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LETTER TO EDITOR
Year : 2014  |  Volume : 62  |  Issue : 1  |  Page : 86-88

Anterior third ventricular epidermoid presenting with diabetes insipidus


Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi, India

Date of Submission25-Dec-2013
Date of Decision11-Jan-2014
Date of Acceptance26-Jan-2014
Date of Web Publication7-Mar-2014

Correspondence Address:
Bhawani S Sharma
Department of Neurosurgery, All India Institute of Medical Sciences, New Delhi
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0028-3886.128345

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How to cite this article:
Kumar A, Singla R, Sharma BS. Anterior third ventricular epidermoid presenting with diabetes insipidus. Neurol India 2014;62:86-8

How to cite this URL:
Kumar A, Singla R, Sharma BS. Anterior third ventricular epidermoid presenting with diabetes insipidus. Neurol India [serial online] 2014 [cited 2019 Nov 12];62:86-8. Available from: http://www.neurologyindia.com/text.asp?2014/62/1/86/128345


Sir,

Epidermoids are rare and account for 0.2-1.0% of all intracranial tumors. [1] The common location for epidermoids are cerebellopontine angle and parasellar region followed by middle cranial fossa. [2] Intraventricular epidermoids are less frequent and third ventricular epidermoids are rarest accounting for 0.7% of all intracranial epidermoids. [2] Though parasellar/suprasellar epidermoids frequently extend into third ventricle, only few cases of truly intra-third ventricular epidermoids have been reported. [3],[4] Intraventricular epidermoids usually present with features of raised intracranial pressure. Though parasellar/suprasellar epidermoids may present with anterior pituitary hormonal dysfunction, diabetes insipidus (DI) as a presenting feature of an intraventricular epidermoid has not been previously described. We hereby describe one such rare case of an anterior third ventricular epidermoid presenting with DI.

A 40-year-old male presented with polydipsia and polyuria for past 2 years and headache for past 6 months. Neurological examination was unremarkable except for mild bilateral papilledema and decreased visual acuity (vision-6/9 both eyes). Urine specific gravity ranged from 1000 to 1004. Serum sodium was between 150 and 160 meq/ml. Anterior pituitary hormonal profile was normal. Radiological evaluation revealed anterior third ventricular lesion that was hypodense on computed tomography scan [Figure 1]a, heterogeneously hypointense on T1W [Figure 1]b and heterogeneously hyperintense on T2W [Figure 1]c images and showed peripheral rim enhancement [Figure 1]d and e. These radiological features raised the suspicion of cystic craniopharyngioma. However, the diagnosis became clear with diffusion weighted sequences showing restricted diffusion [Figure 1]f and g thereby suggesting a diagnosis of epidermoid cyst which was confirmed intraoperatively. Patient underwent removal of anterior third ventricular epidermoid through bifrontal basal interhemispheric trans-lamina terminalis approach. Intraoperatively, characteristic pearly flakes could be seen inside third ventricle [Figure 1]h. Complete removal of epidermoid was achieved except for a small portion of its wall adherent to floor of third ventricle. Post-operative course was uneventful. The symptoms of DI subsided and the patient was discharged home after 5 days.
Figure 1: Anterior third ventricular lesion, hypodense on computed tomography scan (a), heterogeneously hypointense on T1W (b) and heterogeneously hyperintense on T2W (c) images. Contrast images show peripheral rim enhancement (a, d and e). Restricted diffusion can be appreciated on diffusion weighted images (f and g). Intraoperatively, anterior third ventricular pearly white tumor could be seen via trans-lamina terminalis approach (h)

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Epidermoids originate from epithelial remanants implanted during neural tube closure during 3 rd and 5 th weeks of embryogenesis. [5] The squamous epithelium forms the lining of epidermoid cyst that secretes keratin. [5] A study by Iaconetta and Samii [4] proposed that during neurulation, migration of multipotent mesoectodermal cells, derived from neural crest cells, into ventricular ependyma leads to intraventricular epidermoids.

Clinical manifestations of epidermoids depend upon location, rate of growth, obstruction of cerebrospinal fluid flow and compression of neurovascular structures and symptoms usually evolve very slowly. Intraventricular epidermoids are rare and usually involve lateral and fourth ventricles, third ventricular epidermoids being extremely rare. [3],[4] Intraventricular epidermoids present with symptoms of raised intracranial pressure. Patients can also present with recurrent episodes of aseptic meningitis. Though anterior pituitary hormonal dysfunction has been reported in patients with parasellar/suprasellar epidermoids, DI as a manifestation of intraventricular epidermoids has never been reported previously. The development of DI can be explained by direct pressure effect of epidermoid on hypothalamus. The non-occurrence of DI as a manifestation of intraventricular epidermoids can be explained by rarity of intraventricular epidermoids especially the anterior third ventricular epidermoids. This case adds to the spectrum of clinical manifestations of intraventricular epidermoids. Craniopharyngiomas make a close differential of a lesion in this location presenting with DI.

Although complete excision of cyst contents along with its wall is the ideal treatment for epidermoid cysts, extent of excision depends upon the relationship of the cyst to surrounding structures. In the presence of a well-defined cleavage plane between cyst wall and surrounding structures, complete excision can be achieved, however, if cyst wall is adherent to important neural/vascular structures, leaving a portion of wall behind rather than damaging a vital structure, in an overzealous attempt to completely excise it, is a pragmatic approach.

In conclusion, anterior third ventricular epidermoids can rarely present with DI and closely mimick craniopharyngiomas radiologically as well as clinically and should be considered in differential diagnosis of anterior third ventricular lesions presenting in such manner.

 
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1.Kobata H, Kondo A, Iwasaki K. Cerebellopontine angle epidermoids presenting with cranial nerve hyperactive dysfunction: Pathogenesis and long-term surgical results in 30 patients. Neurosurgery 2002;50:276-85.  Back to cited text no. 1
    
2.Liu YG. Epidermoid cysts. In: Liu YG, editor. Congenital Cranial - Cerebral Diseases. 1 st ed. Jinan: Jinan Press; 1993. p. 92-6.  Back to cited text no. 2
    
3.Giannotta SL, Pauli F, Farhat SM. Epidermoid cyst of the third ventricle. Surg Neurol 1976;5:164-6.  Back to cited text no. 3
    
4.Iaconetta G, Samii M. Third ventricle epidermoid cyst. Br J Neurosurg 2001;15:529-30.  Back to cited text no. 4
    
5.Tancredi A, Fiume D, Gazzeri G. Epidermoid cysts of the fourth ventricle: Very long follow up in 9 cases and review of the literature. Acta Neurochir (Wien) 2003;145:905-10.  Back to cited text no. 5
    


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