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, seem to have a poor prognosi...

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Bibliographic Details
Published inAnnales de chirurgie Vol. 131; no. 10; p. 626
Main Authors Bonnet, S, Durand, X, Baton, O, Gimenez-Roqueplo, A-P, Baudin, E, Visset, J, Algayres, J-P, Baranger, B
Format Journal Article
LanguageFrench
Published France 01.12.2006
Subjects
Abdominal Neoplasms - genetics
Adult
Base Sequence - genetics
Exons - genetics
Female
Humans
Iron-Sulfur Proteins - genetics
Mutation - genetics
Omentum - pathology
Paraganglioma - genetics
Peritoneal Neoplasms - genetics
Sequence Deletion - genetics
Succinate Dehydrogenase - genetics
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Summary: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, seem 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 paraganglioma diagnosed belatedly and we will consider management problems raised by non-functional forms.
ISSN:0003-3944
DOI:10.1016/j.anchir.2006.05.004