PAPILLARY NEOPLASM OF THE ENDOLYMPHATIC SAC IN A PATIENT WITH VON HIPPEL-LINDAU DISEASE

Glandular tumours involving the middle ear and the mastoid are rare, a nd distinguishing between adenoma and adenocarcinoma is difficult. Two distinct histopathological patterns are accepted. While their clinica l presentation differs, both require primary surgical treatment and bo th have a high rate of local recurrence. The papillary form takes a mo re aggressive course and wider regional spread. This pattern occurs pr edominantly in women. Its microscopic appearance and clinical course h ave been extensively described by Heffner who considered it as ''low-g rade adenocarcinoma of probable endolymphatic sac origin''. A few case s have been associated with von Hippel-Lindau disease. The case of a 3 2 year old black woman is described. It is suggested that papillary ad enocarcinoma of the endolymphatic sac should be considered in the spec trum of neoplasms seen in von Hippel-Lindau disease.