Abstract
The etiology, prognosis, and optimal management of primary gastric carcinoids remain controversial. Records of 36 consecutive patients with gastric carcinoid (15 men) were reviewed retrospectively between 1975 and 1990. Follow-up was complete in 97% of cases. Mean age at diagnosis was 58.4 years (range 24–82 years). The clinical presentations included anemia (72%), pain (69%), and carcinoid syndrome (11%). Associated autoimmune and endocrine abnormalities were common and included atrophic gastritis (67%), pernicious anemia (58%), hypothyroidism (39%), diabetes (19%), Addison's disease (6%), and hyperparathyroidism (6%). Lesions were nonantral in 78%, involving only the corpus in 42%, the fundus in 28%, and only the antrum in 8%; 42% were multiple. Urinary 5-hydroxyindoleacetic acid (5-HIAA) and serum gastrin levels were elevated in 17% and 50% of those tested, respectively. Histologic examination revealed that 28% of lesions were ≥2 cm, and 33% had liver metastases on presentation or developed them during follow-up. Eight patients (22%) died of tumor with a median survival of 39 months. The presence of metastases, atypical histology, serosal involvement, and size > 2 cm were adverse prognostic factors. In patients without hypergastrinemia (n=6), 66% developed metastases, 60% had elevated 5-HIAA, and 50% died of carcinoid tumor. In sharp contrast, those patients with hypergastrinemia and “typical” gastric carcinoids (n=15), metastases and death did not occur (p<0.003 and p < 0.005, respectively, compared with eugastrinemic patients). In the latter group (hypergastrinemia and typical histology), pernicious anemia was common (73%); solitary lesions and serosal penetration were seen comparatively less often (33% versus 100%, p<0.005 and 0% versus 33%, p<0.05, respectively); and no lesion measured > 2.0 cm compared to 66% in the group without elevated gastrin (p<0.002). No patient with hypergastrinaemia had elevated levels of 5-HIAA. Overall survival at 5 and 10 years was 58% and 28%, respectively. Survival was 80% at 5 and 10 years in the hypergastrinemic group. Gastric carcinoid tumors that are histologically atypical, large (>2 cm), or present without elevated gastrin are potentially lethal and require aggressive therapy. In contrast, gastric carcinoids with typical histology and hypergastrinemia are rarely lethal, and conservative management with local excision and close observation is appropriate.
Résumé
L'étiologie, le pronostic et la thérapeutique optimale du traitement des tumeurs carcinoïdes de l'estomac restent discutés. Les dossiers de 36 patients consécutifs porteurs de tumeurs carcinoïdes (15 hommes), traités entre 1975 et 1990, ont été revus. Le suivi a été complet chez 97% des cas. L'âge moyen au moment du diagnostic était de 58.4 ans (extrêmes 24–82 ans). Les signes révélateurs on été l'anémie (72%), la douleur (69%) et le syndrome carcinoïde (11%). Les anomalies auto-immunes et endocrines associées étaient la gastrite atrophique (67%), l'anémie pernicieuse (58%), l'hypothyroïdie (39%), le diabète (19%), la maladie d'Addison (6%) et l'hyperparathyroïdie (6%). Les lésions intéressaient le corps seul de l'estomac dans 42% des cas, le fundus dans 28% des cas et l'antre seul dans 8% des cas. Dans 42% des cas, les lésions étaient multiples. Les taux de 5-hydroxyindole-acide acétique (5-HIAA) et de gastrine sériques étaient élevés chez, respectivement, 17% et 50% des patients testés. L'examen histologique a révélé que 28% des lésions mesuraient 2 cm ou plus et 33% des patients avaient des métastases hépatiques lors de la première consultation ou en ont développé par la suite. Vingt-deux pourcent des patients (n=8) sont décédés de leur tumeur; la médiane de survie étant de 39 mois. La présence de métastases, l'atypie histologique, l'envahissement de la séreuse, la taille > 2 cm étaient des facteurs de mauvais pronostic. Chez les patients sans hypergastrinémie (n=6), 66% ont développé des métastases, 60% avaient une élévation des 5-HIAA, et 50% sont décédés de leur tumeur carcinoïde. En revanche, chez les patients qui avaient une hypergastrinémie et ceux qui avaient une tumeur carcinoïde gastrique “typique” (n=15), il n'a pas été observé ni de métastase, ni de mortalité (respectivement p<0.003 et p< 0.005 par rapport aux tumeurs eugastrinémiques). Dans ce dernier groupe (hypergastrinémie et histologie typique), l'anémie pernicieuse était fréquente (73%). On a vu moins de lésions solitaires et d'envahissement de la séreuse (33% vs 100%, p< 0.005 et 0% vs 33% p<0.05). Il n'y avait aucune lésion dont le diamétre dépassait 2 cm dans ce groupe comparé à 66% dans le groupe sans hypergastrinémie (p<0.002). Aucun patient avec une hypergastrinémie n'avait de taux élevés de 5-HIAA.
Resumen
La etiología, pronóstico y óptima modalidad de manejo de los carcinoides gástricos primarios siguen siendo motivo de controversia. Se revisaron en forma retrospectiva las historias de 36 pacientes consecutivos con carcinoide gástrico (15 hombres) en el período entre 1975 y 1990. Se logró seguimiento completo en 97% de los casos.
La edad media en el momento del diagnóstico fue 58.4 años trango 24–28. Los síntomas de presentación fueron: anemia (72%), dolor (69%) y síndrome carcinoide (11%). Fueron comunes las anormalidades asociadas de tipo autoinmune y de tipo endocrino, que ìncluyeron gastritis atrófica (67%), anemia perniciosa (58%), hipotiroidismo (39%), diabetes (19%), enfermedad de Addison (6%) e hiperparatiroidismo (6%).
Las lesiones fueron no antrales en el 78% de total, con 42% ubicadas en el cuerpo, 28% en el fundus y 8% afectando el antro sólamente; 42% fueron múltiples. Los niveles urinarios de ácido 5-hidroxiindoloacético (5-HIAA) y los niveles séricos de gastrina aparecieron elevados en 17% y 50% de los pacientes examinados, respectivamente. El examen histológico reveló que 28% de las lesiones eran de 2 cm o mayores, y 33% presentaban metástasis hepáticas en el momento del diagnóstico o las desarrollaron en el curso del seguimiento.
Veintidos por ciento de los pacientes (n=8) murieron por causa del tumor, con una sobrevida media de 39 meses. La presencia de metástasis, la histología atípica, la extensión a la serosa y el tamaño > 2 cm demostraron ser factores adversos de pronósticos. Entre los pacientes con hipergastrinemia (n=6), 66% desarrollaron metástasis, 60% presentaron 5-HIAA elevado y 50% murieron por el tumor carcinoide. En contraste marcado, aquellos pacientes con hipergastrinemia y carcinoides “típicos” (n=15), no se presentaron metástasis ni muertes (p <0.003 y p<0.005 respectivamente), en comparación con los pacientes eugastrinémicos.
En este último grupo (hipergastrinemia e histología típica) fue común la anemia perníciosa (73%); las lesiones solitarias y la penetración a la serosa fueron observadas con frecuencà relativamente menor (33% vs 100%, p<0.005 y 0% vs 33%, p <0.05, respectivamente), y ninguna lesión midió > 2.0 cm, en comparación con 66% en el grupo que no exhibió elevación de gastrina (p<0.002). Ningún paciente con hipergastrinemia presentó niveles elevados de 5-HIAA.
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Gough, D.B., Thompson, G.B., Crotty, T.B. et al. Diverse clinical and pathologic features of gastric carcinoid and the relevance of hypergastrinemia. World J. Surg. 18, 473–479 (1994). https://doi.org/10.1007/BF00353739
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DOI: https://doi.org/10.1007/BF00353739