[Uncommon case of paraganglioma syndrome in combination with malignant paraganglioma]

Laryngorhinootologie. 2008 Feb;87(2):112-7. doi: 10.1055/s-2007-966892.
[Article in German]

Abstract

Background: Combination of paraganglioma syndrome and malignant paraganglioma is an uncommon disease worldwide.

Patient: We report the case of a 69-year old man with a jugular-tympanal paraganglioma, who underwent surgery. Histopathological examination revealed a malignant paraganglioma. An other contralateral carotid-body and a tumor in the thyroid gland had been discovered during staging. The molecular results confirmed the existence of a paraganglioma syndrome.

Discussion: A malignant paraganglioma based on a hereditary paraganglioma syndrome is a rare described case in literature. On the background of the literature we discuss the pathology, pathogenesis, diagnostic and therapy of this disease.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Amino Acid Substitution / genetics
  • Asparagine / genetics
  • Carotid Body Tumor / diagnosis
  • Carotid Body Tumor / genetics*
  • Carotid Body Tumor / pathology
  • Carotid Body Tumor / surgery
  • Chromosomes, Human, Pair 11
  • Diagnosis, Differential
  • Ear Neoplasms / diagnosis
  • Ear Neoplasms / genetics*
  • Ear Neoplasms / pathology
  • Ear Neoplasms / surgery
  • Ear, Inner / pathology
  • Ear, Inner / surgery
  • Ear, Middle* / pathology
  • Ear, Middle* / surgery
  • Genetic Carrier Screening
  • Glomus Jugulare Tumor / diagnosis
  • Glomus Jugulare Tumor / genetics*
  • Glomus Jugulare Tumor / pathology
  • Glomus Jugulare Tumor / surgery
  • Glycine / genetics
  • Hearing Loss, Unilateral / etiology*
  • Humans
  • Lymphatic Metastasis / pathology
  • Male
  • Neoplasm Invasiveness / pathology
  • Neoplasm Staging
  • Neoplasms, Multiple Primary / diagnosis
  • Neoplasms, Multiple Primary / genetics*
  • Neoplasms, Multiple Primary / pathology
  • Neoplasms, Multiple Primary / surgery
  • Paraganglioma / diagnosis
  • Paraganglioma / genetics*
  • Paraganglioma / pathology
  • Paraganglioma / surgery
  • Petrous Bone / pathology
  • Petrous Bone / surgery
  • Succinate Dehydrogenase / genetics
  • Syndrome
  • Thyroid Neoplasms / diagnosis
  • Thyroid Neoplasms / genetics*
  • Thyroid Neoplasms / pathology
  • Thyroid Neoplasms / surgery
  • Tomography, X-Ray Computed

Substances

  • SDHD protein, human
  • Asparagine
  • Succinate Dehydrogenase
  • Glycine