[논문]Progrès récents dans le diagnostic, l’évaluation pronostique et le traitement des phéochromocytomes

Plouin, P.F; Gimenez-Roqueplo, A.P; La; Batide; Alanore, A; Salenave, S; Duclos, J.M

doi:10.1016/s0248-8663(00)00268-x

[해외논문] Progrès récents dans le diagnostic, l’évaluation pronostique et le traitement des phéochromocytomes
Evolving concepts in the diagnosis, prognosis, and treatment of pheochromocytoma.

La Revue de médecine interne, v.21 no.12, 2000년, pp.1075 - 1085

Plouin, P.F (Service d’hypertension arté) , Gimenez-Roqueplo, A.P (rielle, hô) , La Batide Alanore, A (pital europé) , Salenave, S (en Georges-Pompidou, 20-40, rue Leblanc, 75908 Paris cedex ) , Duclos, J.M (15, France)

Abstract ▼ AI-Helper

AbstractIntroduction. – Pheochromocytoma is a catecholamine-secreting neoplasm of chromaffin tissue. It is a rare disease. Biochemical tests should be performed only in patients at high risk of pheochromocytoma, and an imaging procedure only in those with positive biochemical tests.Current knowledge and key points. – The most specific and sensitive diagnostic test for the disease is the determination of plasma or urinary metanephrines. The tumor can be located by computerized tomography, magnetic resonance imaging, and specific scintigraphy. Ten to 20% of pheochromocytomas result from hereditary diseases, including multiple endocrine neoplasia type 2, von Hippel Lindau disease, and neurofibromatosis 1. Familial cases are diagnosed earlier, and are more frequently bilateral and recurring than sporadic cases. About 10% of the cases are malignant either at first operation or during follow-up, malignancy being diagnosed by the presence of lymph node, visceral or bone metastases. The probability of a recurrence is positively correlated with the urinary excretion of metanephrines and tumor size. Recurrences are more frequent in cases with ectopic tumors and in those with a low plasma epinephrine to total catecholamine ratio. Patients, especially those with familial tumors or low epinephrine secretion, should be followed-up indefinitely.Future prospects and projects. – Treatment for malignant recurrences includes surgery, therapeutic embolization, chemotherapy, and the application of therapeutic doses of metaiodobenzylguanidine. Metyrosine, phenoxybenzamine, or somatostatin analogs may help to control blood pressure and to alleviate symptoms in patients with malignant pheochromocytoma; however such a treatment has no antiproliferative effect.

Abstract

RésuméIntroduction. – Les phéochromocytomes sont des tumeurs sécrétant des catécholamines. Leur rareté requiert une démarche diagnostique par étapes : dépistage clinique, preuve biologique, localisation tumorale. Environ 10 % des cas sont ectopiques, 10 % familiaux, et 10 % d’évolution maligne. Les risques évolutifs dans les cas familiaux ou à risque de malignité justifient une enquête génétique, la recherche d’indicateurs pronostiques et une surveillance indéfinie.Actualités et points forts. – Le test diagnostique le plus sensible est la mesure des métanéphrines plasmatiques ou urinaires. La localisation tumorale repose sur la scanographie, l’imagerie par résonance magnétique et les scintigraphies spécifiques. Les signes phénotypiques et les preuves génomiques de l’un des trois syndromes autosomiques dominants associés au phéochromocytome doivent être recherchés dans tous les cas. Ces cas familiaux sont souvent précoces, bilatéraux et récidivants. Les phéochromocytomes peuvent être malins d’emblée ou lors d’une récidive. Les métastases sont ganglionnaires, osseuses ou viscérales. Le risque de récidive croît avec la taille de la tumeur et le débit urinaire des métanéphrines. Il est plus élevé dans les phéochromocytomes ectopiques ou ceux dont la sécrétion est immature. Ce risque justifie la surveillance clinique et biologique au moins biennale de tous les patients opérés d’un phéochromocytome.Perspectives et projets. – L’étude des mécanismes de la tumorogenèse et de l’hypersécrétion du phéochromocytome est en rapide progrès. Des progrès sont également attendus dans le traitement des cas malins : chirurgie, embolisation des métastases viscérales, plastie percutanée des métastases osseuses, radiothérapie in situ par la méta-iodobenzylguanidine iodée, traitement antisécrétoire par la métyrosine ou les analogues de la somatostatine.

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