Carcinoid syndrome: update on the pathophysiology and treatment

Approximately 30-40% of patients with well-differentiated neuroendocrine tumors present with carcinoid syndrome, which is a paraneoplastic syndrome associated with the secretion of several humoral factors. Carcinoid syndrome significantly and negatively affects patients' quality of life; increases costs compared with the costs of nonfunctioning neuroendocrine tumors; and results in changes in patients' lifestyle, such as diet, work, physical activity and social life. For several decades, patients with neuroendocrine tumors and carcinoid syndrome have been treated with somatostatin analogues as the first-line treatment. While these agents provide significant relief from carcinoid syndrome symptoms, there is inevitable clinical progression, and new therapeutic interventions are needed. More than 40 substances have been identified as being potentially related to carcinoid syndrome; however, their individual contributions in triggering different carcinoid symptoms or complications, such as carcinoid heart disease, remain unclear. These substances include serotonin (5-HT), which appears to be the primary marker associated with the syndrome, as well as histamine, kallikrein, prostaglandins, and tachykinins. Given the complexity involving the origin, diagnosis and management of patients with carcinoid syndrome, we have undertaken a comprehensive review to update information about the pathophysiology, diagnostic tools and treatment sequence of this syndrome, which currently comprises a multidisciplinary approach.

Abnormalities of the interdigestive migrating motor complex in a patient with carcinoid syndrome

As pressöes intraluminais do antro gástrico, duodeno e jejuno foram registradas durante 206 minutos após jejum de 12 horas em uma paciente com síndrome carcinóide devida a neoplasia de células enterocromafins do ileo e com metástasis hepáticas. A alteraçäo mais conspícua foi a presença de taquirritmia de 16-17 ondas de pressäo/minuto, predominando nos "fronts" de atividade tanto no duodeno como no jejuno. Períodos com 11-12 ondas/minutos apareceram irregularmente e a ocorrência simultânea de 12 ondas/minuto no duodeno e 16 ondas/minuto no jenuno foi registrada. O antro gástrico permaneceu em quiescência motora durante todo o transcurso do estudo manométrico. Presume-se que as alteraçöes sejam produzidas pelas substâncias secretadas pelo tumor carcinóide

Tumor neuroendocrino de intestino / Neuroendocrine tumor of intestine

Se presentan 6 casos de tumores carcinoides de intestino, que tras una revisión de los preparados histológicos se los clasifica en 3 tumores neuroendócrinos benignos o carcinoides y 3 carcinomas neuroendócrinos. Se analiza la evolución clínica de los mismos. El empleo en la actualidad de técnicas más especificas para el diagnóstico de estos tumores ha permitido un mayor conocimiento de los mismos. Dichas técnicas, inmunomarcación enolasa neuroespecífica, grimelius, cromogranina, etc. son utilizadas por el patólogo cuando por las características macro y microscópicas sospeche un tumor de esta estirpe; (tumor amarillo brillante originado profundamente en la mucosa y que crece tanto hacia la luz como hacia la submucosa). La morfología celular, en base a las mitosis, atípicas celulares y límites del tumor, permite diferenciar a estas neoplasias en tumores de alto y bajo grado de malignidad