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Two cases of gastric carcinoids: Diagnostic and therapeutic aspects

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Abstract

Two cases of gastric carcinoid tumors with the foregut carcinoid syndrome are presented. Due to elevated urinary levels of 5-hydroxyindoleacetic acid (5-HIAA), primary tumors of midgut origin were suspected. Provocation with pentagastrin (PG) elicited atypical flushing and severe bronchoconstriction, most probably caused by the release of histamine. Excessive excretion of the main histamine metabolite, tele-methylimidazole acetic acid (MeImAA), in urine of both patients was demonstrated. Both patients had good symptomatic relief with prednisolone and blockade of histamine receptors. One of the patients had excessive secretion of serotonin (5-HT) as well as of histamine. Peripheral blockade of 5-HT2receptors was therefore added to the medical treatment. This patient had a subjective and biochemical response to cytotoxic treatment with streptozotocin and later responded well to a somatostatin analogue.

On clinical suspicion of a foregut carcinoid tumor, or in patients with carcinoid tumors without anatomical localization, PG provocation should not be used until the excretion of MeImAA has been determined.

Résumé

Deux cas de tumeurs carcinoïdes de l'estomac s'accompagnant d'un syndrome carcinoïde sont rapportés par les auteurs. En raison de l'élévation des taux du 5-HIAA (acide 5 hydroxyindolacétique) le diagnostic de tumeurs carcinoïdes d'origine digestive fut envisagé. L'emploi de la pentagastrine (PG) provoqua un flushing atypique et une broncho-constriction intense plus que probablement provoqués par la libération d'histamine. Une excrétion anormalement élevée du métabolite principal de l'histamine, l'acide acétique téléméthylimidazole (MeImAA) dans l'urine des 2 malades fut mise en évidence. Ils bénéficièrent d'une nette amélioration des symptômes qu'ils présentaient grâce à la prednisolone et au blocage des récépteurs histaminiques. Un des 2 malades accusait une sécrétion excessive de sérotonine (5-HT) et d'histamine; le blocage périphérique des récepteurs 5-HT2 fut de ce fait adjoint au traitement. Ce malade répondit favorablement sur le plan clinique et biochimique à un traitement cystotoxique par la streptozotocine et plus tard par un analogue de la somatostatine. Chez les malades qui sont suspects d'être porteurs d'une tumeur carcinoïde de siège indéterminée l'emploi de la pentagastrine doit être écarté jusqu'à ce que l'excrétion de la MeImAA ait été mise en évidence.

Resumen

Se presentan 2 casos de tumores carcinoides gástricos con el síndrome carcinoide del intestino anterior. Los altos niveles urinarios de ácido 5-hidroxiindolacético (5-HIAA) hicieron sospechar la presencia de tumores primarios de origen en el intestino medio. El estímulo con pentagastrina (PG) provocó un “flush” atípico y broncoconstricción severa, muy probablemente causada por liberación de histamina. La excreción excesiva del metabolito principal de la histamina, el ácido tele-metilimidazolacético (MeImAA) fue demostrada en ambos pacientes, y ambos obtuvieron mejoría sintomática con prednisolona y bloqueo de los receptores de histamina. Uno de los pacientes presentó secreción excesiva de serotonina (5-HT) así como de histamina. Por ello se decidió añadir el bloqueo periférico de los receptores 5-HT2 al regimen terapéutico. Este paciente mostró respuesta subjetiva y bioquímica al tratamiento citotóxico con estreptozotocina y ulteriormente respondió bien a un análogo de la somatostatina.

Cuando se sospeche de un tumor carcinoide del intestino anterior, o en presencia de pacientes con tumores carcinoides sin ubicación anatómica, la prueba de estímulo con PG no debe ser utilizada hasta cuando se haya determinado la excreción de MeImAA.

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Authors and Affiliations

  1. Departments of Surgery I, Pathology, and Clinical Physiology, and Institute of Neurobiology, Sahlgren Hospital, University of Göteborg, Göteborg, Sweden

    Håkan Ahlman M.D., Ph.D., Annica Dahlström M.D., Ph.D., Lennart Enerbäck M.D., Ph.D., Göran Granérus M.D., Ph.D., Ola Nilsson M.D., Stellan Persson M.D. & Lars -Erik Tisell M.D., Ph.D.

Authors
  1. Håkan Ahlman M.D., Ph.D.
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  2. Annica Dahlström M.D., Ph.D.
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  3. Lennart Enerbäck M.D., Ph.D.
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  4. Göran Granérus M.D., Ph.D.
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  5. Ola Nilsson M.D.
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  6. Stellan Persson M.D.
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  7. Lars -Erik Tisell M.D., Ph.D.
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Additional information

This work supported by the Swedish Medical Research Council (grant nos. 2235, 2207, 5520, 6534) and the Swedish Life Insurance Company.

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Ahlman, H., Dahlström, A., Enerbäck, L. et al. Two cases of gastric carcinoids: Diagnostic and therapeutic aspects. World J. Surg. 12, 356–360 (1988). https://doi.org/10.1007/BF01655671

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