[Uncommon case of paraganglioma syndrome in combination with malignant paraganglioma]. / Seltener fall eines paragangliom-syndroms mit auftreten eines malignen paraganglioms.

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.