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

Ann Chir. 2006 Dec;131(10):626-30. doi: 10.1016/j.anchir.2006.05.004. Epub 2006 Jun 19.
[Article in French]

Abstract

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.

Publication types

  • Case Reports
  • English Abstract

MeSH terms

  • 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

Substances

  • Iron-Sulfur Proteins
  • SDHB protein, human
  • Succinate Dehydrogenase