[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 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.
Bonnet
S
S
Service de Chirurgie Viscérale et Vasculaire, Hôpital d'Instruction des Armées du Val-de-Grâce, 74, boulevard de Port-Royal, 75230 Paris cedex 05, France. stephanebonnet@caramail.com
Durand
X
X
Baton
O
O
Gimenez-Roqueplo
A-P
AP
Baudin
E
E
Visset
J
J
Algayres
J-P
JP
Baranger
B
B
D000008:Abdominal Neoplasms / Q000235:genetics*; D000328:Adult; D001483:Base Sequence / Q000235:genetics; D005091:Exons / Q000235:genetics; D005260:Female; D006801:Humans; D007506:Iron-Sulfur Proteins / Q000235:genetics; D009154:Mutation / Q000235:genetics; D009852:Omentum / Q000473:pathology; D010235:Paraganglioma / Q000235:genetics*; D010534:Peritoneal Neoplasms / Q000235:genetics; D017384:Sequence Deletion / Q000235:genetics; D013385:Succinate Dehydrogenase / Q000235:genetics
D007506:Iron-Sulfur Proteins; C108095:SDHB protein, human; D013385:Succinate Dehydrogenase