[Paragangliomas in the head-/neck region. I: Classification and diagnosis].
Paragangliomas of the head and neck occur sporadically or are hereditary. The hereditary phenotype characteristically occurs between the 2nd and 3rd decade of life; the sporadic phenotype beyond the 4th decade. The hereditary phenotype "paraganglioma syndrome type 1" frequently shows multilocular tumor manifestations and rarely cases of maltransformation. Therefore, paragangliomas should be considered a systemic disease. For diagnosis, an interdisciplinary, step by step procedure is mandatory comprising genetic screening, whole body medical check-up including catecholamine metabolism, and optionally an 18F-Dopa-PET and localised imaging procedures.
Schipper
J
J
Universitätsklinik für Hals-Nasen-Ohren-Heilkunde und Poliklinik des Universitätsklinikum Freiburg. schipper@hno.ukl.uni-freiburg.de
Boedeker
C C
CC
Maier
W
W
Neumann
H P H
HP
D003937:Diagnosis, Differential; D020022:Genetic Predisposition to Disease / Q000235:genetics; D006258:Head and Neck Neoplasms / Q000145:classification* / Q000175:diagnosis* / Q000235:genetics; D006801:Humans; D010235:Paraganglioma / Q000145:classification* / Q000175:diagnosis* / Q000235:genetics; D010346:Patient Care Management / Q000379:methods*; D017410:Practice Guidelines as Topic