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Child's Nervous System

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01-05-2015 | Case Report

Fatal juvenile xanthogranuloma presenting as a sellar lesion: case report and literature review

Authors: Sherise D. Ferguson, Steven G. Waguespack, Lauren A. Langford, Joann L. Ater, Ian E. McCutcheon

Published in: Child's Nervous System | Issue 5/2015

Abstract

Introduction

Juvenile xanthogranuloma (JXG) is a histiocytic condition in the spectrum of non-Langerhans histiocytosis that preferentially affects children. Rarely this condition can involve the central nervous system (CNS) with devastating consequences.

Methods

The authors report the unique case of an 11-year-old child who initially presented with a sellar lesion without evidence of the cutaneous stigmata typical of JXG. She was later discovered to have JXG following initial diagnosis of granulomatous hypophysitis, with development of widespread intracranial disease and subsequent neurological deterioration. She underwent subtotal resection of her sellar lesion followed by whole brain radiation and systemic chemotherapy; however, she succumbed to her disseminated disease within 1 month of the JXG diagnosis.

Conclusions

This is a rare case of fatal disseminated intracranial JXG without cutaneous manifestations. Additionally, the initial presentation as a sellar lesion is particularly unusual and seldom described in the literature.

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Title: Fatal juvenile xanthogranuloma presenting as a sellar lesion: case report and literature review
Authors: Sherise D. Ferguson
Steven G. Waguespack
Lauren A. Langford
Joann L. Ater
Ian E. McCutcheon
Publication date: 01-05-2015
Publisher: Springer Berlin Heidelberg
Published in: Child's Nervous System / Issue 5/2015
Print ISSN: 0256-7040
Electronic ISSN: 1433-0350
DOI: https://doi.org/10.1007/s00381-014-2604-3

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Fatal juvenile xanthogranuloma presenting as a sellar lesion: case report and literature review

Abstract

Introduction

Methods

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Introduction

Methods

Conclusions

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