias neuroendócrinas raras que se originam geralmente nas células enterocromafins do trato gastrintestinal (TGI). Secretam substâncias vasoativas, como a prostaglandina e a 5-HT (serotonina). A síndrome carcinoide ocorre quando há metástase para o fígado, local onde tais substâncias são inativadas. Clinicamente, é caracterizada por flusinhg, diarréia, broncoespasmo e manifestação cardíaca. Descrevemos o caso de uma paciente de 46 anos, com história de carcinoide brônquico, internada com dores abdominais e flushing. Tomografia abdominal mostrou múltiplas metástases hepáticas. Ecocardiografia mostrou ventrículo direito aumentado e movimento anômalo do septo interventricular. Valvas tricúspide e mitral difusamente espessadas e com insuficiência grave em ambas. Manifestação cardíaca na síndrome carcinóide é comum e confere um pior prognóstico à doença. Ecocardiograma é o método de escolha para investigação, pois revela lesões valvares patognomônicas. Acometimento das valvas à esquerda ocorre em apenas 15% dos casos, devido à inativação da serotonina nos pulmões, mas pode ocorrer em casos de tumor brônquico, como neste relato.

Carcinoid tumors are rare neuroendocrine neoplasms that commonly originate from the gastrointestinal tract and produce vasoactive substances like serotonine and prostaglandin. Carcinoid syndrome occurs when there is metastasis to the liver, where those substances are inactivated. Characteristic clinical features are flushing, diarrhea, bronchoconstriction and cardiac abnormalities. We describe a case of a 46-year-old female patient with previous carcinoid tumor in the lung, who was admitted with abdominal cramps and flushing symptoms. Abdominal CT scan detected multiple liver metastasis. Echocardiography showed enlarged right ventricle with paradoxical septal motion, tricuspid and mitral valves diffusely thickened with severe regurgitation on both. Cardiac manifestation in carcinoid syndrome is common and confers a worse prognosis. Echocardiography is the method of choice for assessment, since it detects pathognomonic valvular lesions. Left valves are affected in only 15% of cases, because of serotonine inactivation in the lungs, but it can happen in bronchial tumor, like in this case.

Small bowel obstruction by a terminal ileum carcinoid tumor: a case report

Carcinoid tumors are well differentiated neuroendocrine tumors with secretory components. These tumors are uncommon but the most common primary tumors of the distal small intestine. We present a rare terminal ileum carcinoid tumor presenting with a small bowel obstruction. A 65 years old man presented with intermittent, generalized, dull and colicky abdominal pain accompanied with intermittent nausea, fever and chills for 1 year and post prandial generalized colicky abdominal pain from 5 days prior to admission. He also complained of weight loss and frequent constipations during recent year. His abdomen was soft with mild tenderness in periumbilical, right lower quadrant and left lower quadrant without guarding, rebound tenderness and palpable mass. Laboratory findings indicated anemia, and barium enema showed right lower quadrant mass effect in small intestine. Narrowing of terminal ileum was noted in colonoscopy. Free fluid in lower abdomen and pelvis with 37*28*25 paravertebral hypoechoic pelvic mass, without peristalsis was seen in abdomen and pelvic sonography. After mass localization in abdominal CT scan, laparotomy and excisional biopsy was performed. The diagnosis of carcinoid tumor was confirmed by pathologic report. Carcinoid tumors are rare tumors of the Gastro intestinal tract, however, they are the most common primary tumors of the small intestines. Most of these tumors have a very indolent course and may present with non specific symptoms. In view of the poor prognosis associated with the late diagnosis, it is imperative to think of this differential diagnosis in patients presenting with non specific symptoms and in intermittent partial bowel obstruction

Pitfalls in the diagnosis of Cushing's syndrome: [review]

Among endocrine disorders, Cushing's syndrome (CS) is certainly one of the most challenging to endocrinologists due to the difficulties that often appear during investigation. The diagnosis of CS involves two steps: confirmation of hypercortisolism and determination of its etiology. Biochemical confirmation of the hypercortisolaemic state must be established before any attempt at differential diagnosis. Failure to do so will result in misdiagnosis, inappropriate treatment, and poor management. It should also be kept in mind that hypercortisolism may occur in some patients with depression, alcoholism, anorexia nervosa, generalized resistance to glucocorticoids, and in late pregnancy. Moreover, exogenous or iatrogenic hypercortisolism should always be excluded. The three most useful tests to confirm hypercortisolism are the measurement of 24-h urinary free cortisol levels, low-dose dexamethasone-suppression tests, and determination of midnight serum cortisol or late-night salivary cortisol. However, none of these tests is perfect, each one has different sensitivities and specificities, and several are usually needed to provide a better diagnostic accuracy. The greatest challenge in the investigation of CS involves the differentiation between Cushing's disease and ectopic ACTH syndrome. This task requires the measurement of plasma ACTH levels, non-invasive dynamic tests (high-dose dexamethasone suppression test and stimulation tests with CRH or desmopressin), and imaging studies. None of these tests had 100 percent specificity and their use in combination is usually necessary. Bilateral inferior petrosal sinus sampling is mainly indicated when non-invasive tests do not allow a diagnostic definition. In the present paper, the most important pitfalls in the investigation of CS are reviewed.

Entre as doenças endócrinas, a síndrome de Cushing (SC) é certamente uma das mais desafiadoras para o endocrinologista, devido às dificuldades que comumente surgem durante a investigação. O diagnóstico de SC envolve dois passos: a confirmação do hipercortisolismo e a determinação de sua etiologia. A confirmação bioquímica do excesso de cortisol precisa ser estabelecida antes de qualquer tentativa de diagnóstico diferencial; caso contrário, poderá resultar em diagnóstico incorreto, tratamento impróprio e manejo insuficiente. Deve também ser lembrado que hipercortisolismo pode ocorrer em certos pacientes com depressão, alcoolismo, anorexia nervosa, resistência generalizada aos glicocorticóides e no final da gravidez. Além disso, hipercortisolismo exógeno ou iatrogênico deverá ser sempre excluído. Os três testes mais úteis para a confirmação do hipercortisolismo são: a medida do cortisol livre em urina de 24 h, os testes de supressão com dexametasona (TSD) em doses baixas e a determinação do cortisol sérico à meia-noite ou do cortisol salivar no final da noite. Contudo, nenhum deles é perfeito, cada um com sua sensibilidade e especificidade, sendo vários deles usualmente necessários para fornecer uma melhor acurácia diagnóstica. O maior desafio na investigação da SC envolve a diferenciação entre a doença de Cushing e a síndrome do ACTH ectópico. Esta tarefa requer a medida dos níveis plasmáticos de ACTH, testes dinâmicos não-invasivos (TSD com doses altas e testes de estímulo com CRH ou desmopressina) e estudos de imagem. Nenhum desses testes tem 100 por cento de especificidade e muitas vezes é necessário seu uso combinado. Amostragem venosa do seio petroso inferior está indicada principalmente quando os testes não-invasivos não permitem uma definição diagnóstica. Neste artigo, revisaremos as mais importantes armadilhas na investigação da SC.

Ectopic ACTH syndrome

Ectopic adrenocorticotropic secretion (EAS) is responsible for 12-17 percent of cases of Cushing's syndrome (CS) and covers a range of tumours, from undetectable benign lesions to widespread metastases. The syndrome is often associated with severe hypercortisolaemia, which aggravates the underlying condition. EAS requires a complete workup that includes the establishment of endogenous CS, diagnosis of adrenocorticotropic hormone (ACTH) dependency, localization of the source of ACTH secretion and rapid biochemical control of hypercortisolaemia. Dynamic endocrine tests should include inferior petrosal sinus sampling with CRH stimulation. Localization studies depend on the availability of reliable high-resolution cross-sectional imaging. This systematic review of the largest published series of patients with EAS (over 380 patients) reveals the common trends in the prevalence and management of this syndrome. The concept of 'occult' EAS has been revisited and the terms 'overt' and 'covert' EAS introduced. In addition to small cell lung carcinoma, the most common causes of ectopic EAS are bronchial carcinoids, thymic tumours, islet cell tumour of the pancreas, medullary thyroid carcinomas, and phaeochromocytomas. Their prevalence and the best localization modalities are presented. Medical and surgical management is discussed on the basis of the extensive experience of major referral centres.

A secreção ectópica de ACTH (SEA) é responsável por 12-17 por cento dos casos de síndrome de Cushing (SC), cobrindo uma variedade de tumores, desde lesões benignas indetectáveis a metástases disseminadas. A SEA está freqüentemente associada com hipercortisolemia grave, que agrava a condição de base e requer uma avaliação completa, que inclui a confirmação da SC endógena, o diagnóstico da dependência ao ACTH, a localização da fonte da secreção de ACTH e o controle bioquímico rápido da hipercortisolemia. Testes endócrinos dinâmicos devem incluir a coleta de amostras do seio petroso inferior com estímulo pelo CRH. O estudo da localização da fonte depende da disponibilidade de procedimentos de imagem de alta-resolução confiáveis. A revisão sistemática das maiores séries publicadas de pacientes com SEA (mais de 380 pacientes) revela tendências comuns na prevalência e manejo dessa síndrome. O conceito de SEA "oculta" está sendo revisado e os termos SEA "manifesta" e "latente" são introduzidos. Além do carcinoma pulmonar de pequenas células, as causas mais comuns de SEA são os carcinóides brônquicos, tumores tímicos, tumor de ilhotas pancreáticas, carcinoma medular de tiróide e feocromocitoma; sua prevalência e as melhores modalidades para localização são apresentadas. O manejo clínico e cirúrgico é discutido com base na vasta experiência dos principais centros de referência.

A Case of Lambert-Eaton Myasthenic Syndrome Associated with Atypical Bronchopulmonary Carcinoid Tumor

The Lambert-Eaton myasthenic syndrome (LEMS) is typically recognized as a paraneoplastic syndrome associated with a small cell lung carcinoma (SCLC), whereas LEMS with other neuroendocrine lung tumors, including carcinoids or large cell lung carcinoma, are highly unusual. Here, we report a rare case of LEMS with atypical bronchopulmonary carcinoid tumor: A 65-yr-old man presented with progressive leg weakness and a diagnosis of LEMS was made by serial repetitive nerve stimulation test. Chest CT revealed a lung nodule with enlargement of paratracheal lymph nodes, and surgically resected lesion showed pathological features of atypical carcinoid tumor. We concluded that LEMS could be associated with rare pulmonary neuroendocrine tumor other than SCLC, which necessitates pathologic confirmation followed by aggressive treatment for optimal management in these rare cases.

Large nodal metastases from carcinoid tumor causing bowel obstruction.

A 39-year-old man presented with progressively increasing constipation and painful lumps in the abdomen. Exploration showed extensive nodal metastases but no primary lesion was seen. The masses were excised and sigmoid colostomy done. Histology revealed carcinoid tumor with small cell differentiation. A trial of chemotherapy gave no response. At follow up of 18 months he is leading a comfortable life with a colostomy and a billary stent in place (placed for obstructive jaundice due to porta node).

The ectopic ACTH syndrome and superior vena cava obstruction due to a malignant carcinoid tumour of the thymus

We report the case of a 28-year-old African Caribbean woman with Cushing's syndrome and superior vena cava obstruction secondary to an ACTH-secreting carcinoid tumour of the thymus. The case highlights the problems which may be encountered in performing the 2-day high dose dexamethasone suppression test but clinicians are reminded that this test or any other dynamic test is absolutely essential for elucidating the cause of ACTH-dependent Cushing's Syndrome.

Carcinoid tumor of common hepatic duct.

Primary carcinoid tumors of the biliary tract are extremely rare. We report a 36-year-old woman with recurrent acute cholangitis who was diagnosed to have a carcinoid in the common hepatic duct, with enlarged local nodes. She underwent local resection. I-131 metaiodobenzyl guanidine (MIBG) scanning postoperatively showed no uptake in the tumor bed.

Tumor carcinoide duodenal / Duodenum carcinoid tumor

El tumor carcinoide duodenal es un tumor infrecuente. Por esta razón es una patología poco conocida por el cirujano general. Sin embargo, se ha observado un aumento de su frecuencia en los últimos 20 años. Es conveniente tener un conocimiento claro sobre la estrategia diagnóstica en las diferentes etapas de la enfermedad. Esto permitiría adecuar la conducta terapéutica a cada caso en particular. Se revisan los casos de tumor carcinoide duodenal entre 1954 y 1997 en el Hospital San Juan de Dios. Se presenta la experiencia con el tratamiento quirúrgico en los dos únicos pacientes portadores de esta lesión, atendidos en los últimos dos años, con un seguimiento postoperatorio de 12 y 30 meses

Ectopic ACTH syndrome due to bronchial carcinoid tumour--a case report.

A patient is reported who had Cushing's syndrome and carcinoid tumour of the bronchus. The case illustrates the difficulty in preoperative localization of the ectopic ACTH source and the surgical management of such patients.

Carcinoide de intestino delgado: una causa rara de infarto intestino mesentérico / Carcinoid tumor of small intestine, cause of mesenteric intestinal infarction

Las neoplasias del intestino delgado son poco frecuentes. Los carcinoides son el 20 por ciento de éstas. La mayoría son asintomáticos y son hallazgos incidentales, el resto se presenta por sus complicaciones, como cuadros agudos de abdomen, el más frecuente es la oclusión intestinal. El infarto intestino mesentérico y la isquemia intestinal han sido reconocidos como otra presentación muy rara, en estos casos la terapéutica quirúrgica agresiva, a pesar de la gravedad de la presentación, muestra beneficios en el pronóstico. Se presentan 2 casos y se discuten los eventos patogénicos

Tumor carcinoide apendicular y embarazo

El Tumor Carcinoide apendicular es una neoplasia infrecuente en adultos. Su prevalencia es del 0.32 por ciento en los apéndices extraídos en cirugía y del 0.054 por ciento en los estudiantes en autopsias. Su tratamiento y pronóstico dependen de su localización y extensión. No existe ningún caso reportado de metástasis en pacientes previamente apendicectomizadas con tumor carcinoide menor de 2 cm. Se presenta el caso de una mujer de 32 años con embarazo de 24 semanas y tumor carcinoide

Spontaneous appendicocutaneous fistula.

Spontaneous appendicocutaneous fistula is a rare complication of appendicitis. We report a case of appendicular carcinoid who presented with appendicocutaneous fistula.