Anesthetic key points in a patient with a terminal ileum neuroendocrine tumor and a rare carcinoid left heart disease presented for non-cardiac surgery: case report.

BACKGROUND: Carcinoid tumors are rare neuroendocrine malignancies presenting in an increasing number in our center. The incidence of carcinoid tumors is approximatively between 2.5 and 5 cases per 100,000 people of whom about 50% develop carcinoid syndrome. Once the carcinoid syndrome has developed, a carcinoid cardiomyopathy can occur. Carcinoid heart disease (CaHD) remains a serious and rare complication associated with a significant increase in morbidity and mortality. Although carcinoid tumors have been known and studied for several years, there are still scarce data on the anesthetic management and the peri operative period. CASE PRESENTATION: We describe a case of a Caucasian 44-year-old woman with an unusual presentation of left CaHD with an ileal neuroendocrine tumor and liver metastases. Our preoperative somatostatin administration protocol, limit the cardiac damage. The maintenance of stable hemodynamics, the use of balanced anesthetic technique, all along with a good understanding of the pathology, played a major role in the successful management of anesthesia. This case report allows us to introduce our decision algorithm for the management of this type of pathology in our tertiary hospital, Cliniques Universitaires Saint-Luc. CONCLUSION: Despite the paucity of data, anesthetic management of patients with carcinoid tumor can be safely performed with effective hemodynamic monitoring and a good understanding of the pathophysiology. Knowledge and application of a clear institutional algorithm for octreotide administration and multidisciplinary consultation at a referral center are essential for the management of these patients.

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.

Ileal perineurioma as a cause of intussusception / Perineurioma ileal como uma causa de intussuscepção

CONTEXT: Perineuriomas are rare tumors composed of cells resembling those of the normal perineurium. It usually occurs in subcutaneous, soft-tissue or intraneural locations. Very few reports in the literature have described perineuriomas in the gastrointestinal tract, including the stomach, colon and jejunum. CASE REPORT: We report the clinicopathological and immunohistochemical features of a case of ileal perineurioma that was manifested clinically as intestinal obstruction due to intussusception. Ileal perineurioma has not previously been reported at this anatomical location.

CONTEXTO: Perineurioma é uma rara neoplasia composta de células que lembram aquelas do perineuro normal e geralmente ocorre no subcutâneo, tecidos moles ou em localização intraneural. Poucos relatos na literatura descrevem perineuriomas no trato gastrointestinal incluindo estômago, cólon e jejuno. RELATO DE CASO: Os autores apresentam as características clinicopatológicas e imunoistoquímicas de um caso de perineurioma ileal apresentando-se clinicamente por obstrução intestinal decorrente de intussuscepção. Perineurioma ileal não havia sido descrito até o momento nessa localização anatômica.

Lipoma de la válvula ileocecal: informe de un caso clínico y revisión de la literatura / Ileocecal valve lipoma: case report and review of the literature

Introducción: los lipomas son los tumores benignos más frecuentes en tubo digestivo y hasta 50 % se encuentra en el colon. La mayoría son submucosos y varían de acuerdo a su incidencia, localización, sintomatología y patogénesis, lo que dificulta su diagnóstico. Nuestro objetivo fue conocer las diferentes manifestaciones clínicas causadas por un lipoma en la válvula ileocecal, y hacer una revisión de la literatura de esta localización poco frecuente. Caso clínico: mujer de 78 años que presentó cuadros repetidos de oclusión intestinal parcial con distensión abdominal, constipación y obstipación. Se realizó colonoscopia que mostró tumoración submucosa de 30 mm en válvula ileocecal; la tomografía computarizada de abdomen mostró tumoración nodular de densidad grasa en válvula ileocecal. La paciente fue sometida a laparotomía exploradora y cecotomía, donde se identificó y resecó tumoración submucosa, la cual fue enviada a estudio transoperatorio; dado que se trató de una tumoración benigna, se respetó la válvula ileocecal y se realizó cierre primario. El reporte histopatológico definitivo fue lipoma, negativo para malignidad. Conclusiones: es importante conocer las distintas formas de presentación de los lipomas, ya que fácilmente pueden confundirse con lesiones malignas (sobre todo si existe ulceración de la mucosa) y someter al paciente a procedimientos mayores. Sólo deben resecarse si producen síntomas o son mayores a 2 cm.

BACKGROUND: Lipomas are the most frequent benign tumors of the digestive tract and 50% are localized in the colon. Most are found submucosally and may vary in their incidence, localization, symptoms and pathogenesis, making diagnosis difficult. We undertook this study to learn the different presentations of an ileocecal valve (ICV) lipoma and conduct a review of the literature due to this uncommon location. CASE PRESENTATION: We present the case of a 78-year-old female with intermittent small bowel occlusion, abdominal distention and constipation. Colonoscopy showed a submucous tumor at the ICV, CT scan showed a tumor at the ICV with fat density. Laparotomy was performed with primary resection of the lesion by cecotomy, frozen section was reported as nonmalignant so a primary closure without ICV involvement was achieved. Definitive pathology revealed a benign lipoma. CONCLUSION: It is important to know the different presentations of these benign tumors because if there is mucosal ulceration they can be mistaken for a malignant lesion and lead to greater resections. Resection is necessary if they are symptomatic or >2 cm.

Complicaciones agudas del linfoma yeyuno-ileal / Acute complications of jeyuno-ileal lymphoma

El linfoma de intestino delgado es una entidad cuya incidencia oscila entre el 1 y 4 por ciento de todos los tumores malignos del aparato digestivo. Es habitual su presentación clínica como cuadros agudos de abdomen, lo que determina que el cirujano se enfrente a los mismos en los servicios de urgencia. En estas circunstancias, y con un diagnóstico etiológico no siempre establecido, se emprende el tratamiento quirúrgico. Su confirmación anátomo patológica, permite emprender el tratamiento adyuvante en base a quimio y radioterapia. Se analizan en forma retrospectiva seis casos clínicos que se presentaron como cuadros agudos de abdomen y fueron sometidos a cirugía de urgencia, sin complicaciones ni mortalidad operatoria.

Anemia ferropénica debida a tumor del estroma de localización ileal / Iron deficiency anemia due to stromal tumor of the ileum

La anemia ferropénica ocurre en el 3,5% al 5,3% de los varones adultos y de las mujeres menopáusicas. La pérdida digestiva crónica es la causa en la mayoría de los casos. Cinco a 20% de los pacientes quedan sin diagnóstico tras haberse descartado por estudios endoscópicos lesiones en el colon y en el tracto digestivo superior. En el intestino delgado, las angiodisplasias y los tumores son las causas más frecuentes de sangrado intestinal oculto. Su diagnóstico requiere la realización de estudios complejos con bajo rendimiento que necesariamente hay que instrumentar en los casos anemia ferropénica.

Tomografía computada helicoidal y ecografía de las invaginaciones intestinales del adulto / Spiral CT and sonographic finding in intussusception in adults

La invaginación intestinal es una entidad rara en el adulto, secundaria, en la mayoría de los casos, a lesiones orgánicas. Al menos la mitad de estos casos en el colon se debe a tumores malignos, pero en el intestino delgado la mayoría son lesiones benignas. Presentamos una serie de 7 casos de invaginaciones intestinales en adultos debidas tanto a tumores malignos como benignos

Unusual case of Meckel's diverticulum: a case report and review of an atypical form of presentation

This is a review of a child who developed symptomatic anemia secondary to a huge Meckel's Diverticulum (MD). The patient presented with multiple complications, such as: neoplasia, occult chronic bleeding, giant size MD, partial intestinal obstruction and severe symptomatic anemia. There was complete resolution of the condition after resection and ileo-ileal anastomosis. After revision of the literature, this case is the first report of MD occurring concomitantly with such a myriad of signs and symptoms

Tumor autonomico gastrointestinal asociado a enfermedad de von Recklinghausen / Gastrointestinal autonomic tumor associated with von Recklinghausen's disease

El tumor autonómico gastrointestinal es un tipo raro de tumor estromal gasrointestinal que parece originarse del plexo autónomo mioentérico. Ocasionalmente estos tumores han estado asociados a enfermedad de von Recklinghausen (EVR). Presentamos el caso de una mujer de 63 años con EVR con dos tumores ileales que evidenciaron las características de los tumores autonómicos gastrointestinales y una revisión de la literatura sobre esta peculiar combinación.

Esclerose sistêmica progressiva, linfoma maligno de células B em íleo e adenocarcinoma de tireóide / Progressive systemic sclerosis, ileal B-cell malignant lymploma and thyroid adenocarcinoma

Relatamos um caso de paciente portadora de esclerose sistêmica progressiva (ESP) que evoluiu rapidamente para óbito. O exame necroscópico revelou além de alteraçöes morfológicas características de ESP em pele, rim, coraçäo e vasos, a presença de linfoma maligno de células B em íleo com metástases generalizadas e adenocarcinoma de tireóide. Os autores sugerem que o mecanismo possivelmente envolvido na associaçäo destas duas doenças seja dependente da açäo dos oncogenes, que modificam a homeostase celular e a tolerância imunológica

Invaginaçäo intestinal por melanoma / Intestinal intussusception by melanoma

Os autores relatam um caso de invaginaçäo intestinal por melanoma, acreditando estarem contribuindo para acrescentar ao conhecimento desta entidade, por si só, rara algumas características ainda mais curiosas. Simultaneamente, discutem os vários aspectos inerentes à doença de acordo com a literatura revisada