Carcinoid syndrome: diagnosis and medical management

Abstract

Gastro-intestinal carcinoids are slow growing tumors arising from enterochromaffin or Kulchitsky cells. Their clinical presentation depends on what combination of bioactive substances is secreted. Midgut carcinoid can present with the carcinoid syndrome in the presence of liver metastases. Its most typical clinical manifestations include cutaneous flushing and diarrhea. A nonspecific biochemical tumor marker for carcinoid tumors is serum chromogranin A and a specific marker for the carcinoid syndrome is the increased urinary excretion of 5-hydroxy indole acetic acid (5-HIAA). Localizing studies in carcinoid tumors/syndrome are: transabdominal ultrasonography (US), endoscopy, endoscopic US, videocapsule endoscopy, computerized tomography, magnetic resonance imaging, selective abdominal angiography, 111 In-pentetreotide scintigraphy (and intraoperative radionuclide probe), 1 2 3 I ( 1 3 1 I ) -metaiodobenzylguanidine (MIBG) scintigraphy, bone scintigraphy and 11 C-5-HT positron emission tomography (PET). Therapies for carcinoid tumors/syndrome are: surgery, somatostatin analogs, interferon-alpha, radiotherapy, liver dearterialization, liver (chemo, or radio)-embolization, alcohol sclerotherapy of liver metastases, radiofrequency ablation of liver metastases, cryosurgery of liver metastases, occasionally liver transplantation, radiotherapy-coupled somatostatin analogs, 131 I-MIBG and occasionally chemotherapy. Carcinóides gastro-intestinais são tumores de crescimento lento originários das células enterocromafínicas ou de Kulchitsky. Sua apresentação clínica depende das combinações de substâncias bioativas que são secretadas. Carcinóides de intestino delgado podem se apresentar com síndrome carcinóide na presença de metástases hepáticas. A manifestação clínica típica inclui flushing cutâneo e diarréia. A cromogranina-A é um marcador bioquímico tumoral inespecífico de tumores carcinóides e o aumento da excreção urinária de ácido 5-hidroxiindolacético (5-HIAA), um marcador específico para a síndrome carcinóide. Estudos de localização nos tumores/síndrome carcinóide são: ultrassonografia (US) abdominal, endoscopia, US endoscópica, endoscopia com vídeo-cápsula, tomografia computadorizada, ressonância magnética, angiografia abdominal seletiva, cintilografia com 111 In-pentetreotide (e sonda radioisotópica intraoperatória), cintilografia com 123 I ( 131 I) -metaiodobenzilguanidina (MIBG), cintilografia óssea e tomografia por emissão de pósitron ( 11 C-5-HT). Tratamento para tumores/síndrome carcinóide são: cirurgia, análogos de somatostatina, interferon-alfa, radioterapia, embolização arterial hepática, quimioembolização hepática, escleroterapia alcoólica de metástases hepáticas (MH), ablação por radiofreqüência de MH, criocirurgia de

Carcinoid tumor arising in a horseshoe kidney

“…Carcinoid syndrome is rarely associated with primary renal carcinoid tumor, even in case of metastatic disease [1,4,7]. The classical Carcinoid syndrome occurs in less than 10% of patients with gastrointestinal carcinoid and in less than 5% of patients with pulmonary carcinoid [8,9]. This is characterized by cutaneous flushing most commonly on the face, neck and upper chest, and diarrhea in almost 75% of cases.…”

Rarity and review: evidence-based practice for uncommon diseases

“…4 Carcinoid tumours are capable of secreting a variety of vasoactive substances, including serotonin, histamine, and kinin peptides, although only 25% do this, 5 and only 10% result in carcinoid syndrome. 6 Characterized by flushing, diarrhea, abdominal pain, and bronchoconstriction, carcinoid syndrome occurs when the tumour's metabolic products escape hepatic metabolism by either metastasis or primary locations outside the portal circulation. 6 Octreotide, a somatostatin analogue that inhibits secretion of hormones in functioning neuroendocrine tumours, reduces symptoms among those suffering from carcinoid syndrome.…”

“…6 Characterized by flushing, diarrhea, abdominal pain, and bronchoconstriction, carcinoid syndrome occurs when the tumour's metabolic products escape hepatic metabolism by either metastasis or primary locations outside the portal circulation. 6 Octreotide, a somatostatin analogue that inhibits secretion of hormones in functioning neuroendocrine tumours, reduces symptoms among those suffering from carcinoid syndrome. A life-threatening carcinoid crisis characterized by hemodynamic instability, arrhythmias, bronchospasm, and coma 7 may result when tumours are physically or chemically stimulated to release massive quantities of vasoactive substances.…”

“…1 Plusieurs systèmes de classification tentent de décrire ces diverses maladies, 2,3 mais les discussions traitant exclusivement des carcinoïdes mettent souvent l'accent sur l'origine embryologique du tissu parent : l'intestin céphalique (thymus, oesophage, poumon, estomac, duodénum, pancréas), l'intestin moyen (appendice, iléon, caecum, côlon ascendant), et l'intestin postérieur (gros intestin distal, rectum) 4 Bien que seulement 25 % des carcinoïdes le fassent, ces tumeurs peuvent sécréter toutes sortes de substances vasoactives, y compris de la sérotonine, de l'histamine, et des peptides de kinine; en outre, seulement 10 % deviennent un syndrome carcinoïde. 6 Le syndrome carcinoïde est caractérisé par des bouffées vasomotrices, des diarrhées, des douleurs abdominales et de la bronchoconstriction; il survient lorsque les produits métaboliques de la tumeur s'échappent du métabolisme hépatique par métastase ou via des emplacements primaires hors de la circulation porte. 6 L'octréotide, un analogue de la somatostatine qui inhibe la sécrétion hormonale des tumeurs neuroendocriniennes fonctionnelles, réduit les symptômes des personnes souffrant de syndrome carcinoïde.…”

“…6 Le syndrome carcinoïde est caractérisé par des bouffées vasomotrices, des diarrhées, des douleurs abdominales et de la bronchoconstriction; il survient lorsque les produits métaboliques de la tumeur s'échappent du métabolisme hépatique par métastase ou via des emplacements primaires hors de la circulation porte. 6 L'octréotide, un analogue de la somatostatine qui inhibe la sécrétion hormonale des tumeurs neuroendocriniennes fonctionnelles, réduit les symptômes des personnes souffrant de syndrome carcinoïde. Une crise carcinoïde potentiellement fatale, caractérisée par une instabilité hémodynamique, des arythmies, un bronchospasme et un coma, peut survenir lorsque les tumeurs sont stimulées physiquement ou chimiquement de façon à libérer des quantités importantes de substances vasoactives.…”