Laryngo- rhino- otologie · 2008
[Uncommon case of paraganglioma syndrome in combination with malignant paraganglioma].
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Schmahl K, Atamna N, Schönijahn T, Lülsdorf P, Göller T, Jacob R
Аннотация
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.
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.
Предметные рубрики MeSH
AgedAmino Acid SubstitutionAsparagineCarotid Body TumorChromosomes, Human, Pair 11Diagnosis, DifferentialEar NeoplasmsEar, InnerEar, MiddleGenetic Carrier ScreeningGlomus Jugulare TumorGlycineHearing Loss, UnilateralHumansLymphatic MetastasisMaleNeoplasm InvasivenessNeoplasm StagingNeoplasms, Multiple PrimaryParagangliomaPetrous BoneSuccinate DehydrogenaseSyndromeThyroid NeoplasmsTomography, X-Ray Computed