PAPILLARY ADENOMA OF ENDOLYMPHATIC SAC ORIGIN - A TEMPORAL BONE-TUMORIN VON-HIPPEL-LINDAU-DISEASE - CASE-REPORT

This report describes a patient with von Hippel-Lindau disease who pre sented with an g-year history of a slow-growing, locally invasive vasc ularized lesion of the temporal bone involving the cerebellopontine an gle. The mass, studied by computerized tomography scanning and magneti c resonance imaging techniques, was partly cystic in appearance. After removal of the mass, pathological studies confirmed a papillary cysti c tumor with characteristics that have been described in tumors with a n endolymphatic sac origin. These rare neoplasms constitute a distinct pathological entity and deserve wider recognition.