Pheochromocytoma: recommendations for clinical practice from the First International Symposium. October 2005

• The First International Symposium on Pheochromocytoma, held in October 2005, included discussions about developments concerning these rare catecholamine-producing tumors. Recommendations were made during the symposium for biochemical diagnosis, localization, genetics, and treatment. Measurement of plasma or urinary fractionated metanephrines, the most accurate screening approach, was recommended as the first-line test for diagnosis; reference intervals should favor sensitivity over specificity. Localization studies should only follow reasonable clinical evidence of a tumor. Preoperative pharmacologic blockade of circulatory responses to catecholamines is mandatory. Because approximately a quarter of tumors develop secondary to germ-line mutations in any one of five genes, mutation testing should be considered; however, it is not currently cost effective to test every gene in every patient. Consideration of tumor location, presence of multiple tumors, presence of metastases, and type of catecholamine produced is useful in deciding which genes to test. Inadequate methods to distinguish malignant from benign tumors and a lack of effective treatments for malignancy are important problems requiring further resolution.

Nyckelord

Adolescent

Adrenal Gland Neoplasms/*diagnosis/genetics/metabolism/therapy

Adult

Child

Humans

Metanephrine/metabolism

Middle Aged

Mutation

Normetanephrine/metabolism

Paraganglioma/*diagnosis/genetics/metabolism/therapy

Patient Care Planning

Pheochromocytoma/*diagnosis/genetics/metabolism/*therapy

Proto-Oncogene Proteins c-ret/genetics/metabolism

Tumor Markers

Biological/metabolism

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