Multiple system Erdheim-Chester disease with massive hypothalamic-sellar involvement and hypopituitarism.

Abstract:

:Erdheim-Chester disease (ECD) is a rare multiple system histiocytosis that is characterized pathologically by xanthogranulomatous infiltrates and radiologically by symmetrical sclerosis of long bones. The diagnosis is often confirmed by biopsy of bone or of orbital or retroperitoneal soft tissue. Intracranial involvement is rare. The authors report a case of ECD in which the diagnosis was made after biopsy of a hypothalamic mass. The mass had been discovered during a workup for panhypopituitarism in a 55-year-old man with urological and bone disease. Aside from diabetes insipidus, other features of pituitary insufficiency have seldom been reported and no patients have presented with a hypothalamic tumor. The endocrinological and neurological aspects of ECD are discussed, as is its differential diagnosis. Reported cases of the disorder associated with hypopituitarism or found during biopsy of central nervous system structures are also reviewed.

journal_name

J Neurosurg

journal_title

Journal of neurosurgery

authors

Oweity T,Scheithauer BW,Ching HS,Lei C,Wong KP

doi

10.3171/jns.2002.96.2.0344

subject

Has Abstract

pub_date

2002-02-01 00:00:00

pages

344-51

issue

2

eissn

0022-3085

issn

1933-0693

journal_volume

96

pub_type

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