ABSTRACT
Tumor-related diarrhea is a rare cause of chronic diarrhea in children, which is usually one of the clinical manifestations of specific functional syndrome of neuroendocrine neoplasms.Diarrhea may appear as the initial symptom or accompany symptom of disease progression, and it is generally relieved or cured after surgical resection.The present review mainly introduces several common neuroendocrine neoplasms which cause chronic diarrhea in children, and summarizes their different diarrhea features and mechanisms, as well as key points in diagnosis and treatment, in order to improve the rate of early diagnosis and prognosis of children.
ABSTRACT
OBJETIVO: O propósito do presente estudo é analisar as dificuldades quanto ao diagnóstico, avaliação prognóstica e conduta em sete pacientes portadores de tumores neuroendócrinos do pâncreas (apudomas), estudados na última década, comparando os resultados com aqueles discutidos na literatura. MÉTODO: A idade dos pacientes variou de 15 a 66 anos, com média de 38,4 anos. Todos foram submetidos a alguma forma de ressecção pancreática por tumores neuroendócrinos. Os exames histológicos foram feitos pelas técnicas tradicionais e por imuno-histoquímica. RESULTADOS: Três pacientes tiveram um diagnóstico inespecífico de tumor neuroendócrino; dois de vipoma e dois de gastrinoma. As síndromes não se manifestaram claramente, ainda que cada caso tenha tido um rótulo diagnóstico. Os exames por imuno-histoquímica demonstraram a presença de múltiplos hormônios, mas por falta de sintomas clínicos, as correlações ficaram prejudicadas na maioria dos casos, havendo correlação somente em caso de gastrinoma. Um paciente faleceu no pós-operatório; um sobreviveu sete anos e cinco estão vivos, com sobrevida variando entre três e cinco anos. CONCLUSÕES: Não houve uma manifestação sindrômica evidente, porém a sobrevida dos pacientes tem sido compatível com os dados de literatura.
BACKGROUND: The purpose of the present study is to analyze the difficulties concerning diagnostic, prognostic and clinical conduct of seven patients with pancreatic neuroendocrine tumors (APUDomas), evaluated over the past decade. METHODS: Patients varied in age from 15 to 66 years old, with a mean age of 38.4 years. All patients underwent some type of pancreatic resection as treatment of the neuroendocrine tumor. The histological sections were submitted to traditional and imunohistochemical examination. RESULTS: Three patients were diagnosed with unspecific neuroendocrine tumor, two with VIPoma and two with Gastrinoma. Syndromes were not clearly manifested, although every case had a differential diagnosis. Imunohistochemistry showed the presence of multiple hormones, but due to the lack of clinical symptoms, correlation was harmed in most cases, however one Gastrinoma showed typical clinical symptoms. One patient died in the post-operative period, one survived for 7 years and five are still alive, with a life expectancy varying from 3 to 5 years. CONCLUSIONS: We observed a lack of typical clinical syndromes for most cases, but life expectancy was compatible with the one described in the literature.