Tumor e síndrome carcinoide. Relato de caso / Carcinoid tumor and syndrome. Case report

As neoplasias neuroendócrinas são tumores raros, cuja prevalência varia de 0,7 a 4,48 casos em 100 mil habitantes. Menos de um quinto dos pacientes tem a síndrome carcinoide, que pode ser marcada por flushing, diarreia, dor abdominal, alterações cardíacas, pulmonares e pelagra. A dosagem do ácido 5-hidroxi-indolacético urinário e da cromogranina A sérica, exames de imagem e o estudo anatomopatológico da lesão auxiliam no diagnóstico. Neste estudo, relata-se o caso de paciente do sexo masculino, 47 anos, que apresentava diarreia intermitente com evolução de 5 anos e, 2 anos após, dor abdominal e empachamento, bem como percepção de flushing em face, tronco e partes proximais de membros superiores, inicialmente episódico e que, posteriormente, tornou-se fixo, com momentos de exacerbação. Marcadores ácido 5-hidroxi-indolacético urinário e cromogranina A foram positivos. Exame de imagem e estudo anatomopatológico/imuno-histoquímica de lesões focais hepáticas demonstraram tratar-se de tumor neuroendócrino. A cintilografia com octreotide marcado demonstrou lesões hepáticas já conhecidas. Trata-se, portanto, de um tumor neuroendócrino associado à síndrome carcinoide. Foi proposto tratamento com análogo de somatostatina. A síndrome carcinoide é uma manifestação rara dos tumores neuroendócrinos, mas sua identificação precoce é de suma importância para que possa ser oferecido tratamento com intuito curativo e melhor qualidade de vida.

Neuroendocrine tumors are rare. Their prevalence ranges from 0.7 to 4.48 cases per 100,000 inhabitants. Less than 1/5 of the patients have carcinoid syndrome, which can be marked by flushing, diarrhea, abdominal pain, cardiac and pulmonary disorders, pellagra. The measurement of urinary 5-hydroxyindoleacetic acid, the serum chromogranin A, imaging studies, and pathological study of the lesion support the diagnosis. In this study we report the case of a 47-year-old male patient, with five years of intermittent diarrhea and three years of abdominal pain and bloating, as well as perception of flushing in the face, trunk and proximal portions of the upper limbs, initially episodic but that became fixed with moments of exacerbation. The 5-hydroxy-indoleacetic acid and chromogranin A markers were positive. Imaging studies and the histopathological study/immunohistochemistry of the focal hepatic lesions demonstrated that these lesions were neuroendocrine tumors. The marked octreotide scintigraphy showed known liver lesions. It is, therefore, a neuroendocrine tumor associated with carcinoid syndrome. Treatment with a somatostatin analog was proposed. Carcinoid syndrome is a rare manifestation of neuroendocrine tumors, but its early detection is of paramount importance, so that clinicians can offer treatment with curative intent and better quality of life.

Síndrome de carcinoide con manifestación inicial a nivel valvular cardíaco: a raíz de un caso clínico / Carcinoid syndrome presenting as valvular diseas: report of one case

Carcinoid syndrome is observed in one third of carcinoid tumors and usually appears when there are liver metastases. One of the main complications of this syndrome is the appearance of tricuspid or pulmonary valvular disease. We report a 56 years old male presenting with malaise and a weight loss of 10 kg. On physical examination, a heart murmur suspicious of a double tricuspid lesion was found. The echocardiogram was suggestive of a carcinoid valvular disease. The abdominal CAT scan showed a small bowel tumor. Urinary 5-hydroxy-indol- acetic acid values were highly elevated. The patient was subjected to excision of the distal ileum, liver metastasectomy and hemicolectomy. The pathological study of the surgical piece confirmed the diagnosis of carcinoid tumor. Two years after surgery, the patient is in stable conditions.

Síndrome carcinoides / Carcinoid syndrome

A raíz del estudio de un caso clínico, se describen las diarreas neuroendocrinas, señalando su escasa frecuencia; analizando su fisiopatología y detallando sus manifestaciones clínicas. Entre éstas destaca la diarrea que se caracteriza por ser hipersecretora, alta (entérica), liquida, voluminosa (mas de 500 ml) y explosiva. En todos estos cuadros se secretan sustancias hormonales que estimulan la motilidad intestinal y la secreción de agua y de electrolitis por las células de las criptas de Lieberkhun. De todas las diarreas neuro-endocrinas, las mas conocidas son el carcinoides y el vipoma o síndrome Werner-Morrison (pseudocólera pancreático).

Carcinoid crisis during a partial hepatic resection; lack of essential drugs: a cause for concern in the tropics.

We report a patient of carcinoid syndrome with hepatic metastasis (primary lesion in the ileum) who was posted for a right hepatic lobe resection and excision of the primary lesion. The patient developed carcinoid crisis with excessive blood loss due to vasodilation and severe hypotension during surgery. This case highlights the lack of essential drugs in the management of an intraoperative carcinoid crisis in this part of the world.

Carcinoma temprano con epitelio atípico de vesícula biliar (Reporte de un caso con expresión aumentada del P53) / Early gallbladder carcinoma with atypical epitheelium

El gen supresor de tumor P53 se cree que juega un rol importante en la progresión de los tumores malignos a través de la mutación y la expresión aumentada. La inmunohistoquímica de la proteina P53 y el Ki-67 fueron realizados en un carcinoma temprano con epitelio atípico de vesícula biliar. La expresión aumentada de la proteina P53 fue encontrado en el área del adenocarcinoma con una distribución difusa de las células positivas (index:76+/-24 por ciento), y en las áreas de epitelio atípico con focos de células positivas, de distribución difusa (Index:30+/-14 por ciento), mientras el index del Ki-67 fue 6+/- en el área del adenocarcinoma y 6 +/- en el área del epitelio atípico en cada área correspondiente a la medida del P53. Esto sugiere que la expresión aumentada del P53 ocurre en carcinomas tempranos y/o epitelio atípico, al menos en algunos casos y que esto es un evento temprano en el desarrollo del carcinoma de vesícula biliar además de que el epitelio pueda ser parte del carcinoma.

Malignant carcinoid syndrome: failure of anti-oestrogenic therapy

A pre-menopausal patient with a six-year history of symptoms of the metastatic carcinoid syndrome leading to progressively worsening carcinoid heart disease is described. The failure of anti-oestrogenic therapy (sequential bilateral oophorectomy and Tamoxifen therapy) to halt progression of disease was documented. Death resulted from right ventricular failure associated with pulmonary and tricuspid valvular disease

Síndrome de Cassidy-Scholte-Thorson-Björck-Waldestrom ou, simplesmente, síndrome carcinóide: relato de um caso / Cassidy-Scholte-Thorson-Björck-Wasdetrom syndrome: carcinoide syndrome; a case report

Os autores apresentam um caso de síndrome carcinóide com manifestaçöes clínicas típicas, exceto quanto ao broncoespasmo. Exibem a documentaçäo feita por biópsia hepática. Utilizaram o cipro-heptomicina, cuja eficácia foi discreta. Frisam que a literatura médica brasileira é escassa quanto a síndrome carcinóide