Atormac
Neurology India
menu-bar5 Open access journal indexed with Index Medicus
  Users online: 679  
 Home | Login 
About Editorial board Articlesmenu-bullet NSI Publicationsmenu-bullet Search Instructions Online Submission Subscribe Videos Etcetera Contact
  Navigate Here 
 Search
 
  
 Resource Links
    Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
    Article in PDF (1,590 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
    Viewed1647    
    Printed58    
    Emailed0    
    PDF Downloaded48    
    Comments [Add]    
    Cited by others 1    

Recommend this journal

 


 
Table of Contents    
LETTER TO EDITOR
Year : 2012  |  Volume : 60  |  Issue : 5  |  Page : 534-536

Ossified rathke's cleft cyst: A rare variant


1 Department of Neurosurgery, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh-500082, India
2 Department of Pathology, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh-500082, India

Date of Web Publication3-Nov-2012

Correspondence Address:
Mudumba Vijayasaradhi
Department of Neurosurgery, Nizam's Institute of Medical Sciences, Hyderabad, Andhra Pradesh-500082
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.103214

Rights and Permissions



How to cite this article:
Vijayasaradhi M, Prasad VB, Uppin MS, Sundaram C. Ossified rathke's cleft cyst: A rare variant. Neurol India 2012;60:534-6

How to cite this URL:
Vijayasaradhi M, Prasad VB, Uppin MS, Sundaram C. Ossified rathke's cleft cyst: A rare variant. Neurol India [serial online] 2012 [cited 2020 Feb 18];60:534-6. Available from: http://www.neurologyindia.com/text.asp?2012/60/5/534/103214


Sir,

Rathke's cleft cysts (RCC) are predominantly sellar-suprasellar lesions thought to arise from remnants of embryologic Rathke's pouch during fourth week of development. The presence or the absence of calcification is an important feature in distinguishing them from other commonly encountered cystic lesions in the sellar-suprasellar region. [1] Craniopharyngiomas very commonly show calcifications and the presence of the same is uncommon in Rathke's cleft cyst. The case presented here is one of such uncommon variants.

A 45-year-old female presented with secondary amenorrhoea and infertility of 10 years duration with 6 months history of onset of headaches and visual disturbances. Examination of visual fields showed bi- temporal hemianopia. Evaluation of serum hormones showed low levels of follicle stimulating hormone (FSH) (1.48 mIU/ml; normal 3-10 mIU/ml) and luteinizing hormone (LH) (0.23 mIU/ml; normal 3-10 mIU on day 3) and normal levels of prolactin, growth hormone, cortisol (8 am value), and T3, T4, and thyroid stimulating hormone (TSH). Magnetic resonance imaging (MRI) revealed a well-defined oval (2.4 x 2.4 x 2.7 cm) T1- weighted isointense and T2-weighted hyperintense lesion with a surrounding faint rim of hypointensity in sellar region extending into the suprasellar compartment and compressing the optic chiasm and displacing it superiorly. The contrast study showed peripheral rim of enhancement [Figure 1]. Complete surgical excision of the lesion was performed by a right pterional craniotomy and trans-sylvian approach. Peroperatively the lesion was cystic and contained about 10 ml of xanthochromic fluid and was surrounded circumferentially by an unusual egg shelllike calcified hard layer which was removed near totally [Figure 2]. Histopathology of the cyst wall showed flattened to columnar epithelial lining of the cyst wall with focal stratification and keratinisation was not seen. Sub-epithelial tissue showed ossification [Figure 3]. Immunohistochemistry with pancytokeratin was positive in the lining of the cyst wall suggestive of RCC [Figure 4].
Figure 1: Post contrast sagital MR T1WI showing the rim like peripheral contrast enhancement

Click here to view
Figure 2: Photograph of the ossified cyst wall

Click here to view
Figure 3: High power view of cyst wall (a) The cyst wall lining showing fl attened epithelium (H and E, ×40), (b) Focal stratifi cation of lining epithelium (H and E, ×200), (c) High power view of cyst wall H and E stain showing mature osteocytes in bone matrix

Click here to view
Figure 4: Immunohistochemistry showing positivity for pancytokeratin

Click here to view


Clinically, RCCs are mostly asymptomatic and occasionally may present with varied symptoms like visual disturbances, headache, and amenorrhea/galactorrhoea due to pressure effects on the adjacent neural structures. On histology, RCC's usually have a lining by columnar or cuboidal epithelium with stratification, cilia, or goblet cells. They might sometimes contain gland like structures in the sub-epithelial layer. In suprasellar examples, the cyst wall may show squamous epithelium. [1] Histology in our patient showed flattened to ciliated epithelium with no keratinization thereby differentiating it from craniopharyngioma and other epithelial cysts in the sellar-suprasellar region. The expression of cytokeratins 8 and 20 was positive in RCC but not in craniopharyngioma. [2] The contents of the cyst were xanthochromic indicating old hemorrhage. The calcification and ossification seen in the cyst wall could be a consequence of old hemorrhage or caused by osteoid metaplasia in mesenchymal cells by inflammatory, chemical, or mechanical stimulation causing metaplastic ossification of the wall. [3]

In a study of 11 symptomatic patients by Rao and colleagues, 8 patients initially had visual symptoms. [4] In another study of 19 patients by Eguchi and co-authors, visual symptoms occurred in 47% of patients, 4 patients had diabetes insipidus, 3 patients had amenorrhea and/or galactorrhea, and 2 of the 19 patients had panhypopituitarism. [5] In the study by Voelker and colleagues, [6] the most common finding was pituitary hypofunction with multiple endocrinopathies. Younger patients, aged 4-22 years, had evidence of hypopituitarism from an early age, with resultant growth retardation. The second most common symptom was visual disturbance, which included visual field defects resulting from chiasmatic compression. The next most common symptom was headache, of which 57% were frontal. Shin and co- authors described impotence or low libido as the most common endocrine abnormality in men; in women the most common manifestation was hyperprolactinemia. [7] The case presented here had symptoms of headache, visual disturbances in the form of bitemporal hemianopia, and primary infertility. She was evaluated outside for infertility and was found to have normal reproductive anatomy except for low FSH and LH levels. On further evaluation she was detected to have a cystic sellar-suprasellar lesion with calcification. A preoperative CT scan would have been ideal for detection of calcification but was not done as the possibility of rathke's cleft cyst was not suspected initially. Our patient had selective deficiency of serum FSH and LH, other hormones being in the normal range which explains the cause of her infertility. Her post operative course was uneventful except for transient hyponatremia for initial few days which later subsided with dietary supplementation and also improvement in headache and vision at the end of 1 year follow up.

 
  References Top

1.Keyaki A, Hirano A, Llena JF. Asymptomatic and symptomatic Rathke's cleft cysts. Histological study of 45 cases. Neurol Med Chir(Tokyo) 1989;29:88-93.  Back to cited text no. 1
    
2.Le BH, Towfighi J, Kapadia SB, Lopes MB. Comparative immunohistochemical assessment of craniopharyngioma and related lesions. Endocr Pathol 2007;18:23-30.  Back to cited text no. 2
    
3.Palaoglu S, Bavbek M, Peker S, Onol B, Sungur A, Erbengi A. Ossified somatotropinoma. Surg Neurol 1994;41:143-6.  Back to cited text no. 3
    
4.Rao GP, Blyth CP, Jeffreys RV. Ophthalmic manifestations of Rathke's cleft cysts. Am J Ophthalmol 1995;119:86-91.  Back to cited text no. 4
    
5.Eguchi K, Uozumi T, Arita K, Kurisu K, Yano T, Sumida M, et al. Pituitary function in patients with Rathke's cleft cyst: Significance of surgical management. Endocr J 1994;41:535-40.  Back to cited text no. 5
    
6.Voelker JL, Campbell RL, Muller J. Clinical, radiographic, and pathological features of symptomatic Rathke's cleft cysts. J Neurosurg 1991;74:535-44.  Back to cited text no. 6
    
7.Shin JL, Asa SL, Woodhouse LJ, Smyth HS, Ezzat S. Cystic lesions of the pituitary: Clinicopathological features distinguishing craniopharyngioma, Rathke's cleft cyst, and arachnoid cyst. J Clin Endocrinol Metab 1999;84:3972-82.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

This article has been cited by
1 Rathke Cleft Cyst with Entirely Ossified Cyst Wall and Partially Solid Cyst Content: A Case Report and Literature Review
Yasuhiko Hayashi,Masahiro Oishi,Issei Fukui,Yasuo Sasagawa,Ken-ichi Harada,Mitsutoshi Nakada
World Neurosurgery. 2017; 98: 882.e15
[Pubmed] | [DOI]



 

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