Abstract
Eighty-five patients with endocrine pancreatic tumors associated with clinical syndromes of hormone excess were retrospectively analyzed regarding symptomatology, means of diagnosis, and results of surgical and medical treatment during follow-up of 3–18 years (median 8 years). The combination of angiography and computed tomography was most successful in pre-operative localization of both primary tumors and metastases. Surgery provided long term cure in 39 of 44 patients with benign islet cell lesions, the majority having insulinomas. Forty-one patients had malignant tumors, which at the time of diagnosis or operation were associated with liver and/or regional lymph gland metastases in 56% and 24%, respectively. Sixteen patients with metastatic disease and/or very large tumors were considered inoperable, 5 patients underwent palliative resection of their malignant tumors, while grossly radical tumor removal was accomplished in 20 patients. Long-term cure was achieved in 5 patients by excision of primary tumors and localized liver or lymph gland metastases. Half of the patients, particularly those with insulinoma, gastrinoma, or vipoma, showed response to streptozotocin, in combination with other cytostatics, for a median of 24 months or a response to interferon for a median of 10 months. The overall 5-year and 10-year survival among the patients with malignant islet cells tumors was 54% and 28%, respectively. Absence of liver metastases at time of operation/diagnosis, smaller size of the primary tumor, grossly radical tumor resection as well as response to medical therapy predicted the more favorable survival.
Résumé
Les 85 patients atteints de tumeur pancréatique endocrine associée à un syndrome clinique endocrinien ont fait l'objet d'une étude rétrospective portant sur les symptômes, les moyens diagnostiques et les résultats des traitements, chirurgical et médical, au cours d'un suivi allant de 3 à 18 ans (moyenne: 8 ans). L'association d'une angiographie et d'une tomodensitométrie était plus efficace pour localiser en préopératoire à la fois la tumeur primitive et les métastases. La chirurgie a permis la guérison de 40 patients sur 44 porteurs de tumeur bénigne langerhansienne, en majorité un insulinome. Quarante et un patients avaient une tumeur maligne qui, au moment du diagnostic ou lors de l'intervention, s'était compliquée de métastases hépatiques et/ou ganglionnaires locorégionales dans 56 et 24% des cas respectivement. Seize patients présentant une tumeur disséminée et/ou volumineuse ont été considérés comme inopérables; 5 patients ont eu une résection palliative de la tumeur maligne tandis que 20 patients ont pu bénéficier d'une exérèse à peu près radicale de leur tumeur. Une guérison à long terme a même été obtenue chez 5 patients grâce à l'exérèse de la tumeur primitive et des métastases hépatiques et ganglionnaires. La moitié des patients, essentiellement porteurs d'insulinome, de gastrinome ou de vipome ont eu des résponses à la streptozotocine, en association à d'autes cytostatiques pendant une moyenne de 24 mois ou en association à l'interféron pendant une moyenne de 10 mois. La survie globale à 5 et 10 ans chez les patients atteints de tumeur maligne était respectivement de 54 et 28%. L'absence de métastases hépatiques au moment de l'intervention ou du diagnostic, la petite taille de la tumeur primitive, l'exérèse radicale de la tumeur aussi bien que la réponse au traitement médical sont des éléments prédictifs de meilleure survie.
Resumen
Ochenta y cinco pacientes con tumores pancreáticos asociados con síndromes clínicos de exceso hormonal fueron sometidos a un estudio retrospectivo en lo referente a sintomatología, métodos de diagnóstico utilizados y resultados del tratamiento quirúrgioco y médico en el curso de un seguimiento de 3–18 años (media 8 años). La combinación de angiografía y tomografía computadorizada apareció como el método más eficaz para la localización preoperatoria tanto de los tumores primarios como de las metástasis. La cirugía produjo curación a largo plazo en 40/44 pacientes con lesiones benignas de las células insulares, insulinomoas en su mayoría. Cuarenta y un pacientes tenían tumores malignos que en el momento del diagnóstico o de la operación aparecieron asociados con metástasis al hígado y/o los ganglios linfáticos regionales, 56% y 24% respectivamente. Dieciseis pacientes con metástasis y/o tumores enormes fueron considerados inoperables; en 5 se realizó resección paliativa y en 20 se practicó la resección macroscópica radical del tumor. Se obtuvo curación a largo plazo aun en 5 pacientes con enfermedad avanzada mediante la resección de los tumores primarios y de las metástasis hepáticas localizadas o de las metástasis ganglionares. La mitad de los casos, y esencialmente los de insulinoma, gastrinoma o vipoma, exhibieron respuesta bioquímica a la Estreptozotacina en combinación con otros agentes citostásicos, durante un periodo medio de 24 meses, o al interferón durante un periodo medio de 10 meses. La tasa global de sobrevida a 5 y 10 años en los pacientes con tumores malignos de células insulares fueron de 54% y 28% respectivamente. La ausencia de metástasis hepáticas en el momento de la operación/diagnóstico, un tamaño menor del tumor primario, la resección macroscópica radical y la respuesta a la terapia médica, fueron todos factores de predicción de una sobrevida favorable.
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Grama, D., Eriksson, B., Mårtensson, H. et al. Clinical characteristics, treatment and survival in patients with pancreatic tumors causing hormonal syndromes. World J. Surg. 16, 632–639 (1992). https://doi.org/10.1007/BF02067341
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DOI: https://doi.org/10.1007/BF02067341