Atormac
Neurology India
menu-bar5 Open access journal indexed with Index Medicus
  Users online: 1060  
 Home | Login 
About Editorial board Articlesmenu-bullet NSI Publicationsmenu-bullet Search Instructions Online Submission Subscribe Videos Etcetera Contact
  Navigate Here 
 Search
 
  » Next article
  » Previous article 
  » Table of Contents
  
 Resource Links
  »  Similar in PUBMED
 »  Search Pubmed for
 »  Search in Google Scholar for
  »  Article in PDF (104 KB)
  »  Citation Manager
  »  Access Statistics
  »  Reader Comments
  »  Email Alert *
  »  Add to My List *
* Registration required (free)  

 
  In this Article
 »  References
 »  Article Figures

 Article Access Statistics
    Viewed6612    
    Printed97    
    Emailed0    
    PDF Downloaded1116    
    Comments [Add]    
    Cited by others 3    

Recommend this journal

 


 
NEUROIMAGE
Year : 2007  |  Volume : 55  |  Issue : 1  |  Page : 91-92

Langerhans cell histiocytosis of pituitary stalk


Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi - 110 029, India

Date of Acceptance03-Sep-2006

Correspondence Address:
Subramanian Subramanian
Department of Radiodiagnosis, All India Institute of Medical Sciences, New Delhi - 110 029
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.30447

Rights and Permissions



How to cite this article:
Kandpal H, Subramanian S, Hari S. Langerhans cell histiocytosis of pituitary stalk. Neurol India 2007;55:91-2

How to cite this URL:
Kandpal H, Subramanian S, Hari S. Langerhans cell histiocytosis of pituitary stalk. Neurol India [serial online] 2007 [cited 2019 Aug 23];55:91-2. Available from: http://www.neurologyindia.com/text.asp?2007/55/1/91/30447


A four-year-old male child presented with increased urine output and excessive thirst for eight months. On examination he had multiple scalp swellings with mild hepatosplenomegaly. Urine specific gravity was decreased suggestive of diabetes insipidus. Skull radiograph revealed multiple well-defined geographic lytic lesions in the calvarium with bevelled margins [Figure - 1]. On MR examination of sella, the posterior pituitary bright spot was not seen on T1W image [Figure - 2]. The pituitary stalk was thickened and showed uniform enhancement [Figure - 3]. The characteristic radiographic feature of skull lesions, MRI findings and hepatosplenomegaly strongly suggested the possibility of Langerhans cell histiocytosis (LCH) which was confirmed on bone marrow biopsy.

LCH is a wide-spectrum disease characterized by proliferation of abnormal histiocytes. It encompasses three classical clinical syndromes which in increasing order of severity are: eosinophilic granuloma (solitary bone lesion), Hand-Schuller-Christian disease (skull lesion, exophthalmos and diabetes insipidus) and Letterer-Siwe disease (multisystemic rapidly progressive form). The skull bones are most commonly involved. Typically the lesions are round or oval-shaped with well-defined margins and bevelled edge. Individual lesions may coalesce giving a geographic appearance to the skull vault. A button sequestrum may be seen within them. Diabetes insipidus although rare at initial presentation may occur in 25% patients during the course of the disease.[1] Cranial MRI findings in LCH include thickening and abnormal enhancement of the pituitary stalk with absence of posterior pituitary hyperintensity.[1],[2] Infundibular involvement may be diffuse or focal[1] and may precede other typical manifestations of LCH.[3],[4] Although germinoma, neurosarcoid, tuberculosis and occasionally idiopathic diabetes insipidus can cause pituitary stalk thickening, clinical and radiological features usually suggest the diagnosis of LCH, which can be confirmed on biopsy from lesions in the bone, bone marrow, lymph node or skin lesions.

 
 » References Top

1.Maghnie M, Arico M, Villa A, Genovese E, Beluffi G, Severi F. MR of the hypothalamic-pituitary axis in Langerhans cell histiocytosis. AJNR Am J Neuroradiol 1992;13:1365-71.  Back to cited text no. 1  [PUBMED]  
2.Tien RD, Newton TH, McDermott MW, Dillon WP, Kucharczyk J. Thickened pituitary stalk on MR images in patients with diabetes insipidus and Langerhans cell histiocytosis. AJNR Am J Neuroradiol 1990;11:703-8.  Back to cited text no. 2  [PUBMED]  
3.Schmitt S, Wichmann W, Zachmann M Schoenle EJ. Pituitary stalk thickening with diabetes insipidus preceding typical manifestations of Langerhans cell histiocytosis in children. Eur J Pediatr 1993;152:399-401.  Back to cited text no. 3    
4.Halefoglu AM. Magnetic resonance imaging of thickened pituitary stalk proceeding to Langerhans cell histiocytosis in a child. Australas Radiol 2006;50:175-8.  Back to cited text no. 4  [PUBMED]  [FULLTEXT]


    Figures

[Figure - 1], [Figure - 2], [Figure - 3]

This article has been cited by
1 Endocrine manifestations of Langerhans cell histiocytosis diagnosed in adults
M. S. García Gallo, M. P. Martínez, M. S. Abalovich, S. Gutiérrez, M. A. Guitelman
Pituitary. 2010; 13(4): 298
[VIEW] | [DOI]
2 Different courses of recurrent or multisystem Langerhans cells histiocytosis in adults - Description of 22 cases from one centre | [Odlišné průběhy recidivující anebo multisystémové formy histiocytózy z Langerhansových buněk u dospělých osob - Popis 22 pří padů z jednoho pracoviště]
Adam, Z., Krejčí, M., Pour, L., Szturz, P., Neubauer, J., Nebeský, T., Řehák, Z., (...), Vorlíček, J.
Vnitrni Lekarstvi. 2010; 56(6): 542-556
[Pubmed]
3 Langerhans cell histiocytosis in adult patients - A disease with many faces experience of a centre and an overview of the disease symptoms | [Histiocytóza z langerhansových buněk u osob dospělého věku - Nemoc s mnoha tvářemi. Zkušenosti jednoho pracoviště a přehled projevů nemoci]
Adam, Z., Pour, L., Krejčí, M., Neubauer, J., Vaníček, J., Vašků, V., Hájek, R.
Vnitrni Lekarstvi. 2008; 54(11): 1063-1080
[Pubmed]



 

Top
Print this article  Email this article
Previous article Next article
Online since 20th March '04
Published by Wolters Kluwer - Medknow