[Uncommon case of paraganglioma syndrome in combination with malignant paraganglioma].
BACKGROUND
Combination of paraganglioma syndrome and malignant paraganglioma is an uncommon disease worldwide.


METHODS
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.


CONCLUSIONS
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.
Schmahl
K
K
Abt. XIII, Pathologie, Bundeswehrzentralkrankenhaus Koblenz.
Atamna
N
N
Schönijahn
T
T
Lülsdorf
P
P
Göller
T
T
Jacob
R
R
D000368:Aged; D019943:Amino Acid Substitution / Q000235:genetics; D001216:Asparagine / Q000235:genetics; D002345:Carotid Body Tumor / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D002880:Chromosomes, Human, Pair 11; D003937:Diagnosis, Differential; D004428:Ear Neoplasms / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D007758:Ear, Inner / Q000473:pathology / Q000601:surgery; D004432:Ear, Middle / Q000473:pathology / Q000601:surgery; D006580:Genetic Carrier Screening; D005925:Glomus Jugulare Tumor / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D005998:Glycine / Q000235:genetics; D046088:Hearing Loss, Unilateral / Q000209:etiology*; D006801:Humans; D008207:Lymphatic Metastasis / Q000473:pathology; D008297:Male; D009361:Neoplasm Invasiveness / Q000473:pathology; D009367:Neoplasm Staging; D009378:Neoplasms, Multiple Primary / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D010235:Paraganglioma / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D010579:Petrous Bone / Q000473:pathology / Q000601:surgery; D013385:Succinate Dehydrogenase / Q000235:genetics; D013577:Syndrome; D013964:Thyroid Neoplasms / Q000175:diagnosis / Q000235:genetics* / Q000473:pathology / Q000601:surgery; D014057:Tomography, X-Ray Computed
C491545:SDHD protein, human; D001216:Asparagine; D013385:Succinate Dehydrogenase; D005998:Glycine