Malignant hereditary paraganglioma: problems raised by non-functional forms management

Non-functional paraganglioma have not clinical or biological characteristics, so that the diagnostic is, most of the time delayed and. made on the occasion of advanced abdominal tumor or symptomatic metastasis management. Hereditary forms, notably those with SDHB mutation, se em to have a poor prognosis. On the other hand, and on the oposite to sporadic forms, they are the only ones to benefit from genetic testing which make possible, if positive, an earlier diagnostic, before apparition of symptoms, recurrence or metastasis. We report a case of non-functional malignant hereditary paragangliom'a diagnosed belatedly and we will consider management problems raised by non-functional forms. (c) 2006 Elsevier Masson SAS. Tous droits reserves.