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, and distinguishing between adenoma and adenocarcinoma is difficult. Two distinct histopathological patterns are accepted. While their clinical presentation differs, both require primary surgical treatment and both have a high rate...
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Published in | Journal of clinical pathology Vol. 47; no. 10; pp. 959 - 961 |
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Main Authors | , , , , |
Format | Journal Article |
Language | English |
Published |
London
BMJ Publishing Group Ltd and Association of Clinical Pathologists
01.10.1994
BMJ BMJ Publishing Group LTD |
Subjects | |
Online Access | Get full text |
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Summary: | Glandular tumours involving the middle ear and the mastoid are rare, and distinguishing between adenoma and adenocarcinoma is difficult. Two distinct histopathological patterns are accepted. While their clinical presentation differs, both require primary surgical treatment and both have a high rate of local recurrence. The papillary form takes a more aggressive course and wider regional spread. This pattern occurs predominantly in women. Its microscopic appearance and clinical course have been extensively described by Heffner who considered it as "low-grade adenocarcinoma of probable endolymphatic sac origin". A few cases have been associated with von Hippel-Lindau disease. The case of a 32 year old black woman is described. It is suggested that papillary adenocarcinoma of the endolymphatic sac should be considered in the spectrum of neoplasms seen in von Hippel-Lindau disease. |
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Bibliography: | local:jclinpath;47/10/959 istex:AED846CAE0812549039327660A523E07038B0BE3 href:jclinpath-47-959.pdf PMID:7962613 ark:/67375/NVC-4TGGGRW3-W ObjectType-Case Study-2 SourceType-Scholarly Journals-1 ObjectType-Feature-4 content type line 23 ObjectType-Report-1 ObjectType-Article-3 |
ISSN: | 0021-9746 1472-4146 |
DOI: | 10.1136/jcp.47.10.959 |